TY - JOUR
AB -   262 An echo and CMR study in children with repaired tetralogy of Fallot and their correlation with peak VO2 Martina Avesani Martina Avesani Royal Brompton Hospital, London, United Kingdom University of Padua, Italy Nunzia Borrelli Nunzia Borrelli Royal Brompton Hospital, London, United Kingdom Elena Filippini Elena Filippini Royal Brompton Hospital, London, United Kingdom Grazia Delle Donne Grazia Delle Donne Royal Brompton Hospital, London, United Kingdom Jolanda Sabatino Jolanda Sabatino Royal Brompton Hospital, London, United Kingdom Sylvia Krupickova Sylvia Krupickova Royal Brompton Hospital, London, United Kingdom Manjit Josen Manjit Josen Royal Brompton Hospital, London, United Kingdom Sabino Iliceto Sabino Iliceto University of Padua, Italy Giovanni Di Salvo Giovanni Di Salvo Royal Brompton Hospital, London, United Kingdom Royal Brompton Hospital, London, United Kingdom University of Padua, Italy Background: Severe pulmonary regurgitation (PR) and right ventricular (RV) disfunction are common in patients with repaired Tetralogy of Fallot (r-TOF), and CMR has become the gold standard for their assessment. However, in paediatric patients CMR use can have some limitations. The aim of our study was to correlate Echo and CMR parameters and to analyse which parameter was associated with peak oxygen consumption (Vo2) in a paediatric population of r-TOF with at least moderate PR assessed by Echo. Methods: r-TOF patients (<18 years) with at least moderate PR were included in the study. Echocardiographic parameters: PR was assessed by Color and Continuous-wave (CW) Doppler and derived parameters such as pressure half time (PHT), PR index, ratio of diastolic and systolic time-velocity integrals (DSTVI) of the main pulmonary artery; RV end-diastolic area (RVEDA), end-systolic area (RVESA), right ventricle outflow tract (RVOT) end-diastolic area, fractional area change (FAC) and TAPSE; by speckle tracking analysis we measured RV global longitudinal strain (RVGLS) and right atrial strain (RAS). CMR parameters: we evaluated PR as RF, end-diastolic and systolic volumes (RVEDV, RVESV) and right ventricle ejection fraction (RVEF). In addition, we collected values of peak Vo2 evaluated by cardiopulmonary exercise test (CPET). Results: Fiftythree r-TOF patients (aged 13.8 ± 2.5 years, male 57%) were included. Free PR (RF > 35%) was diagnosed in 38 out of 53 patients and nobody had > mild tricuspid regurgitation. We found a significant correlation between RVEDA and CMR RVEDV (p < 0.0001, r = 0.73), which slightly improved adding RVOT area (p < 0.0001, r = 0.75). RVEDAi (indexed)> 21.9 cm2/m2 was found to have a good sensitivity (81.8%) for RVEDVi (indexed)>150 ml/m2. No correlation was found neither between TAPSE, FAC, RVGLS measured by echo and RVEF calculated by CMR nor between echo Doppler parameters and PR RF. None of the CMR parameters correlated with peak Vo2. Among the studied echo parameters only RAS demonstrated a significant correlation (p < 0.0001, r = 0.70) with peak Vo2. At the multivariate analysis only RAS was the best independent predictor of peak Vo2 (p < 0.0001). Conclusion: Echo Doppler parameters studied to assess PR were unsatisfactory and did not correlate with PR RF by CMR. RVEDA was well correlated with CMR volumes. RAS was the best predictor of peak Vo2 and should be included in the Follow-up of children with r-TOF. 56 Clinical impact of right ventricular diastolic patterns in idiopathic pulmonary arterial hypertension by speckle traiking echocardiography Roberto Badagliacca Roberto Badagliacca Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Beatrice Pezzuto Beatrice Pezzuto Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Cristiano Miotti Cristiano Miotti Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Silvia Papa Silvia Papa Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Roberto Poscia Roberto Poscia Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Giovanna Manzi Giovanna Manzi Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Antonella Pascaretta Antonella Pascaretta Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Federico Luongo Federico Luongo Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Roberto Torre Roberto Torre Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Gavino Casu Gavino Casu Dipartimento di Cardiologia-Nuoro Susanna Sciomer Susanna Sciomer Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Francesco Fedele Francesco Fedele Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Robert Naeije Robert Naeije Dipartimento di Cardiologia-Erasme University Hospital, Bruxelles Carmine Dario Vizza Carmine Dario Vizza Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Dipartimento di Cardiologia-Nuoro Dipartimento di Cardiologia-Erasme University Hospital, Bruxelles Objectives: Aim of this study was to describe strain-derived right ventricular (RV) diastolic patterns by speckle-tracking echocardiography (STE) and its clinical impact in idiopathic pulmonary arterial hypertension (IPAH). Background: STE of the RV has been extensively described in PAH. However, diastolic function has been yet underlooked (neglected) for no reason. Methods: In 108 consecutive IPAH patients we identified three distinct strain-derived patterns from the mid-basal RV free wall segments. Each patient underwent baseline clinical, hemodynamic and complete echocardiographic evaluation and followed-up for clinical worsening occurrence. Results: The three strain-derived diastolic patterns were characterized by high reproducibility (Cohen's κ = 0.64, p = 0.0001). Pattern 1 was associated with mild pulmonary hypertension and preserved clinical and RV function (preserved RV phenotype). This pattern was repetitively found in a cohort of 30 healthy subjects. Pattern 2 was associated with moderate to severe pulmonary hypertension, WHO functional class II and III, still preserved RV function (RV adaptive phenotype). Pattern 3 was associated with advanced stage of IPAH, characterized by high right atrial pressure, low cardiac index and severe RV remodelling (RV maladaptive phenotype). Multivariable models for clinical worsening (CW) prediction demonstrated that the addition of RV diastolic patterns to clinical and hemodynamic variables significantly increased the prognostic power of the model (0.79 vs 0.66; p < 0.001). Freedom from CW rates at 1 and 2 years from baseline were, respectively, 100% and 93% for Pattern 1; 80% and 55% for Pattern 2; 60% and 33% for Pattern 3. Conclusions: The results of the present study suggest that using speckle tracking echocardiography we can identify three phenotypically distinct, reproducible and clinically meaningful RV strain-derived diastolic patterns. 57 Adding right ventricular reverse remodeling criteria to risk assessment scores in pulmonary arterial hypertension Roberto Badagliacca Roberto Badagliacca Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Silvia Papa Silvia Papa Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Giovanna Manzi Giovanna Manzi Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Roberto Poscia Roberto Poscia Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Beatrice Pezzuto Beatrice Pezzuto Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Matteo Minnucci Matteo Minnucci Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Antonella Pascaretta Antonella Pascaretta Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Cristiano Miotti Cristiano Miotti Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Federico Luongo Federico Luongo Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Susanna Sciomer Susanna Sciomer Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Roberto Torre Roberto Torre Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Francesco Ciciarello Francesco Ciciarello Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Nadia Cedrone Nadia Cedrone Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Francesco Fedele Francesco Fedele Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Robert Naeije Robert Naeije Dipartimento di Patofisiologia-Free University of Brussels Carmine Dario Vizza Carmine Dario Vizza Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Dipartimento di Patofisiologia-Free University of Brussels Background: Risk assessment is important for prognostication and therapeutic adjustments in pulmonary arterial hypertension (PAH). However, currently used scores do not include direct assessments of the right ventricle (RV) and most patients are assigned the uncertainty of intermediate risk. Objectives: To evaluate the added value echocardiographic criteria of right heart reverse remodelling (RHRR) in advanced PAH. Methods: 110 consecutive PAH patients started on parenteral prostanoids were followed with periodic clinical, hemodynamic, and echocardiographic assessments. RHRR at 1-year assessment was defined by the combined reductions in right atrial (RA) and RV dimensions and left ventricular eccentricity index. Results: Thirty-six (32.7%) patients had a RHRR at 1-year follow-up. After a mean follow-up of 1375 ± 1359 days, 48 (43.6%) patients died. The European Respiratory and Cardiology Societies guidelines-derived risk score based on WHO class, 6-min walk distance, RA pressure and cardiac index at 1-year predicted long-term survival. The combination of low-risk European score and RHRR was able to improve the prognostic power of the model (c-statistic: 0.83, C.I. 0.75-0.92, vs 0.75, C.I. 0.64-0.86, respectively; p < 0.001). Similar results applied to the United States Registry to Evaluate Early and Long-Term PAH Disease Management risk score 2.0. A reduction of pulmonary vascular resistance (PVR) >50% was associated with higher probability of RHRR. Patients with RHRR had greater improvement in WHO class, 6 min walk distance and hemodynamics. Conclusions: Echocardiographic RHRR improves risk assessment in advanced PAH under prostacyclin therapy. A reduction in PVR by more than 50 % is associated with a high probability RHRR and improved prognosis. 58 Risk reduction and right heart reverse remodeling by upfront triple combination therapy in pulmonary arterial hypertension Roberto Badagliacca Roberto Badagliacca Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Paola Argiento Paola Argiento Dipartimento di Cardiologia, Ospedale Monaldi- Università di Napoli L.Vanvitelli Emanuele Romeo Emanuele Romeo Dipartimento di Cardiologia, Ospedale Monaldi- Università di Napoli L.Vanvitelli Andrea Farro Andrea Farro Dipartimento di Cardiologia, Ospedale Monaldi- Università di Napoli L.Vanvitelli Silvia Papa Silvia Papa Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Giovanna Manzi Giovanna Manzi Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Cristiano Miotti Cristiano Miotti Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Federico Luongo Federico Luongo Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Berardo Sarubbi Berardo Sarubbi Dipartimento di Cardiologia, Ospedale Monaldi- Università di Napoli L.Vanvitelli Maria Giovanna Russo Maria Giovanna Russo Dipartimento di Cardiologia, Ospedale Monaldi- Università di Napoli L.Vanvitelli Carmine Dario Vizza Carmine Dario Vizza Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Paolo Golino Paolo Golino Dipartimento di Cardiologia, Ospedale Monaldi- Università di Napoli L.Vanvitelli Robert Naeije Robert Naeije Dipartimento di Patofisiologia-Free University of Brussels Michele D´Alto Michele D´Alto Dipartimento di Cardiologia, Ospedale Monaldi- Università di Napoli L.Vanvitelli Dipartimento di Cardiologia, Ospedale Monaldi- Università di Napoli L.Vanvitelli Dipartimento di Scienze Cardiovascolari e Respiratorie-Sapienza Università di Roma Dipartimento di Patofisiologia-Free University of Brussels Background: Combinations of therapies are currently recommended for the most severely ill patients with pulmonary arterial hypertension (PAH), and excellent results have been reported with triple upfront combination of these drugs. We evaluated the effects of this approach on right ventricular (RV) function and outcome in patients with severe PAH. Methods: Twenty-one patients aged 44 ± 15 years with newly diagnosed high-risk idiopathic PAH that was non-reversible by the inhalation of nitric oxide were treated upfront with a combination of ambrisentan, tadalafil and subcutaneous treprostinil between 2014 and 2018. Clinical evaluation, World Health Organization functional class, 6-min walk distance, biomarkers, echocardiography and right heart catheterization data were recorded at baseline and during follow-up. Results: At a median follow-up of 2 years, all patients were still alive. The REVEAL score decreased from 10 ± 1 to 5 ± 1, right atrial pressure from 13 ± 3 to 5 ± 2 mmHg, mean pulmonary artery pressure from 60 ± 9 to 42 ± 5 mmHg, pulmonary vascular resistance (PVR) from 16.4 ± 4.4 to 5.5 ± 1.3 Wood units, NT-proBNP from 3379 ± 1921 to 498 ± 223 pg/mL and World Health Organization functional class from 3.4 ± 0.5 to 2.0 ± 0.4 (all p < 0.001). Cardiac index increased from 1.8 ± 0.3 to 3.5 ± 0.8 L/min/m2 and 6-min walk distance from 158 ± 130 to 431 ± 66 m (both p < 0.001). Echocardiography showed decreased right atrial and RV areas, improved left ventricular eccentricity index and increased fractional area change (all p < 0.001) in proportion to treatment-induced decrease in PVR. Conclusions: Triple upfront combination therapy with ambrisentan, tadalafil and subcutaneous treprostinil in severe non-reversible PAH is associated with considerable clinical and hemodynamic improvement and right heart reverse remodelling. 532 Diastolic dysfunction in post-surgical tetralogy of Fallot patients: comparison between cardiac MRI and echocardiography Pier Paolo Bassareo Pier Paolo Bassareo University College of Dublin, Irlanda Martino Deidda Martino Deidda Dipartimento di Scienze Mediche e Sanità Pubblica, Università degli Studi di Cagliari Andrea Raffaele Marras Andrea Raffaele Marras Università degli Studi di Cagliari Luca Saba Luca Saba Dipartimento di Scienze Mediche e Sanità Pubblica, Università degli Studi di Cagliari Giuseppe Mercuro Giuseppe Mercuro Dipartimento di Scienze Mediche e Sanità Pubblica, Università degli Studi di Cagliari University College of Dublin, Irlanda Università degli Studi di Cagliari Dipartimento di Scienze Mediche e Sanità Pubblica, Università degli Studi di Cagliari Introduction: right ventricular (RV) systolic dysfunction is associated with poor outcomes in Tetralogy of Fallot (ToF) patients. Conversely, the influence of diastolic dysfunction is poorly known. In addition, evaluation of diastolic function with cardiac MR imaging is rarely performed. We studied the possible association between traditional echocardiographic parameters of RV diastolic function and cardiac MRI evaluation of trans-tricuspidal flow. Methods: twenty-four surgically treated ToF patients were enrolled (54% males; mean age at study: 34 ± 2.7 years; mean age at surgery: 2.2 ± 0.7 years). They were studied by echocardiography to assess RV diastolic function in terms of traditional and TDI derived parameters (E/A, E/e', deceleration time, MPI). At cardiac MRI, RV diastolic function was assessed by using phase-contrast analysis of flow through the tricuspid valve in short axis view. Diastolic dysfunction was graded as impaired relaxation, pseudonormal, or restrictive physiology. Results: fifteen (62.5%) ToF subjects had echocardiographic evidence of diastolic dysfunction and eleven (45.8%) at cardiac MRI. A statistically significant correlation was not found between echocardiographic and cardiac MRI parameters of diastolic dysfunction. At cardiac MRI, higher degree of RV diastolic dysfunction (restrictive physiology) was weakly associated with larger indexed right atrial area (p < 0.04). Greater number of interim palliative procedures was associated with higher E/e' (diastolic dysfunction) at echocardiography. Conclusions: diastolic dysfunction, as determined by echocardiography and cardiac MRI-derived measures, is prevalent in ToF. These measures are not associated with each other. This lack of correlation between cardiac MRI-based and traditional trans-thoracic parameters of diastolic function seems to highlight some limitations which need to be considered, at least in the ToF setting. As soon as cardiac MRI will have the ability to better characterize a range of diastolic impairments, it will likely become an important diagnostic test in the future, capable of comprehensive RV function evaluation in ToF. 586 Diagnostic performance of echocardiographic coronay arteries abnormalities assesment Francesco Bianco Francesco Bianco Dipartimento di Cardiologia Pediatrica e Congenita e di Chirurgia Cardiaca- Azienda Ospedaliera- Ospedali Riuniti di Ancona Massimo Colaneri Massimo Colaneri Dipartimento di Cardiologia Pediatrica e Congenita e di Chirurgia Cardiaca- Azienda Ospedaliera- Ospedali Riuniti di Ancona Annaclara Biasi Annaclara Biasi Istituto di Cardiologia Universitaria- Università G. D’Annunzio- Chieti Pescara Valentina Bucciarelli Valentina Bucciarelli Dipartimento di Cardiologia Pediatrica e Congenita e di Chirurgia Cardiaca- Azienda Ospedaliera- Ospedali Riuniti di Ancona Francesca Chiara Surace Francesca Chiara Surace Dipartimento di Cardiologia Pediatrica e Congenita e di Chirurgia Cardiaca- Azienda Ospedaliera- Ospedali Riuniti di Ancona Federica Valentina Iezzi Federica Valentina Iezzi Dipartimento di Cardiologia Pediatrica e Congenita e di Chirurgia Cardiaca- Azienda Ospedaliera- Ospedali Riuniti di Ancona Martina Primavera Martina Primavera Istituto di Cardiologia Universitaria- Università G. D’Annunzio- Chieti Pescara Giuliano Giusti Giuliano Giusti Dipartimento di Cardiologia Pediatrica e Congenita e di Chirurgia Cardiaca- Azienda Ospedaliera- Ospedali Riuniti di Ancona Sante Donato Pierdomenico Sante Donato Pierdomenico Istituto di Cardiologia Universitaria- Università G. D’Annunzio- Chieti Pescara Sabina Gallina Sabina Gallina Istituto di Cardiologia Universitaria- Università G. D’Annunzio- Chieti Pescara Marco Pozzi Marco Pozzi Dipartimento di Cardiologia Pediatrica e Congenita e di Chirurgia Cardiaca- Azienda Ospedaliera- Ospedali Riuniti di Ancona Dipartimento di Cardiologia Pediatrica e Congenita e di Chirurgia Cardiaca- Azienda Ospedaliera- Ospedali Riuniti di Ancona Istituto di Cardiologia Universitaria- Università G. D’Annunzio- Chieti Pescara Aims: to assess the diagnostic performance of a new echocardiographic-based screening program for coronary arteries abnormalities (CAA) in pediatric and young adults’ population. Methods: in 2014-2018, we screened for CAA 5, 998 patients applying a focused 4-views echocardiographic coronary assessment: parasternal short-axis, parasternal long-axis, apical 5-chambers, and parasternal long-axis for the right ventricle outflow tract. All the suspected CAA underwent a coronary-CT scan for confirmation. Two independent physicians retrospectively reviewed the echocardiographic images and adjudicated the diagnosis in a double-blinded fashion. Results: over 5 years, we detected 27 total CT-confirmed CAA. The overall prevalence was 0.0045%, 0.027% every 1, 000 patients. N = 17/27 (63%) were anomalous aortic origin of coronary arteries (AAOCA), N = 3/27 (11%) anomalous coronary arteries from the pulmonary artery (ACAPA), and 7/27 (26%) fistulas. We found a progressive increment of CAA diagnosis (P for Trend = 0.038), in particular of AAOCA: both left and right coronaries (P-trend = 0.021 and P=trend 0.010, respectively). Our method showed better sensitivity than traditional CAA echocardiographic evaluation: 85% vs 55%, P = 0.032 [AUC 0.77, 95% CI (0.68, 0.87) and AUC 0.92, 95% CI (0.85, 0.99), respectively], with a good interrater agreement for adjudicated retrospective diagnosis, blinded to coronary-CT (99.75%, K = 0.73, P < 0.001). Conclusions: the application of an echocardiographic screening program for CAA-detection led to a significantly increased rate of identified anomalies. This approach demonstrated better sensitivity than traditional echocardiographic one-view assessment. Implementing this protocol in clinical practice may improve the CAA diagnosis, and probably reduce the occurrence of CAA-related sudden cardiac death. Keywords: Coronary arteries abnormalities; anomalous aortic origin of coronary arteries; anomalous coronary arteries from the pulmonary artery; coronary origin; coronary screening. 651 Blood flow speckle tracking as new technique to discriminate outflow tract obstruction: a TGA case report Nunzia Borrelli Nunzia Borrelli Royal Brompton Hospital, Londra, Uk Martina Avesani Martina Avesani Royal Brompton Hospital, Londra, Uk Jolanda Sabatino Jolanda Sabatino Royal Brompton Hospital, Londra, Uk Aladino Ibrahim Aladino Ibrahim Royal Brompton Hospital, Londra, Uk Manjit Josen Manjit Josen Royal Brompton Hospital, Londra, Uk Giovanni Di Salvo Giovanni Di Salvo Royal Brompton Hospital, Londra, Uk Royal Brompton Hospital, Londra, Uk Case report: We present the case of a 3.5-month-old baby girl with an antenatal diagnosis of Transposition of Great Arteries (TGA). At first day of life, an urgent echocardiogram confirmed TGA diagnosis with a large perimembranous ventricular septal defect (VSD) shunting right to left. A tunnel like sub-pulmonary infundibulum was also detected. After the fall of pulmonary resistances, and in presence of blood hyperflow through the VSD, an increased gradient across the pulmonary valve was noticed at CW Doppler assessment. Since the presence of pulmonary stenosis can condition the surgical approach, we decided to perform another echocardiogram by using blood flow speckle tracking (BFST) technique to assess the flow at outflow tracts.This evaluation showed laminar flow at pulmonary valve level, without any turbulences. The patient underwent a successful arterial switch operation (ASO) and VSD closure. The post-operative echocardiogram confirmed the absence of neo-aortic and neo-pulmonary stenosis and the absence of outflow tracts obstruction. Discussion: Left ventricle outflow tract (LVOT) obstruction complicates almost 10% of TGAs with associated VSD. To perform ASO it is generally required competent and unobstructed ventriculo-arterial connections. Frequently, an abnormal shunt like a VSD can be responsible of an hyperdynamic flow. However, this is a functional phenomenon rather than a true anatomic lesion. This kind of functional gradient does not need correction and generally solves repairing the abnormal shunts. However, true anatomic obstructions must be carefully assessed for the practicability of surgical relief and change completely the surgical approach. In presence of TGA with LVOT functional gradient, BFST may help to discriminate between a true obstruction and an increase in pressure gradient secondary to flow overload. Indeed, in this patient with d-TGA, VSD and no pulmonary stenosis, BFST demonstrated a central laminar flow across the unobstructed left outflow tract with no turbulences and no vortex appearing in main pulmonary artery arising from left ventricle, despite the presence of a significant gradient at traditional Doppler assessment. 215 A multimodal approach in a patient with an uncorrected tetralogy of Fallot: a clinical case report Fabrizio Nicolò Stefano Perrone Fabrizio Nicolò Stefano Perrone Policlinico Tor Vergata Beggio Elisa Beggio Elisa Policlinico Tor Vergata Luciano Emidio Di Battista Luciano Emidio Di Battista Policlinico Tor Vergata Marina Vellini Marina Vellini Policlinico Tor Vergata Saverio Muscoli Saverio Muscoli Policlinico Tor Vergata Marcello Chiocchi Marcello Chiocchi Policlinico Tor Vergata Francesco Romeo Francesco Romeo Policlinico Tor Vergata Valeria Cammalleri Valeria Cammalleri Policlinico Tor Vergata Policlinico Tor Vergata Tetralogy of Fallot (TOF) is one of the most common congenital heart diseases. The clinical presentation generally begins in the first days of life, with difficult in feeding, breathing and cyanosis. TOF usually needs a surgical correction to allow a better quality of life. Without such surgical correction, mortality is frequent. Cases of uncorrected TOF that reach adult age without a surgical correction are rare. Here in, we describe a case report of a 61-year-old male patient with an uncorrected TOF, diagnosed at 21 years old. In 2012, complaining chest pain and dyspnoea, he was admitted to the emergency room (ER) of our institute. The electrocardiogram showed a 2: 1 atrioventricular block (AV) with normal cardiac markers of necrosis. For this reason, first a temporary pacemaker was placed, followed by a definitive pacemaker implantation. During the same hospitalization, a coronary angiography was performed showing anomalies in the origin of the coronary branches: the right sinus of Valsalva gave rise to the right coronary artery, which divided to form the anterior descending artery and the right coronary artery. The left circumflex arose from the left sinus of Valsalva with a dominant pattern. A ventriculography was performed: the left ventricle was hypertrophic with normal ejection fraction; right ventricle was hypertrophic with infundibular stenosis. No hemodynamically significant coronary stenosis were found. Four years later, because of recurrence of chest pain, he arrived at the ER and a new coronarography study was performed confirming the coronaries anomaly without evidence of stenosis. To better evaluate the anatomy of the heart, a thoracic computed tomography angiography was performed showing the four typical structural anomalies of TOF and a residual communication between the aortic isthmus and the proximal tract of the left pulmonary artery. In this case report we can evaluate the association of different congenital anomalies. It is not common to find an uncorrected tetralogy of Fallot in old age and the hemodynamic stability of this patient is possible thanks to a wide ventricular septal defect and a residual patent ductus arteriosus that creates the possibility to bypass the pulmonary stenosis and determinates a left-to-right shunt. It is rare to have an AV block in a patient without a coronary artery disease and an uncorrected tetralogy of Fallot. It is also uncommon, to find anomalies in the origin of the coronaries. In conclusion, our study highlights the importance of a multimodality approach in adults with complex congenital heart disease, especially if symptoms such as chest pain and bradyarrhythmias are present. 666 Lesson from a LMNA-associated cardiomyopathy: from pathogenic mechanisms to pharmacological targets Andrea Gerbino Andrea Gerbino Dipartimento di Bioscienze, Biotecnologie e Biofarmaceutica-Universita’ degli Studi di Bari Roberta De Zio Roberta De Zio Dipartimento di Bioscienze, Biotecnologie e Biofarmaceutica-Universita’ degli Studi di Bari Giuseppe Procino Giuseppe Procino Dipartimento di Bioscienze, Biotecnologie e Biofarmaceutica-Universita’ degli Studi di Bari Maria Svelto Maria Svelto Dipartimento di Bioscienze, Biotecnologie e Biofarmaceutica-Universita’ degli Studi di Bari Martino Pepe Martino Pepe Dipartimento delle Emergenze e dei Trapianti d’Organo-Universita’ delgi Studi di Bari Stefano Favale Stefano Favale Dipartimento delle Emergenze e dei Trapianti d’Organo-Universita’ delgi Studi di Bari Cinzia Forleo Cinzia Forleo Dipartimento delle Emergenze e dei Trapianti d’Organo-Universita’ delgi Studi di Bari Monica Carmosino Monica Carmosino Dipartimento di Scienze-Universita’ della Basilicata Dipartimento di Bioscienze, Biotecnologie e Biofarmaceutica-Universita’ degli Studi di Bari Dipartimento delle Emergenze e dei Trapianti d’Organo-Universita’ delgi Studi di Bari Dipartimento di Scienze-Universita’ della Basilicata Mutations in the LMNA gene, which encodes A-type nuclear Lamins, are among the most frequent genetic cause of Arrhythmogenic Cardiomyopathies with conduction defects, often leading to sudden death (SD). So far the only effective treatment consists of the implantable cardioverter defibrillator (ICD) to prevent SD. In this scenario, the dissection of the pathogenic mechanisms triggered by LMNA mutations may reveal new pharmacological targets for the managements of these cardiomyopathies. We identified a novel LMNA nonsense mutation (Q517X) in an Italian family co-segregating with a clinical history of Sick Sinus-Node Syndrome (SSS) as onset of the pathology. Thereafter a permanent Atrial Fibrillation (AF) became the main clinical feature of the mutant carriers. Later on a DCM with left ventricular dysfunction leaded to a sever heart insufficiency. Aim of this study is to gain insights into the unknown pathogenic mechanism triggered by the expression of Q517X at cellular level. We functionally characterized the mutant variant LMNA Q517X in murine atrial cardiomyocytes, which acquired the ability to beat spontaneously once in culture, thus representing a unique model for studying the pathophysiology of atrial automaticity. Studying the Action Potential (AP) propagation by patch clamp experiments, we found a significant prolongation of both Action Potential duration (APD) and AP Cycle length in LMNA Q517X-expressing cardiomyocytes. Moreover, the expression of LMNA Q517X mutant conferred a proarrhythmic activity to the cardiomyocytes, which showed after depolarization (AD) events during the AP firing, never observed in LMNA WT-expressing cardiomyocytes. These electrical features, such as APD prolongation together with AD events, may trigger AF in humans. Indeed, we have been able to reproduce the clinical phenotype at single cell level upon Q517X expression in atrial cardiomyocytes. Of note, we identify a defect in calcium release from the Sarcoplasmatic Reticulum (SR) through the Ryanodine Receptors (RyRs) as the molecular pathway involved in the AD events in Q517X-expressing cardiomyocytes. So far, these results suggest the RyRs as potential pharmacological targets for this LMNA associated cardiomyopathy. 8 Ventilatory power: a novel cardiopulmonary exercise testing parameter for the prediction of pulmonary hypertension Ilenia Monaco Ilenia Monaco Tatarella Hospital, Cerignola, Foggia, Italy Lucia Tricarico Lucia Tricarico Department of Cardiology, University of Foggia Michele Correale Michele Correale Department of Cardiology, University of Foggia Francesca Pia D´Altilia Francesca Pia D´Altilia Department of Cardiology, University of Foggia Gianfranco Acanfora Gianfranco Acanfora Department of Cardiology, University of Foggia Stefania Fabrizi Stefania Fabrizi Department of Cardiology, University of Foggia Carla Di Turi Carla Di Turi Department of Cardiology, University of Foggia Armando Ferraretti Armando Ferraretti Civic Hospital, Canosa di Puglia Bat, Italy Matteo Di Biase Matteo Di Biase GVM, Santa Maria Hospital, Bari Natale Daniele Brunetti Natale Daniele Brunetti Department of Cardiology, University of Foggia Department of Cardiology, University of Foggia GVM, Santa Maria Hospital, Bari Tatarella Hospital, Cerignola, Foggia, Italy Civic Hospital, Canosa di Puglia Bat, Italy Background: Several cardiopulmonary exercise test (CPET) parameters (peak VO2, PetCO2 and VE/VCO2) emerged as tools for the prediction of pulmonary arterial hypertension (PAH). Less is known on ventilatory power (VP) in patients with suspect PAH. Aim: To ascertain possible correlations between VP derived at CPET and hemodynamic parameters at right heart catheterization (RHC) indicative of PH. Methods: Forty-seven consecutive outpatients with suspect of PAH were assessed by CPET and RHC; VP was defined as peak SBP divided by the minute ventilation-CO2 production slope at CPET and Diastolic Pressure Gradient (DPG), Trans-pulmonary Pressure Gradient (TPG), mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR) at RHC were also assessed and compared with VP. Results: VP values were inversely related to mPAP (r -0.427, p 0.003), DPG (r -0.36, p 0.019), TPG (r: -0.43, p 0.004), and PVR (r -0.52, p 0.001).Correlations remained significant even after correction at multivariate analysis for age and gender.VP values below median identified subjects with mPAP ≥ 25 mmHg with an odds ratio of 4.5 (95% confidence interval 1.05-19.36, p < 0.05), an accuracy of 0.712 at ROC curve analysis (95% confidence interval 0.534-0.852, p < 0.05) and a positive predictive power 82%. Conclusion: In patients with suspected PAH, VP assessed at CPET might provide further information in predicting PAH at RHC. Correlations with PVR and DPG may be helpful in differentiating patients with isolated post-capillary PH from those with combined post-capillary and pre-capillary. 70 Clinical and echocardiographic follow-up in outpatients with pulmonary arterial hypertension Carla Di Turi Carla Di Turi Department of Cardiology, University of Foggia Giuseppina Padovano Giuseppina Padovano Department of Cardiology, University of Foggia Lucia Tricarico Lucia Tricarico Department of Cardiology, University of Foggia Michele Correale Michele Correale Department of Cardiology, University of Foggia Armando Ferraretti Armando Ferraretti Civic Hospital, Canosa di Puglia Bat Ilenia Monaco Ilenia Monaco Tatarella Hospital, Cerignola FG Alessandra Leopizzi Alessandra Leopizzi Department of Cardiology, University of Foggia Salvatore Tucci Salvatore Tucci Department of Cardiology, University of Foggia Giuseppina Merolla Giuseppina Merolla Department of Cardiology, University of Foggia Natale Daniele Brunetti Natale Daniele Brunetti Department of Cardiology, University of Foggia Matteo Di Biase Matteo Di Biase GVM, Santa Maria Hospital, Bari Department of Cardiology, University of Foggia Civic Hospital, Canosa di Puglia Bat ASL Foggia, Foggia GVM, Santa Maria Hospital, Bari Tatarella Hospital, Cerignola FG Background: PAH is a progressive, life-threatening disease often requiring hospitalization. This study sought to evaluate the relationship between some echocardiographic and clinical parameters and the hospitalization rate of outpatients with pulmonary arterial hypertension (PAH) already treated with at least one specific PAH drug. Methods: Patients with PAH previously diagnosed with RHC were followed up between September 2018 and January 2019. All patients underwent clinical evaluation, 6MWT, blood analysis (in particular NT-proBNP or BNP, CA 125, renal function test) and conventional and TDI echocardiography in an ambulatory setting under resting conditions, at the beginning and after 6 months of therapy with at least one PAH drug. Results: Twenty-five consecutive PAH outpatients (mean age 66, 93 ± 11, 22 years, male: 48%, III NYHA class 52%, PAPm 38, 76 ± 10, 78 mmHg) were enrolled in the study. The following clinical variables were correlated with the number of hospitalization for heart failure: NT-proBNP (r: 0, 75, p = 0, 000), diastolic eccentricity index (r: 0, 52, p = 0, 010), degree of tricuspid regurgitation (r: 0, 48, p = 0, 015), dilated inferior vena cava with reduced respiratory collapse (r: 0, 49, p = 0, 015) and presence of pericardial effusion (r: 0, 64, p = 0, 001). The most significant correlations concern the NTproBNP and the diastolic eccentricity index. Furthermore, the severe degree of tricuspid regurgitation was statistically significant associated with a greater number of admissions due to heart failure compared to the moderate one. Also the presence of dilated VCI and with reduced inspiratory collapse and the presence of pericardial effusion compared with their absence, were statistically significant associated with a greater rate of hospitalization due to heart failure. Conclusions: In outpatients with PAH, our study was able to find some clinical and echocardiographic parameters associated with an increased number of re-hospitalizations. Furthermore, it could identify patients who should be treated with multiple medications specific for PAH. However further multicentric studies with larger population are necessary to confirm it. 20 Experience of three years of balloon pulmonary angioplasty in a single centre: safety and short term results Mariangela Rotunno Mariangela Rotunno DIMES: Department of Experimental, Diagnostic and Specialty Medicine Massimiliano Palazzini Massimiliano Palazzini DIMES: Department of Experimental, Diagnostic and Specialty Medicine Francesco Saia Francesco Saia DIMES: Department of Experimental, Diagnostic and Specialty Medicine Fabio Dardi Fabio Dardi DIMES: Department of Experimental, Diagnostic and Specialty Medicine Nevio Taglieri Nevio Taglieri DIMES: Department of Experimental, Diagnostic and Specialty Medicine Annachiara Tellarini Annachiara Tellarini DIMES: Department of Experimental, Diagnostic and Specialty Medicine Andrea Rinaldi Andrea Rinaldi DIMES: Department of Experimental, Diagnostic and Specialty Medicine Alessandro De Lorenzis Alessandro De Lorenzis DIMES: Department of Experimental, Diagnostic and Specialty Medicine Filippo Pasca Filippo Pasca DIMES: Department of Experimental, Diagnostic and Specialty Medicine Daniele Guarino Daniele Guarino DIMES: Department of Experimental, Diagnostic and Specialty Medicine Elisa Zuffa Elisa Zuffa DIMES: Department of Experimental, Diagnostic and Specialty Medicine Ilenia Magnani Ilenia Magnani DIMES: Department of Experimental, Diagnostic and Specialty Medicine Alberto Ballerini Alberto Ballerini DIMES: Department of Experimental, Diagnostic and Specialty Medicine Alessandra Manes Alessandra Manes DIMES: Department of Experimental, Diagnostic and Specialty Medicine Nazzareno Galiè Nazzareno Galiè DIMES: Department of Experimental, Diagnostic and Specialty Medicine DIMES: Department of Experimental, Diagnostic and Specialty Medicine Background: Balloon pulmonary angioplasty (BPA) has recently been developed as an alternative and less-invasive treatment strategy for chronic thromboembolic pulmonary hypertension (CTEPH) but therapeutic efficacy and technical safety of the technique have to be established. Purpose: To examine the effects of BPA on patients with inoperable disease or residual pulmonary hypertension (PH) after pulmonary endarterectomy (PEA). Methods: From June 2015 to January 2019 we enrolled symptomatic (WHO-FC ≥II) inoperable CTEPH patients and patients with residual PH after PEA. At baseline and after 3 months after last BPA session all patients underwent clinical evaluation, six-minute walking distance (6MWD) and right heart catheterization. For comparisons Friedman test (with Bonferroni post-hoc pairwise analysis) was used. Results: forty-one patients [male 49%, median age 65 (52-75) years, 34 inoperable and 7 with residual PH after PEA] were treated for a total of 111 sessions (median number of sessions for each patient: 2); during each session we treated 2 (2.0-2.5) vessels. Results are shown in the Table. Forty patients were treated with medical therapy before BPA (16 with combination therapy). Four pulmonary artery dissection and 2 haemoptysis with clinical impairment were documented during the procedures; 27 patients had lung injury (radiographic opacity with/without haemoptysis and/or hypoxemia), none had renal dysfunction, 6 patients had access site complications. Five patients died during follow-up (none within 30 days from the procedure) because of sepsis (1), heart failure (1), cancer (1), arrhythmic storm (1) and sudden death in a patients with severe coronary atherosclerosis (1). Conclusions: BPA is a safe and effective treatment able to improve symptoms and hemodynamic profile in inoperable CTEPH patients and in patients with residual PH after PEA. 20 Table median (interquartile range) . Baseline (n = 41) . Baseline ÷ Pre-BPA 8 (3-49) months . Pre-BPA (n = 41) . Pre-BPA ÷ Post-BPA 10 (6-18) months . Post-BPA (n = 32) . Global p-value . WHO-FC III-IV (%) 88 N.S. 83 <0.05 42 <0.001 6MWD (m) 430 (346-560) N.S. 425 (357-500) <0.05 450 (375-605) <0.001 RAP (mmHg) 6 (5-8) N.S. 6 (5-8) N.S. 6 (4-8) 0.023 mPAP (mmHg) 46 (40-52) <0.05 43 (33-50) <0.05 36 (28-41) <0.001 CI (l/min/m2) 2.6 (2.2-3.0) N.S. 2.7 (2.2-3.0) <0.05 3.1 (2.6-3.5) 0.004 PVR (WU) 7.5 (5.6-11.5) <0.05 6.5 (4.7-10.3) <0.05 4.1 (3.3-5.9) <0.001 SvO2 Sat (%) 69 (63-71) N.S. 69 (63-72) N.S. 69 (63-73) 0.002 median (interquartile range) . Baseline (n = 41) . Baseline ÷ Pre-BPA 8 (3-49) months . Pre-BPA (n = 41) . Pre-BPA ÷ Post-BPA 10 (6-18) months . Post-BPA (n = 32) . Global p-value . WHO-FC III-IV (%) 88 N.S. 83 <0.05 42 <0.001 6MWD (m) 430 (346-560) N.S. 425 (357-500) <0.05 450 (375-605) <0.001 RAP (mmHg) 6 (5-8) N.S. 6 (5-8) N.S. 6 (4-8) 0.023 mPAP (mmHg) 46 (40-52) <0.05 43 (33-50) <0.05 36 (28-41) <0.001 CI (l/min/m2) 2.6 (2.2-3.0) N.S. 2.7 (2.2-3.0) <0.05 3.1 (2.6-3.5) 0.004 PVR (WU) 7.5 (5.6-11.5) <0.05 6.5 (4.7-10.3) <0.05 4.1 (3.3-5.9) <0.001 SvO2 Sat (%) 69 (63-71) N.S. 69 (63-72) N.S. 69 (63-73) 0.002 Open in new tab 20 Table median (interquartile range) . Baseline (n = 41) . Baseline ÷ Pre-BPA 8 (3-49) months . Pre-BPA (n = 41) . Pre-BPA ÷ Post-BPA 10 (6-18) months . Post-BPA (n = 32) . Global p-value . WHO-FC III-IV (%) 88 N.S. 83 <0.05 42 <0.001 6MWD (m) 430 (346-560) N.S. 425 (357-500) <0.05 450 (375-605) <0.001 RAP (mmHg) 6 (5-8) N.S. 6 (5-8) N.S. 6 (4-8) 0.023 mPAP (mmHg) 46 (40-52) <0.05 43 (33-50) <0.05 36 (28-41) <0.001 CI (l/min/m2) 2.6 (2.2-3.0) N.S. 2.7 (2.2-3.0) <0.05 3.1 (2.6-3.5) 0.004 PVR (WU) 7.5 (5.6-11.5) <0.05 6.5 (4.7-10.3) <0.05 4.1 (3.3-5.9) <0.001 SvO2 Sat (%) 69 (63-71) N.S. 69 (63-72) N.S. 69 (63-73) 0.002 median (interquartile range) . Baseline (n = 41) . Baseline ÷ Pre-BPA 8 (3-49) months . Pre-BPA (n = 41) . Pre-BPA ÷ Post-BPA 10 (6-18) months . Post-BPA (n = 32) . Global p-value . WHO-FC III-IV (%) 88 N.S. 83 <0.05 42 <0.001 6MWD (m) 430 (346-560) N.S. 425 (357-500) <0.05 450 (375-605) <0.001 RAP (mmHg) 6 (5-8) N.S. 6 (5-8) N.S. 6 (4-8) 0.023 mPAP (mmHg) 46 (40-52) <0.05 43 (33-50) <0.05 36 (28-41) <0.001 CI (l/min/m2) 2.6 (2.2-3.0) N.S. 2.7 (2.2-3.0) <0.05 3.1 (2.6-3.5) 0.004 PVR (WU) 7.5 (5.6-11.5) <0.05 6.5 (4.7-10.3) <0.05 4.1 (3.3-5.9) <0.001 SvO2 Sat (%) 69 (63-71) N.S. 69 (63-72) N.S. 69 (63-73) 0.002 Open in new tab Legend: CI, Cardiac Index; mPAP, mean Pulmonary Arterial Pressure; PVR, Pulmonary Vascular Resistance; RAP, Right Atrial Pressure; 6MWD, 6 Minute Walking Distance; SvO2 Sat, Mixed Venous Oxygen Saturation; WHO-FC, World Health Organization Functional Class 21 Prognostic value of stroke volume index in patients with pulmonary arterial hypertension at intermediate risk Filippo Pasca Filippo Pasca DIMES: Department of Experimental, Diagnostic and Specialty Medicine Fabio Dardi Fabio Dardi DIMES: Department of Experimental, Diagnostic and Specialty Medicine Massimiliano Palazzini Massimiliano Palazzini DIMES: Department of Experimental, Diagnostic and Specialty Medicine Andrea Rinaldi Andrea Rinaldi DIMES: Department of Experimental, Diagnostic and Specialty Medicine Elisa Zuffa Elisa Zuffa DIMES: Department of Experimental, Diagnostic and Specialty Medicine Daniele Guarino Daniele Guarino DIMES: Department of Experimental, Diagnostic and Specialty Medicine Alessandro De Lorenzis Alessandro De Lorenzis DIMES: Department of Experimental, Diagnostic and Specialty Medicine Ilenia Magnani Ilenia Magnani DIMES: Department of Experimental, Diagnostic and Specialty Medicine Mariangela Rotunno Mariangela Rotunno DIMES: Department of Experimental, Diagnostic and Specialty Medicine Alberto Ballerini Alberto Ballerini DIMES: Department of Experimental, Diagnostic and Specialty Medicine Alessandra Manes Alessandra Manes DIMES: Department of Experimental, Diagnostic and Specialty Medicine Nazzareno Galiè Nazzareno Galiè DIMES: Department of Experimental, Diagnostic and Specialty Medicine DIMES: Department of Experimental, Diagnostic and Specialty Medicine Background: current pulmonary hypertension (PH) guidelines stratify the risk of patients with pulmonary arterial hypertension (PAH) using a multiparametric approach. Low, intermediate and high-risk are defined by estimated 1-year mortality of < 5%, 5-10% and >10%, respectively. This risk assessment has been recently validated in 3 cohorts of PAH patients and a simplified risk table for patients with idiopathic/heritable (I/H) PAH and PAH associated with connective tissue disease (CTD) and congenital heart disease (CHD) has been recently proposed and validated. However, with this method most patients are classified in the intermediate risk category and additional strategies are required to further stratify this group of PAH patients. Stroke volume index (SVI) has been recently highlighted as an important prognostic parameter beyond the factors included in current PH guidelines risk table. Purpose: To evaluate the prognostic value of SVI measured with right heart catheterization (RHC) in patients at intermediate-risk. Methods: All treatment naïve patients with I/H-PAH, CTD-PAH and CHD-PAH referred to a single centre were included from 2003 to 2017. All patients were assessed at baseline and at the 1st follow-up at 3-4 months after starting PAH-specific therapy (1st F-UP) with RHC, brain natriuretic peptide (BNP) plasma levels, 6-min walking distance (6MWD) and WHO functional class. We applied a simplified risk assessment strategy using the following criteria: WHO functional class, 6MWD, right atrial pressure or BNP plasma levels and cardiac index (CI) or mixed venous oxygen saturation (SvO2). The last 2 criteria were based on which parameter was available; if both were available the worst was chosen. Risk strata were defined as: Low risk= at least 3 low risk and no high-risk criteria; High risk= at least 2 high risk criteria including CI or SvO2; Intermediate risk= definitions of low or high risk not fulfilled. The prognostic value of SVI was assessed using Cox regression analysis. Intermediate risk patients were further stratified in intermediate-low and intermediate-high risk taking into account the value of SVI that best discriminate prognosis (according to ROC curve analysis). Kaplan Meier curves and Log-rank test were used for survival analysis. Results: Seven hundred and twenty-five patients were enrolled. SVI is able to stratify the prognosis of PAH patients at 1st F-UP [HR 0.979 (0.964-0.994), p-value= 0.008] but not at baseline [HR 0.986 (0.970-1.002), p-value= 0.085]. The best predictive cut-off value is 38 ml/m2 (AUC= 0.66, sensitivity= 73%, specificity= 59%). Survival curves are shown in the Figure. Conclusions: SVI assessed at 1st F-UP is predictive of prognosis and the cut off value of 38 ml/m2 is able to further stratify the survival of intermediate risk PAH patients. 22 Short term effect of Selexipag in pulmonary arterial hypertension patients started on double combination therapy with ERA and PDE-5 inhibitors Daniele Guarino Daniele Guarino DIMES: Department of Experimental, Diagnostic and Specialty Medicine Fabio Dardi Fabio Dardi DIMES: Department of Experimental, Diagnostic and Specialty Medicine Massimiliano Palazzini Massimiliano Palazzini DIMES: Department of Experimental, Diagnostic and Specialty Medicine Andrea Rinaldi Andrea Rinaldi DIMES: Department of Experimental, Diagnostic and Specialty Medicine Elisa Zuffa Elisa Zuffa DIMES: Department of Experimental, Diagnostic and Specialty Medicine Filippo Pasca Filippo Pasca DIMES: Department of Experimental, Diagnostic and Specialty Medicine Alessandro De Lorenzis Alessandro De Lorenzis DIMES: Department of Experimental, Diagnostic and Specialty Medicine Ilenia Magnani Ilenia Magnani DIMES: Department of Experimental, Diagnostic and Specialty Medicine Mariangela Rotunno Mariangela Rotunno DIMES: Department of Experimental, Diagnostic and Specialty Medicine Alberto Ballerini Alberto Ballerini DIMES: Department of Experimental, Diagnostic and Specialty Medicine Alessandra Manes Alessandra Manes DIMES: Department of Experimental, Diagnostic and Specialty Medicine Nazzareno Galiè Nazzareno Galiè DIMES: Department of Experimental, Diagnostic and Specialty Medicine DIMES: Department of Experimental, Diagnostic and Specialty Medicine Background: The event-driven, phase 3, randomized, double-blind, placebo-controlled GRIPHON trial demonstrated that Selexipag reduces the risk of a composite Endpoint of death or morbidity events in patients with pulmonary arterial hypertension (PAH). Purpose: To evaluate the effects of Selexipag in PAH patients initially started with double-combination therapy with an endothelin receptor antagonist (ERA) and a phosphodiesterase-5 inhibitor (PDE5-I). Methods: We enrolled patients on double combination therapy with ERA + PDE5-I. Selexipag was weekly uptitrated to the maximum tolerated dose or to 1600 μg twice daily. All patients were systematically assessed with WHO-functional class (FC), brain natriuretic peptide (BNP), six minute walk test (6MWT) and right heart catheterization before treatment and 3 months after reaching a stable dose of Selexipag. Results: eleven patients with idiopathic/heritable PAH, 5 patients with connective tissue disease associated PAH and 2 patients with congenital heart disease associated PAH underwent a complete revaluation 6 (5-8) months after starting selexipag. The median dose was 1600 (range 1200-1600) mcg b.i.d. Results are shown in the Table. Legend: CI, cardiac index; mPAP, mean pulmonary arterial pressure; PVR, pulmonary vascular resistance; RAP, right atrial pressure; SvO2, mixed venous oxygen saturation. Conclusions: Selexipag improves haemodynamic profile in patients with PAH already on double combination therapy. This may explain the long term beneficial effect of the drug on the outcome of these patients. median (interquartile range) . Pre Selexipag . Post Selexipag . p-value . WHO-FC III/IV (%) 50 39 0.157 6MWT (m) 456 (300-568) 475 (339-584) 0.313 BNP (pg/ml) 150 (63-449) 101 (46-202) 0.074 RAP (mmHg) 7 (6-10) 6 (4-9) 0.096 mPAP (mmHg) 55 (49-63) 47 (41-54) 0.011 CI (l/min/m2) 2.5 (2.1-2.8) 2.9 (2.4-3.3) <0.001 PVR (WU) 11 (8-13) 7 (6-10) 0.001 SvO2 (%) 67 (60-70) 66 (62-71) 0.337 median (interquartile range) . Pre Selexipag . Post Selexipag . p-value . WHO-FC III/IV (%) 50 39 0.157 6MWT (m) 456 (300-568) 475 (339-584) 0.313 BNP (pg/ml) 150 (63-449) 101 (46-202) 0.074 RAP (mmHg) 7 (6-10) 6 (4-9) 0.096 mPAP (mmHg) 55 (49-63) 47 (41-54) 0.011 CI (l/min/m2) 2.5 (2.1-2.8) 2.9 (2.4-3.3) <0.001 PVR (WU) 11 (8-13) 7 (6-10) 0.001 SvO2 (%) 67 (60-70) 66 (62-71) 0.337 Open in new tab median (interquartile range) . Pre Selexipag . Post Selexipag . p-value . WHO-FC III/IV (%) 50 39 0.157 6MWT (m) 456 (300-568) 475 (339-584) 0.313 BNP (pg/ml) 150 (63-449) 101 (46-202) 0.074 RAP (mmHg) 7 (6-10) 6 (4-9) 0.096 mPAP (mmHg) 55 (49-63) 47 (41-54) 0.011 CI (l/min/m2) 2.5 (2.1-2.8) 2.9 (2.4-3.3) <0.001 PVR (WU) 11 (8-13) 7 (6-10) 0.001 SvO2 (%) 67 (60-70) 66 (62-71) 0.337 median (interquartile range) . Pre Selexipag . Post Selexipag . p-value . WHO-FC III/IV (%) 50 39 0.157 6MWT (m) 456 (300-568) 475 (339-584) 0.313 BNP (pg/ml) 150 (63-449) 101 (46-202) 0.074 RAP (mmHg) 7 (6-10) 6 (4-9) 0.096 mPAP (mmHg) 55 (49-63) 47 (41-54) 0.011 CI (l/min/m2) 2.5 (2.1-2.8) 2.9 (2.4-3.3) <0.001 PVR (WU) 11 (8-13) 7 (6-10) 0.001 SvO2 (%) 67 (60-70) 66 (62-71) 0.337 Open in new tab 23 Prognostic role of laboratory parameters in patients with pulmonary arterial hypertension Alberto Ballerini Alberto Ballerini DIMES: Department of Experimental, Diagnostic and Specialty Medicine Elisa Zuffa Elisa Zuffa DIMES: Department of Experimental, Diagnostic and Specialty Medicine Fabio Dardi Fabio Dardi DIMES: Department of Experimental, Diagnostic and Specialty Medicine Giacomo Pretolani Giacomo Pretolani DIMES: Department of Experimental, Diagnostic and Specialty Medicine Massimiliano Palazzini Massimiliano Palazzini DIMES: Department of Experimental, Diagnostic and Specialty Medicine Andrea Rinaldi Andrea Rinaldi DIMES: Department of Experimental, Diagnostic and Specialty Medicine Filippo Pasca Filippo Pasca DIMES: Department of Experimental, Diagnostic and Specialty Medicine Alessandro De Lorenzis Alessandro De Lorenzis DIMES: Department of Experimental, Diagnostic and Specialty Medicine Daniele Guarino Daniele Guarino DIMES: Department of Experimental, Diagnostic and Specialty Medicine Ilenia Magnani Ilenia Magnani DIMES: Department of Experimental, Diagnostic and Specialty Medicine Mariangela Rotunno Mariangela Rotunno DIMES: Department of Experimental, Diagnostic and Specialty Medicine Alessandra Manes Alessandra Manes DIMES: Department of Experimental, Diagnostic and Specialty Medicine Nazzareno Galié Nazzareno Galié DIMES: Department of Experimental, Diagnostic and Specialty Medicine DIMES: Department of Experimental, Diagnostic and Specialty Medicine Background: Current European pulmonary hypertension (PH) guidelines stratify the risk of patients with pulmonary arterial hypertension (PAH) using a multiparametric approach and define low, intermediate and high-risk according to the estimated 1-year mortality (respectively <5%, 5-10% and >10%). The risk stratification table includes clinical, functional, exercise, haemodynamic and echocardiographic parameters; the only laboratory parameter included is brain natriuretic peptide (BNP)/NT-proBNP but it is unknown if other laboratory evaluations are important in PAH risk stratification. Purpose: To evaluate the prognostic value of laboratory parameters in patients with idiopathic, heritable and drug-induced (I/H/D)-PAH. Methods: All patients with I/H/D-PAH referred to a single centre were included from 2002 to December 2018. All patients were assessed at baseline with right heart catheterization, BNP plasma levels, 6-min walking distance (6MWD), WHO functional class (FC) and complete laboratory evaluation (assessing, in particular, coagulation, inflammation, auto-antibodies, immunofixation, renal and hepatic function, complete blood count, B and C hepatitis virus profile, lipid profile, thyroid function, iron status and electrolytes, angiotensin converting enzyme, creatine phosphokinase, homocysteine, lactate dehydrogenase levels). All patients were treated according to current guidelines. The prognostic value of each variable was assessed using Cox regression model and a p-value <0.1 was consider for inclusion in the multivariate analysis. Results: Four hundred and seventy-six patients were included (median age 48 years, 60% female, 65% WHO-FC III-IV, median 6MWD 413 m, median right atrial pressure/mean pulmonary artery pressure/cardiac index/pulmonary vascular resistance (PVR)/mixed venous oxygen saturation were, respectively, 7 mmHg/52 mmHg/2.4 l/min/m2/11 WU/64%). The independent predictors of all-cause death at the multivariate analysis were: age (HR 1.042, 95% CI 1.027-1.059, p-value <0.001), male gender (HR 3.165, 95% CI 2.060-4.862, p-value <0.001), BNP (HR 2.008, 95% CI 1.478-2.728, p-value <0.001), PVR (HR 1.03, 95% CI 1.000-1.063, p-value 0.047) and serum sodium levels (HR 0.928, 95% CI 0.863-0.997, p-value 0.043). Conclusions: Our study confirm Literature data according to which age, male gender, BNP levels and PVR are well defined determinants of PAH prognosis. Among laboratory parameters we found that serum sodium level is an independent predictor of death. This underline a parallelism with other organ insufficiencies, such as cirrhosis and left heart failure, in which hyponatremia reflects the neuro-hormonal activation associated with reduced effective volemia that, in patients with PAH, is indicative of a severe disease with peripheral and splanchnic hypoperfusion. 26 Prognostic role of comorbidities in patients with pulmonary arterial hypertension Alessandro De Lorenzis Alessandro De Lorenzis DIMES: Department of Experimental, Diagnostic and Specialty Medicine Fabio Dardi Fabio Dardi DIMES: Department of Experimental, Diagnostic and Specialty Medicine Riccardo Bertozzi Riccardo Bertozzi DIMES: Department of Experimental, Diagnostic and Specialty Medicine Massimiliano Palazzini Massimiliano Palazzini DIMES: Department of Experimental, Diagnostic and Specialty Medicine Andrea Rinaldi Andrea Rinaldi DIMES: Department of Experimental, Diagnostic and Specialty Medicine Filippo Pasca Filippo Pasca DIMES: Department of Experimental, Diagnostic and Specialty Medicine Elisa Zuffa Elisa Zuffa DIMES: Department of Experimental, Diagnostic and Specialty Medicine Daniele Guarino Daniele Guarino DIMES: Department of Experimental, Diagnostic and Specialty Medicine Ilenia Magnani Ilenia Magnani DIMES: Department of Experimental, Diagnostic and Specialty Medicine Mariangela Rotunno Mariangela Rotunno DIMES: Department of Experimental, Diagnostic and Specialty Medicine Alberto Ballerini Alberto Ballerini DIMES: Department of Experimental, Diagnostic and Specialty Medicine Alessandra Manes Alessandra Manes DIMES: Department of Experimental, Diagnostic and Specialty Medicine Nazzareno Galiè Nazzareno Galiè DIMES: Department of Experimental, Diagnostic and Specialty Medicine DIMES: Department of Experimental, Diagnostic and Specialty Medicine Background: Epidemiology of pulmonary arterial hypertension (PAH), as outlined by the most important registries, is changing; in particular the age at diagnosis is increasing. This means that comorbidities are becoming more frequent. Anyway, whether they influence the prognosis or not is substantially undefined. Purpose: To evaluate the prognostic value of comorbidities in patients with PAH and in the different clinical subgroups. Methods: All patients with PAH referred to a single centre were included from 1977 to december 2018. All patients were assessed at baseline with right heart catheterization, brain natriuretic peptide (BNP) plasma levels, 6-min walking distance (6MWD), WHO functional class (FC) and anamnestic comorbidities [in particular: thyroid disorders, diabetes, dyslipidemia, smoke, chronic obstructive pulmonary disease, obstructive sleep apnea syndrome, systemic hypertension, coronary artery disease, previous pulmonary embolism, chronic kidney disease, anaemia, body mass index (BMI), arrhythmia and cancer]. All patients were treated according to current guidelines. The prognostic value of each variable was assessed using Cox regression model and a p-value <0.1 was consider for inclusion in the multivariate analysis. Results: Thirteen hundred and eleven patients were included [median age 51 years, 63% female; etiology: 522 idiopathic/heritable/drug-induced (I/H/D)-PAH, 258 connective tissue disease (CTD)-associated PAH, 242 congenital heart disease (CHD)-associated PAH, 196 portal hypertension/HIV (PoHIV)-associated PAH and 93 pulmonary veno-occlusive disease (PVOD)]. Only 5% of patients have no comorbidities. At multivariate analysis comorbidities independently associated with prognosis are: systemic hypertension in I/H/D [HR 0.616 (0.397-0.955), p = 0.030], mean systemic blood pressure in CTD [HR 0.980 (0.968-0.993), p = 0.002] and PVOD [HR 0.962 (0. 936-0.989), p = 0.006], dyslipidemia in CTD [HR 0.447 (0.283-0.707), p = 0.001] and PoHIV [HR 0.201 (0.049-0.824), p = 0.026], estimated glomerular filtration rate in PoHIV [HR 1.000 (0.999-1.000), p < 0.001] and BMI [HR 0.966 (0.930-1.003), p = 0.069] in CTD. In CHD comorbidities are not independent predictors of prognosis. Advance age is an independent risk factor in all PAH subgroups (except PVOD, maybe because their age is usually advanced) and male gender is an independent risk factor in I/H/D. 6MWD is an independent predictor of prognosis in I/H/D [HR 0.995 (0.993-0.997), p < 0.001], CTD [HR 0.997 (0.996-0.998), p < 0.001] and PVOD [HR 0.994 (0.991-0.997), p < 0.001]. BNP is an independent predictor of death in I/H/D [HR 1.525 (1.149-2.025), p = 0.004], CHD [HR 1.865 (1.097-3.170), p = 0.021] and PVOD [HR 1.628 (0.928-2.856), p = 0.089]. Haemodynamic independent predictors of prognosis are cardiac index in CTD [HR 0.555 (0.406-0.757), p < 0.001], right atrial pressure in I/H/D [HR 1.053 (1.003-1.105), p = 0.039] and mixed venous oxygen saturation in CHD [HR 0.964 (0.933-0.996), p = 0.029]. Conclusions:Our study confirm Literature data according to which the mean age at PAH diagnosis and the prevalence of comorbidities are increasing. Anyway, their prognostic role seems of poor relevance. As a matter of fact, we found a protective role of these variables: high systemic blood pressure (maybe indicative of a better haemodynamic stability) is protective in I/H/D, CTD and PVOD; dyslipidemia and high BMI (maybe indicative of a better nutritional status and a less severe autoimmune disease) are protective in CTD; dyslipidemia and a high glomerular filtration rate (both indicative of a less severe hepatic disease) are protective in PoHIV. 28 Prognostic value of pulmonary artery diameter in patients with pulmonary arterial hypertension Ilenia Magnani Ilenia Magnani DIMES: Department of Experimental, Diagnostic and Specialty Medicine Fabio Dardi Fabio Dardi DIMES: Department of Experimental, Diagnostic and Specialty Medicine Federico Donato Federico Donato DIMES: Department of Experimental, Diagnostic and Specialty Medicine Massimiliano Palazzini Massimiliano Palazzini DIMES: Department of Experimental, Diagnostic and Specialty Medicine Andrea Rinaldi Andrea Rinaldi DIMES: Department of Experimental, Diagnostic and Specialty Medicine Elisa Zuffa Elisa Zuffa DIMES: Department of Experimental, Diagnostic and Specialty Medicine Filippo Pasca Filippo Pasca DIMES: Department of Experimental, Diagnostic and Specialty Medicine Alessandro De Lorenzis Alessandro De Lorenzis DIMES: Department of Experimental, Diagnostic and Specialty Medicine Daniele Guarino Daniele Guarino DIMES: Department of Experimental, Diagnostic and Specialty Medicine Mariangela Rotunno Mariangela Rotunno DIMES: Department of Experimental, Diagnostic and Specialty Medicine Alberto Ballerini Alberto Ballerini DIMES: Department of Experimental, Diagnostic and Specialty Medicine Alessandra Manes Alessandra Manes DIMES: Department of Experimental, Diagnostic and Specialty Medicine Nazzareno Galiè Nazzareno Galiè DIMES: Department of Experimental, Diagnostic and Specialty Medicine DIMES: Department of Experimental, Diagnostic and Specialty Medicine Background: Pulmonary artery (PA) dilation is common in pulmonary arterial hypertension (PAH) but its prognostic role has been poorly evaluated even if it can cause a poli-compressive syndrome including left main coronary artery (LMCA) compression, a potential cause of sudden cardiac death. The prevalence and treatment strategies of LMCA compression in PAH patients symptomatic for angina have been recently evaluated, anyway little is known about asymptomatic patients. Purpose: To evaluate the prognostic value of PA diameter (at baseline and at last radiological evaluation) in patients with PAH and the prevalence of LMCA compression in patients both symptomatic and asymptomatic for angina. Methods: All patients with PAH referred to a single centre that underwent a baseline angio-CT scan evaluation were included from 2000 to December 2018. If an angio-CT scan was repeated during the follow-up the last available PA diameter was considered to calculate the velocity of PA diameter increase. The prognostic value of each variable was assessed using Cox regression model. Patients that during follow-up complained of angina or developed radiological CT scan signs of suspicion for LMCA compression underwent a coronary (cor)-CT scan. Patients with a not negative cor-CT, as described by Galiè et al., underwent an invasive coronary angiography. In case of acute coronary syndrome, known coronary artery disease, very high cardiovascular risk at baseline assessment or indication for invasive surgical procedure an invasive coronary angiography was performed without a preliminary cor-CT. To evaluate the predictivity for compression at coronary angiography the PA diameter at cor-CT or at the nearest angio-CT scan was considered. Results: Nine hundred and fifty-eight patients were included (median age 52 years, 66% female). PA diameter at baseline is higher in patients with congenital heart disease (CHD) and is not predictive of death neither in the overall population nor in the different etiological subgroups. The velocity of PA diameter increase is lower in patients with connective tissue disease (CTD) and is a risk factor of death in both the overall population and in the different etiological subgroups except CHD and pulmonary veno-occlusive disease (PVOD). During the follow-up 180 patients complained of angina and underwent cor-CT and/or coronary angiography and 50 patients had LMCA compression treated with coronary stenting. Among the 778 asymptomatic patients 120 underwent cor-CT and/or coronary angiography and 15 patients had LMCA compression treated with coronary stenting. The best cut-offs predicting LMCA compression in symptomatic/asymptomatic patients were, respectively, a PA diameter of 40/42 mm (sensitivity and specificity, respectively, 80 and 72%/87 and 77%) and a PA/Aorta ratio of 1.5/1.4 (sensitivity and specificity, respectively, 78 and 72%/80 and 71%). Conclusions: 28% of patients with angina have LMCA compression anyway also a not negligible percentage of asymptomatic patients (13%) in our study have a critical LMCA stenosis at coronary angiography. Despite a not systematic study we found a similar PA diameter predicting LMCA compression in symptomatic and asymptomatic patients. PA diameter, instead, is not predictive of prognosis and this can be due to the fact that most deaths in patients with PAH are related to heart failure and that patients with a significant LMCA stenosis are treated with stenting, maybe preventing sudden cardiac death. Eventually, PA diameter velocity increase is associated with prognosis anyway this may simply reflect a more severe disease refractory to medical therapy. 453 Pre-capillary pulmonary hypertension in sarcoidosis patient Laura De Michieli Laura De Michieli Department of Cardiac-Thoracic-Vascular Sciences and Public Health, University of Padua, Italy Lodovica Videsott Lodovica Videsott Department of Cardiac-Thoracic-Vascular Sciences and Public Health, University of Padua, Italy Andrea Candelora Andrea Candelora Department of Cardiac-Thoracic-Vascular Sciences and Public Health, University of Padua, Italy Monica Loy Monica Loy Dipartimento di Scienze Cardio-Toraco-Vascolari, Azienda Ospedaliera di Padova, Italia Elisabetta Balestro Elisabetta Balestro Dipartimento di Scienze Cardio-Toraco-Vascolari, Azienda Ospedaliera di Padova, Italia Manuel De Lazzari Manuel De Lazzari Dipartimento di Scienze Cardio-Toraco-Vascolari, Azienda Ospedaliera di Padova, Italia Francesco Tona Francesco Tona Department of Cardiac-Thoracic-Vascular Sciences and Public Health, University of Padua, Italy Sabino Iliceto Sabino Iliceto Department of Cardiac-Thoracic-Vascular Sciences and Public Health, University of Padua, Italy Martina Perazzolo Marra Martina Perazzolo Marra Department of Cardiac-Thoracic-Vascular Sciences and Public Health, University of Padua, Italy Giulia Famoso Giulia Famoso Dipartimento di Scienze Cardio-Toraco-Vascolari, Azienda Ospedaliera di Padova, Italia Dipartimento di Scienze Cardio-Toraco-Vascolari, Azienda Ospedaliera di Padova, Italia Department of Cardiac-Thoracic-Vascular Sciences and Public Health, University of Padua, Italy Clinical case Past History: male, 61 years old, pulmonary sarcoidosis diagnosed in 1987 after chest x-ray (histologic confirmation with transbronchial biopsy), moderate to severe restrictive respiratory deficit, pre-capillary pulmonary hypertension. GERD and chronic gastritis, OSAS treated with nocturnal oxygen therapy. Since 2007 he suffered from exercise-induced worsening dyspnoea; in 2013 underwent right heart catheterization with evidence of pre-capillary pulmonary hypertension; off-label bosentan therapy was started with first clinical improvement and subsequent clinical stabilization. In 2015, echocardiogram showed chronic cor pulmonale, mild right ventricle (RV) dilatation with normal systolic function, pulmonary hypertension (PAPs 52 mmHg). Because of worsening dyspnoea, present for mild efforts, and profound asthenia, he underwent a second right heart catheterization with evidence of normal pulmonary wedge pressure, mild pulmonary hypertension with severe increase of arteriolar resistance, normal cardiac index (CI 2.93 lit/min/mq). Combination therapy with bosentan and sildenafil (off-label) was started but the patient did not tolerate it because of important asthenia. Recent history. February 2018: he underwent bilateral pulmonary transplantation with ECMO support. He suffered from several episodes of atrial fibrillation during post-operative hospitalization. Echocardiogram: biventricular normal dimensions and function. In March 2018 severe desaturation due to right total pneumothorax, treated with drenaige. In May 2018: cardiac arrest due to massive pulmonary embolism and, thereafter, left arm compartment syndrome treated with surgical fasciectomy. To date, the patient is in fair clinical condition and hemodynamic compensation; he suffers from reduced effort tolerance due to muscular hypotrophy secondary to the cardiac arrest hospitalization. Discussion: Pulmonary sarcoidosis is an uncommon cause of pre-capillary pulmonary hypertension. Our patient was treated off-label with bosentan first and bosentan plus sildenafil after; which would be the optimal medical therapy in this kind of patients? Are indication and timing to pulmonary transplantation acceptable? Which could be the cause and the appropriate therapy for pulmonary embolism in this patient? This case could lead to substantial discussion and confrontation. 549 Efficacy of rivaroxaban treatment of an extended deep vein thrombosis with pulmonary embolism in an antithrombin iii deficient patient Marianna Appignani Marianna Appignani Intensive Cardiac Care Unit, “SS. Annunziata” Hospital – Chieti Adolfo Sciartilli Adolfo Sciartilli Intensive Cardiac Care Unit, “SS. Annunziata” Hospital – Chieti Nicoletta Adezio Nicoletta Adezio Intensive Cardiac Care Unit, “SS. Annunziata” Hospital – Chieti Antonino Scarinci Antonino Scarinci Intensive Cardiac Care Unit, “SS. Annunziata” Hospital – Chieti Tanya Salvatore Tanya Salvatore School of Cardiology, “G. D’Annunzio” University – Chieti Marcello Caputo Marcello Caputo Intensive Cardiac Care Unit, “SS. Annunziata” Hospital – Chieti Enrico Di Girolamo Enrico Di Girolamo Arrhythmology Unit, “SS. Annunziata” Hospital - Chieti Intensive Cardiac Care Unit, “SS. Annunziata” Hospital – Chieti School of Cardiology, “G. D’Annunzio” University – Chieti Arrhythmology Unit, “SS. Annunziata” Hospital - Chieti Background: Patients with low Antithrombin III (AT III) have an increased risk of artero-venous thromboembolic (TE) disease and heparin resistance. Case Report: We report a 28-year-old woman presenting with spontaneous right calf pain and swelling lasting about one week. A heterozygous AT III deficiency with phenotype expression of deep vein thrombosis also in fist-degree relatives (mother and sister) was reported. Thrombophilia study showed residual AT III activity of 58% and normal protein C and protein S laboratory samples. Autoimmune essays were negative. The computed tomographic angiography (CTA) that revealed a bilateral subsegmental pulmonary embolism (Fig. 1)and a deep vein thrombosis (right leg) extended into the inferior vena cava up to the confluence of the left renal vein. Echocardiography showed an extended thrombosis within the inferior vena Cava (Fig. 2) Thrombolytic therapy, haemodynamic or ventilator support, or placement of an inferior vena cava filter were excluded and, given the hemodynamic stability, anticoagulant therapy with Rivaroxaban 15 mg bid was started. After 10 days of treatment with Rivaroxaban, the echocardiographic examination (Fig. 3) revealed the complete resolution of the thrombus located in the inferior vena cava and the CTA (Fig. 4) documented the complete resolution of the pulmonary embolism. Discussion and Conclusions: Patients with AT III deficiency could be heparin-resistant and may need for higher heparin doses or AT III replacement therapy, with an increased hemorrhagic risk. Direct Factor Xa inhibition by Rivaroxaban allowed to obtain an anticoagulant effect through an alternative mechanism, particularly useful in this patient with familial AT III deficiency, deep vein thrombosis and pulmonary embolism. 239 Pulmonary hypertension and right ventricular functional reserve: the additional information provided by low dose dobutamine during right heart catheterization and transthoracic echocardiography Giuseppe Esposito Giuseppe Esposito Cardiologia Ospedaliera-Policlinico di Bari Fabrizio Fumarola Fabrizio Fumarola Cardiologia Ospedaliera-Policlinico di Bari Tiziana Leopizzi Tiziana Leopizzi Cardiologia Ospedaliera-Policlinico di Bari Nino Camassa Nino Camassa Cardiologia Ospedaliera-Policlinico di Bari Antonia Mannarini Antonia Mannarini Cardiologia Ospedaliera-Policlinico di Bari Elisabetta De Tommasi Elisabetta De Tommasi Cardiologia Ospedaliera-Policlinico di Bari Lucrezia De Michele Lucrezia De Michele Cardiologia Ospedaliera-Policlinico di Bari Paolo Colonna Paolo Colonna Cardiologia Ospedaliera-Policlinico di Bari Carlo D´agostino Carlo D´agostino Cardiologia Ospedaliera-Policlinico di Bari Cardiologia Ospedaliera-Policlinico di Bari Background: Individuals with pulmonary arterial hypertension (PAH) have reduced resistance to physical exercise proportionate to the degree of impairment of the heart-lung system. In the current guidelines the evaluation of the right heart function as a prognostic index is not codified, therefore our group has started to show interest in this field of research. Aim: We hypothesized that the basal alterations in the right cardiac function and in the pulmonary circulation are not sufficient to correctly stratify the patients affected by PAH; we therefore assumed that the evaluation of the hemodynamic and echocardiographic response to pharmacological stress could better identify patients who, given the same baseline values, show an absence of functional reserve of the right heart and pulmonary circulation. Probably this patient setting, could have a worse prognosis. Materials and Methods: We enrolled 23 patients with PAH diagnosis belonging to group 1. They underwent right heart catheterization (RHC) and simultaneously transthoracic echocardiography (ETT) at rest and during pharmacological stress with low-dose dobutamine (BD) (10 mcg / kg / min). The study population was divided into three groups based on baseline cardiac index (CI) and mean pulmonary artery pressure (PaPm) values (Table 1); the main hemodynamic and echocardiographic parameters were collected together with hematochemical (NT-ProBNP), functional (six minute walking test) and echocardiographic (right atrial area) indices currently used to estimate mortality at one year. Subsequently, an analysis of the variance with repeated measures (ANOVA Repeated Measure) (Table 1) and of multiple linear regression models was carried out to verify the existence of correlations between the hemodynamic, echocardiographic and clinical-laboratory indicators. Results: Baseline and stress values of TAPSE and S’TDI were significantly reduced in subjects belonging to group 3 (basal vs dobutamine: TAPSE p-value = 0.003 *; S’TDI p-value = 0.011 *). Furthermore, exclusively for TAPSE, this reduction is also significant in the comparison between groups regardless of pharmacological stress (between subject: TAPSE p-value = 0.009 *). Analogous to the echocardiographic indexes of right cardiac function also the pulmonary resistances (PVR) were significantly increased both in comparison between basal values and during stress (basal vs dobutamine: PVR p-value = 0.009 *), both in comparison to groups (between subject: PVR p-value = 0.015 *). The Tukey Hsd Test showed a statistically difference between the subjects of group 3 and the remaining two groups (Table 2). Conclusion: The use of pharmacological stress in patients with PAH belonging to group 1 during right cardiac catheterization, is a safe test that can evaluate the functional reserve of the pulmonary circulation and the right heart. In this study, patients belonging to group 3 show a reduced functional reserve (higher PVR, lower TAPSE and lower S’ TDI) during dobutamine BD. Evaluation of hemodynamic parameters and echocardiographic indexes during pharmacological stress could be effective in identifying the most compromised patients and with a higher risk profile. Further studies are needed to validate these preliminary data and to better define the prognostic role of pharmacological stress in this patient setting. 448 Clinically diagnosis of right ventricle arrhytmogenic cardiomyopathy associate with an unreported gene mutation Paola Genoni Paola Genoni Dipartimento Cardiovascolare Ospedale di Circolo - Universita’ dell’Insubria, Varese Antonella Mancinelli Antonella Mancinelli Dipartimento Cardiovascolare Ospedale di Circolo - Universita’ dell’Insubria, Varese Matteo Crippa Matteo Crippa Dipartimento Cardiovascolare Ospedale di Circolo - Universita’ dell’Insubria, Varese Raffaella Marazzi Raffaella Marazzi Dipartimento Cardiovascolare Ospedale di Circolo - Universita’ dell’Insubria, Varese Roberto De Ponti Roberto De Ponti Dipartimento Cardiovascolare Ospedale di Circolo - Universita’ dell’Insubria, Varese Dipartimento Cardiovascolare Ospedale di Circolo - Universita’ dell’Insubria, Varese Right ventricle arrhythmogenic cardiomyopathy (ARVC) is a chronic, progressive, heritable cardiomyopathy; genes associated to ARVC predominantly encode desmosomal proteins. More genes have been reported to be associated with ARVC, but the frequencies of gene variations in these genes are unknown. ARVC is one of the leading causes of sudden unexpected cardiac death in young, apparently healthy individuals. Prevalence is estimated to be 1: 1000-1: 5000. We report a case of hyperlipidemic 42-year-old man, admitted to our emergency department the day after a prolonged episode of palpitations. He was known to have suffered from Guillaume-Barrè syndrome, gastro oesophageal reflux disease and vitiligo; he has two sisters with BRCA mutations and a young nephew known for cardiac arrest in ARVC. At the admission, electrocardiogram showed negative T waves in V4-V6 and inferior leads. Troponin T was elevated and transthoracic echocardiogram (TTE) showed inferior-lateral kinetic abnormalities. DAPT, anticoagulant, beta-blocker and statin were administered, and coronary angiography was performed showing right coronary arteriovenous fistula. During hospitalization ventricular tachycardia (178 b.p.m., left branch block and superior axial deviation) was observed. Amiodarone infusion has been effective to stop arrhythmia. Electrophysiological study was then performed and showed inducibility of a non-sustain form of clinical tachycardia (maximum run of three beats) by programmed ventricular stimulation with dual extrastimuli from the right apex. Electroanatomic mapping of right ventricle was performed and low-potential areas were detected in the infundibulum and in the anterolateral peritricuspid region; moreover, late potentials were recorded in a limited region of the anterior infundibulum of the right ventricle. Another TTE was performed to analyze right chambers and it revealed only basal wall slight hypertrapping and two middle-distal recesses, split from the hyperechoic moderator band. Cardiac magnetic resonance confirmed compatible pattern with ARVC. Bicameral ICD was implanted. Molecular evaluation of genes related to ARVC (DSC2, DSG2, DSP, PKP2, JUP, TMEM43, RYR2, PLN) was performed. The molecular analysis of the entire coding region of PKP2 gene has detected the nucleotide substitution c.2182G> T (NM_004572.3) in heterozygous exon 11; it codes for a premature stop codon p.Glu728X (chr12: g.32955454> A, GRCh37 / hg19). No other mutations were identified. One of the two sisters had the same mutation, which had never been associated with ARVC. Nevertheless, clinical presentation, imaging morphology and familiar anamnesis suggest a possible relation to ARVC. 146 A case of platypnea-orthodeoxia syndrome: diagnosis by exclusion or something to look for? Michele Golino Michele Golino Dipartimento Cardiovascolare, Ospedale di Circolo—Università dell’Insubria—Varese Giulia Damiani Giulia Damiani Dipartimento Cardiovascolare, Ospedale di Circolo—Università dell’Insubria—Varese Francesca Ceriani Francesca Ceriani Dipartimento Cardiovascolare, Ospedale di Circolo—Università dell’Insubria—Varese Mariangela Lattanzio Mariangela Lattanzio Dipartimento Cardiovascolare, Ospedale di Circolo—Università dell’Insubria—Varese Sergio Ghiringhelli Sergio Ghiringhelli Dipartimento Cardiovascolare, Ospedale di Circolo—Università dell’Insubria—Varese Roberto De Ponti Roberto De Ponti Dipartimento Cardiovascolare, Ospedale di Circolo—Università dell’Insubria—Varese Dipartimento Cardiovascolare, Ospedale di Circolo—Università dell’Insubria—Varese Initial Presentation: A 60-year-old man was admitted to the hospital with worsening of chronic hypoxemia in a known obstructive and restrictive pulmonary disease, apparently not justified by the underlying pulmonary disease. His vital signs were normal except for arterial oxygen saturation (SO2) of 75% on room air while seated. Transthoracic echocardiogram (TTE) didn’t reveal intracardiac shunts and chest-CT scan showed no evidence of pulmonary thromboembolism nor aortic dissection. Diagnosis and Management: Patient’s arterial blood gas tests demonstrated a significant drop in arterial oxygen partial pressure (pO2) from clinosatism to orthostatism, confirming the suspicion of Platypnea-Orthodeoxia Syndrome (POS). In the absence of heart and lung failure, a right-to-left shunting was suspected as the cause of this clinical condition. Transesophageal echocardiography (TEE) revealed the presence of a Patent Foramen Ovale (PFO) with right-to-left shunt through two sites in the centre and in the lower part of the foramen ovale. This shunt, due to the change in the anatomic relationship between the atrial septum and inferior vena cava (IVC), occurred only while the patient was sitting or standing. In fact, in this position the dilated aortic root deformed the atrial septum and the right atrium, so that the blood coming from the IVC was directed to the PFO. Cardiac catheterization showed a reduced right cardiac output (pulmonary blood flow= 3.34 l/min; 1.62 l/min/mq; systemic blood flow= 3.69 l/min; 1.8 l/min/mq; Qp/Qs= 0.9) and the absence of pulmonary hypertension (mean right atrial pressure 5 mmHg and mean pulmonary pressure 12 mmHg). Oximetry during catheterization showed significant SO2 decrease in the aorta (86%) compared to pulmonary veins (98.4%) and a temporary PFO closure by balloon inflation showed an immediate improvement of the SO2 in the aorta. Thus, it was decided to close the PFO using a 35/25-mm Amplatzer PFO-occluder device (Figure 1a and 1 b) with immediate cessation of the episodes of hypoxemia, and SO2 in sitting position increased from 78% to 97% without any residual shunts. At the 6-month follow-up, the patient was free of symptoms with no cyanosis and normal SO2 in every position. Conclusions: This case showed a rare clinical entity known as POS. In our case, the aortic dilatation changed the anatomic relationship between the atrial septum and the inferior vena cava, facilitating desaturated blood flow redirection through the PFO. The gold standard for the diagnosis is right and left cardiac catheterization, which shows a mismatch in oxygen saturation between the pulmonary veins and the aorta. Additionally, less invasive exams, like contrast echocardiography or thoracic computer tomography, can be performed to reach the same diagnosis. In conclusion we recognize that POS is not a very common disease and for this reason it can be under-diagnosed. In the presence of position-dependent hypoxemia, it is mandatory to consider this diagnosis and to exclude other causes of hypoxemia. 328 Potential extended role of ultrasound-enhanced catheter-directed thrombolysis in intermediate-high-risk acute pulmonary embolism Massimo Slavich Massimo Slavich Clinical Cardiology Unit, San Raffaele Scientific Institute, Milan, Italy Mario Gramegna Mario Gramegna Clinical Cardiology Unit, San Raffaele Scientific Institute, Milan, Italy Antonio Boccellino Antonio Boccellino Clinical Cardiology Unit, San Raffaele Scientific Institute, Milan, Italy Davide Margonato Davide Margonato Clinical Cardiology Unit, San Raffaele Scientific Institute, Milan, Italy Mario D´Egidio Mario D´Egidio Clinical Cardiology Unit, San Raffaele Scientific Institute, Milan, Italy Michele Oppizzi Michele Oppizzi Clinical Cardiology Unit, San Raffaele Scientific Institute, Milan, Italy Roberto Spoladore Roberto Spoladore Clinical Cardiology Unit, San Raffaele Scientific Institute, Milan, Italy Cosmo Godino Cosmo Godino Clinical Cardiology Unit, San Raffaele Scientific Institute, Milan, Italy Gabriele Fragasso Gabriele Fragasso Clinical Cardiology Unit, San Raffaele Scientific Institute, Milan, Italy Alberto Margonato Alberto Margonato Clinical Cardiology Unit, San Raffaele Scientific Institute, Milan, Italy Clinical Cardiology Unit, San Raffaele Scientific Institute, Milan, Italy Introduction: Pulmonary Embolism (PE) is a frequent clinical syndrome. Guidelines provide indications about acute-phase treatment according to patients’ risk class, but do not clearly indicate the role of ultrasound-enhanced catheter-directed thrombolysis (UCDT) in intermediate-risk classes. The purpose of this study is to evaluate the potential role of UCDT in the intermediate-risk classes. Materials and Methods: We evaluated 108 consecutive patients admitted with a diagnosis of acute PE to our cardiac intensive care unit (CCU) between April 2010 and June 2018. Patients’ clinical data, laboratory tests, CT-scan and echocardiographic reports were obtained from our Institute internal database. All patients were classified into early-mortality-risk classes according to guidelines, and the risk-adjusted therapeutic strategies were evaluated in terms of clinical and imaging outcomes. Results: General in-hospital mortality was 12% (N = 13). The highest mortality (40%, N = 10) occurred in the high-risk class (N = 25). In high-risk population (N = 23) the in-hospital mortality rate was 60% (N = 3) in patients that received UCDT (N = 5) and 71.4% (N = 5) in patients treated by anticoagulant therapy (N = 7). Among intermediate-high-risk patients (N = 39), in-hospital death occurred only among those treated by anticoagulant therapy; major bleeding complications occurred in 11.1% (N = 2) of patients treated by anticoagulant therapy (N = 18), in 6.7% (N = 1) of patients treated by UCDT (N = 15), in none of patients treated by low-dose thrombolysis (N = 2), and in 25% (N = 1) of patients treated by full-dose thrombolysis (N = 4). 93.3% (N = 14) of intermediate-high-risk patients treated by UCDT (N = 15) were discharged with TAPSE > 20. All patients at intermediate- low-risk treated by UCDT (N = 2) showed PASP ≤40 and TAPSE > 20 at discharge. Conclusion: The present retrospective analysis shows that UCDT could represent an alternative to full-dose systemic thrombolysis in compromised hemodynamically unstable patients with a high risk of bleeding and for whom the latter is contraindicated. Additionally, UCDT instead of anticoagulation alone appears safe and effective in intermediate-high risk patients and could have a role in intermediate-low-risk patients. 394 Pulmonary embolism: prognostic stratification of consecutive patients admitted to our cardiac care unit and cardiology departments Marco Luppi Marco Luppi U.O. di Cardiologia, Ospedale Sant’Anna, Asst Lariana, Como Dipartimento Cardiovascolare Ospedale di Circolo - Universita’ degli Studi dell’Insubria, Varese Carlo Campana Carlo Campana U.O. di Cardiologia, Ospedale Sant’Anna, Asst Lariana, Como Roberto De Ponti Roberto De Ponti Dipartimento Cardiovascolare Ospedale di Circolo - Universita’ degli Studi dell’Insubria, Varese Roberto Bonatti Roberto Bonatti U.O. di Cardiologia, Ospedale Sant’Anna, Asst Lariana, Como U.O. di Cardiologia, Ospedale Sant’Anna, Asst Lariana, Como Dipartimento Cardiovascolare Ospedale di Circolo - Universita’ degli Studi dell’Insubria, Varese Introduction: Pulmonary embolism (PE) is a potentially life-threatening disease, therefore risk stratification is mandatory to guide therapy. European Society of Cardiology (ESC) 2019 guidelines proposed a new risk-adjusted algorithm introducing in normotensive patients two validated scores, BOVA and modified FAST, to further stratify the severity of the acute PE. Aim: The aim of the study is to evaluate the 1-month EP-related mortality of patients admitted for PE in our Cardiac Care Unit and Cardiology Departments and to evaluate the performance of PESI, sPESI and ESC 2019 scores in all population and of BOVA and modified FAST scores in normotensive patients. Methods: Consecutive adult patients with a diagnosis of PE, admitted between 01 January 2015 and 01 August 2019 were retrospectively included. We recorded patient characteristics, diagnostic work-up and treatment, mortality during a 1-month follow-up, PESI and sPESI variables (age, gender, cancer, heart failure, chronic lung disease, pulse ≥ 110/’, systolic blood pressure < 100 mmHg, respiratory rate ≥ 30/’, temperature < 36 °C, altered mental status, arterial oxygen saturation < 90%), and other variables included in ESC 2019 prognostic algorithm (troponin, BNP, signs of RVD), in BOVA score (pulse ≥ 110/’, systolic blood pressure 90-100 mmHg, troponin) and in modified FAST score (pulse > 100/', troponin, syncope). Results: 194 patients were enrolled in this study. The mean age was 68 years (± SD 12); PE was unprovoked in 59% patients and cancer-related in 10.3% patients. Overall mortality rate were 5.1% (CI 95%, 2.8-9.2%) at 1 month; all deaths were EP-related. All high risk patients without contraindications to it were treated with systemic thrombolysis; 23% of intermediate-high risk patients considered by clinicians at lower risk of bleeding and at higher risk of early death received full-dose fibrinolysis, 6% of them low-dose fibrinolysis and 16% loco-regional ultrasound-assisted fibrinolysis. Conclusion: In this consecutive patients population, ESC 2019, PESI and sPESI scores confirmed their prognostic accuracy; all patients who died were classified at intermediate-high or high risk according to them. In normotensive patients BOVA score was able to predict 1 month EP-related mortality. 664 Role of an integrated non-invasive ultrasound imaging test for diagnosis of right-to-left shunt secondary to PFO Antonella Mancinelli Antonella Mancinelli Dipartimento Cardiovascolare Ospedale di Circolo - Università dell’Insubria, Varese Emilio Miglierina Emilio Miglierina Dipartimento Cardiovascolare Ospedale di Circolo - Università dell’Insubria, Varese Federica Matteo Federica Matteo Dipartimento Cardiovascolare Ospedale di Circolo - Università dell’Insubria, Varese Battistina Castiglioni Battistina Castiglioni Dipartimento Cardiovascolare Ospedale di Circolo - Università dell’Insubria, Varese Roberto De Ponti Roberto De Ponti Dipartimento Cardiovascolare Ospedale di Circolo - Università dell’Insubria, Varese Dipartimento Cardiovascolare Ospedale di Circolo - Università dell’Insubria, Varese Although patent foramen ovale (PFO) is present in asymptomatic subjects, previous data confirm that its presence associates with a three-fold increased risk of cerebral embolism. Moreover, ischaemic stroke, migraine with aura, decompression sickness and platipnea-ortodeoxia appear related to PFO. Purpose: The purpose of this study was to evaluate the role of an integrated, non-invasive ultrasound imaging test, performed in the same session, for diagnosis of right-to-left shunt secondary to PFO. Methods: 64 patients with history of cryptogenic cerebral ischaemic events documented on MRI (55 patients, 86%), diver decompression syndrome (3 patients, 5%), and persistent migraine with aura (6 patients, 9%) were evaluated by 1) trans-thoracic echocardiography (TTE) [Figure 1] and 2) trans-cranial ecocolorDoppler (TCCD) [Figure 2] with sampling of middle cerebral artery and basilar artery. Using TTE we studied: 1) atrial septum morphology, 2) presence of right-to-left shunts, before and after microbubble injection, at rest and after Valsalva maneuver, 3) site of microbubbles passage, 4) time of onset of microbubbles in the left atrium within three cardiac cycles from opacization of the right atrium. Using TCCD we evaluated: 1) presence of shunts, 2) Doppler signal disturbance duration, calculated from the first to the last recordable microembolic signal (MES). Feasibility of methods and concordance of results between the two exams were also evaluated. When discrepancy between the results were found, trans-oesophageal echocardiogram (TEE) was performed. Results: In all cases, TTE and basilar artery sampling were performed; mean cerebral artery sampling was feasible in 58 patients (90.6%). An excellent agreement between the two methods (k test with k > 0.80) to confirm and quantify the shunt was found. In thirty patients (46.8%), the evaluation was negative for right-to-left shunt using both methods, in thirty-one (48.4%), it was positive by both methods, while in three (4.8%) the two methods gave discordant results. The TEE was concordant with TTE in 2 of these 3 patients; in the third one it was concordant with TCCD. There was no statistically significant association (p = 0.831) between the extent of interatrial shunt and the presence of septal aneurysm. The MES had a longer duration in the TCCD with basilar artery sampling compared to TCCD with middle cerebral artery sampling (7065 ± 893 ms vs. 5922 ± 781 ms; p = 0.2), reducing the risk of underestimating the shunt. Conclusions: TTE, with the help of Doppler sampling of basilar and mean cerebral artery, performed in the same session, shows high value in terms of feasibility and accuracy to screen and evaluate patients with PFO. Therefore, an advanced approach with TEE could be limited to the small group of patients in whom a first-level imaging test shows discordant results. The sampling rate of basilar artery was higher than that of mean cerebral artery and it can be a valid alternative to estimate the shunt. 17 Long-term outcome and blood pressure profile in adult patients with repaired coarctation Fabio Marsico Fabio Marsico Adult Congenital Heart Disease, University Hospital of Bern Scienze Biomediche Avanzate, Federico II University, Naples Matthias Greutmann Matthias Greutmann Adult Congenital Heart Disease, University Hospital of Zurich Daniel Tobler Daniel Tobler Adult Congenital Heart Disease, University Hospital of Basel Fabienne Schwitz Fabienne Schwitz Adult Congenital Heart Disease, University Hospital of Bern Markus Schwerzmann Markus Schwerzmann Adult Congenital Heart Disease, University Hospital of Bern Kerstin Wustmann Kerstin Wustmann Adult Congenital Heart Disease, University Hospital of Bern Adult Congenital Heart Disease, University Hospital of Bern Scienze Biomediche Avanzate, Federico II University, Naples Adult Congenital Heart Disease, University Hospital of Zurich Adult Congenital Heart Disease, University Hospital of Basel Background: Aortic coarctation (CoA) is a congenital narrowing of the proximal descending aorta. Despite improvement in interventional techniques, prevalence of cardiovascular (CV) events and aortic re-interventions is high with an increased prevalence of arterial hypertension (aHT). The aim of this study is to assess the incidence of adverse CV events, defining outcome predictors, and to assess blood pressure (BP) profile in patients (Pts) with repaired CoA. Methods: We retrospectively evaluated adult Pts after CoA-repair by different techniques, who underwent clinical and imaging follow-up. Primary endpoint was defined as a composite of heart failure (HF), atrial fibrillation (AF), cerebrovascular events, coronary revascularization, aortic dissection, re-CoA requiring intervention and pseudoaneurysm formation. Residual aHT was defined as the presence of antihypertensive therapy, or a BP ≥ 140/90 mmHg. Nocturnal dipping was assessed by ambulatory-BP-monitoring (ABPM) (n = 141). Results: Overall, 281 Pts (112 females, 40%), age 33, 7 ± 13, 3 years were included in the study. After a mean Follow-up of 25, 6 ± 12, 7 years after first CoA repair, primary endpoint occurred in 134 Pts (48%): re-CoA requiring intervention (102; 36%), pseudoaneurysm formation (28; 10%) with 10 Pts (36% of Pts with pseudoaneurysm; 3, 6% of the whole population) requiring re-intervention, AF (20; 7%), cerebrovascular events (9; 3%), HF (5; 2%), coronary revascularization (4; 1, 4%), aortic dissection (3; 1%) and death (1; 0.4%). Residual aHT was present in 137 Pts (49%), and 135 Pts (48%) were treated with anti-hypertensive medications: mono-therapy in 38 Pts (28%), dual therapy in 54 Pts (40%), triple therapy in 32 Pts (23%) and 11 Pts are treated with four agents (8%). Impaired nocturnal BP response was found in 45 of 141 Pts (32%), and in 14 normotensive Pts (10%) in the 24-h ABPM. By Cox regression analysis, Pts treated by primary stenting procedure (HR 25.330, 95%CI 12.447-51.548, p < 0.001) and the presence of hypoplastic aortic arch (HR 1.518, 95%CI 1.067-2.158, p = 0.02) were predictors of the composite endpoint. At the same time, Pts with an hypoplastic aortic arch (HR 1.683, 95%CI 1.192-2.376, p = 0.003) and male Pts (HR 0.547, 95%CI 0.372-0.803, p = 0.002) are at higher risk for residual aHT as well as Pts treated with stenting procedure (HR 10.438, 95%CI 5.226-20.846, p < 0.001), maybe due to an older age at the time of stenting procedure. Conclusions: Adult Pts with repaired CoA present a high prevalence of residual aHT, which is a driving risk factor for the development of CV disease in older age. Careful clinical and imaging follow-up is needed to address the occurrence of re-CoA or pseudoaneurysm formation and tailor antihypertensive treatment. CoA-Pts treated with stenting procedure are at greatest risk for CV events. 807 Advance 3D visualization tools in the pre-operative assessment of double outlet right ventricle Elena Giulia Milano Elena Giulia Milano Universita’ degli Studi di Verona UCL and Great Ormond Street Hospital for Children, London, UK Endrit Pajaziti Endrit Pajaziti UCL and Great Ormond Street Hospital for Children, London, UK Martin Kostolny Martin Kostolny UCL and Great Ormond Street Hospital for Children, London, UK Jan Marek Jan Marek UCL and Great Ormond Street Hospital for Children, London, UK William Regan William Regan UCL and Great Ormond Street Hospital for Children, London, UK Massimo Caputo Massimo Caputo Bristol Heart Institute, University of Bristol, Bristol, UK Giovanni Battista Luciani Giovanni Battista Luciani Universita’ degli Studi di Verona Kristian Mortensen Kristian Mortensen UCL and Great Ormond Street Hospital for Children, London, UK Jo Wray Jo Wray UCL and Great Ormond Street Hospital for Children, London, UK Giovanni Biglino Giovanni Biglino Bristol Heart Institute, University of Bristol, Bristol, UK Andrew Cook Andrew Cook UCL and Great Ormond Street Hospital for Children, London, UK Andrew Taylor Andrew Taylor UCL and Great Ormond Street Hospital for Children, London, UK Silvia Schievano Silvia Schievano UCL and Great Ormond Street Hospital for Children, London, UK Claudio Capelli Claudio Capelli UCL and Great Ormond Street Hospital for Children, London, UK Universita’ degli Studi di Verona UCL and Great Ormond Street Hospital for Children, London, UK Bristol Heart Institute, University of Bristol, Bristol, UK Background: Advance 3 D-visualization in congenital heart disease has gained importance in procedural planning, however it is still unclear which 3 D-modelling technique represents the most informative tool in the surgical workup. Objectives: We aim to determine if there is additional benefit of 3 D-modelling compared to conventional cross-sectional imaging when deciding on surgical strategy in patients with complex double outlet right ventricle (DORV) and to identify the best 3 D modality to support procedural planning. Methods: We retrospectively selected ten patients with DORV and complex interventricular communications, who underwent biventricular repair with intracardiac tunnelling. CT or cardiac MRI images were used to reconstruct patient-specific 3 D anatomies, which were then presented using different visualisation modalities: 3Dpdf, physical 3 D printed models, and virtual reality (VR) models. Two senior paediatric cardiac surgeons reviewed each case blindly and were asked to decide on the suitability of biventricular repair and possibility of performing arterial switch operation (ASO). The abovementioned modalities were used to guide them in their decisions. A comparison was made to actual operation performed. Results: After CT/CMR images review, the two surgeons correctly identified the surgical strategy in 75% of the cases; this improved to 85% after revision of the 3 D printed models and to 95% after VR visualisation. 3Dpdf visualisation instead resulted in a reduction of correct identification to 70%. When asked about suitability for ASO the answers were in accordance with actual surgery in 45% after reviewing of CT/cardiac MRI data and 3dpdf; this increased to 55% and 60% after the inspection of the 3 D printed and VR model, respectively. Conclusions: 3 D printed models and VR can enhance understanding of suitability for biventricular repair in patients with complex DORV. 131 Sometimes the heart could appear different if seen under a magnetic resonance scanner: misdiagnosed cases of isolated left ventricle apical hypoplasia and double chambered left ventricle Gianluca Trocchio Gianluca Trocchio U.O.C. Cardiologia, Dipartimento Cardiovascolare, Istituto Giannina Gaslini, Genova Sara Moscatelli Sara Moscatelli U.O.C. Cardiologia, Ospedale Policlinico San Martino, Genova Nicola Stagnaro Nicola Stagnaro U.O.C. Radiologia, Istituto Giannina Gaslini, Genova Francesca Rizzo Francesca Rizzo U.O.C. Radiologia, Istituto Giannina Gaslini, Genova Lamia Ait-Ali Lamia Ait-Ali Istituto di Fisiologia Clinica, CNR, Massa-Pisa Pierluigi Festa Pierluigi Festa Fondazione G. Monasterio CNR-Regione Toscana, Ospedale del Cuore, UO Cardiologia Pediatrica, Massa Gian Michele Magnano Gian Michele Magnano U.O.C. Radiologia, Istituto Giannina Gaslini, Genova Maurizio Marasini Maurizio Marasini U.O.C. Cardiologia, Dipartimento Cardiovascolare, Istituto Giannina Gaslini, Genova U.O.C. Cardiologia, Dipartimento Cardiovascolare, Istituto Giannina Gaslini, Genova Istituto di Fisiologia Clinica, CNR, Massa-Pisa Fondazione G. Monasterio CNR-Regione Toscana, Ospedale del Cuore, UO Cardiologia Pediatrica, Massa U.O.C. Radiologia, Istituto Giannina Gaslini, Genova U.O.C. Cardiologia, Ospedale Policlinico San Martino, Genova Introduction: Congenital heart diseases (CHD) are detected in 1% of children, often usually the first year of life; however, many defects are diagnosed later or remain undiagnosed. Both congenital and acquired disorders could affect the left ventricle (LV). First-line assessment includes echocardiography; nonetheless, because of intrinsic or technical limitations and artefacts, further investigation may be required. Cardiac Magnetic Resonance (CMR) is capable of providing anatomical and functional information without many of limitations and drawbacks of echocardiography. We describe CMR findings of misdiagnosed cases of two rare congenital LV abnormalities: isolated LV apical hypoplasia (ILVAH) and double-chambered LV (DCLV). Case Report 1: An 18 yrs girl diagnosed with dilated cardiomyopathy (DCM) was submitted to our CMR Lab for a functional assessment. After birth, deep Q waves in the inferior leads were noted on the ecg, and an echocardiographic examination showed a dilated and hypokinetic LV. At 1 year of age, she underwent cardiac catheterization that excluded coronary arteries anomalies and confirmed a reduced LV systolic function. A diagnosis of idiopathic DCM was formulated and she was initiated with anticongestive therapy. During the follow-up she felt well with normal exercise tolerance, longitudinal echocardiography did not show any substantial modification over the years. In 2018, a CMR study was performed. Surprisingly, the cardiac apex was formed exclusively by the right ventricle, wrapped around the LV. The LV appeared spherical and truncated inferiorly, and the apical portion was missing; LV volumes and ejection fraction were normal; regional akinesia and subendocardial late gadolinium enhancement (LGE) were evident at the inferior wall. These findings were consistent of ILVAH. Case Report 2: A 24 yrs old boy with prenatal diagnosis of LV diverticulum came to our observation for a CMR study. He was asymptomatic, particularly, no palpitations or syncope were referred, nor arrhythmias were detected during the follow-up; exercise tolerance was normal. CMR showed a coarse muscle band in the LV cavity, extending from the apex to the posterior papillary muscle, thus delimiting a contractile accessory chamber. LV volumes and global systolic function were normal. The LV accessory chamber presented a normal structured free lateral wall with a normal systolic thikening, except at the apical infero-lateral segment where it appeared thinned and akinetic and showed LGE with a subendocardial pattern. These findings were consistent of DCLV. Conclusions: The LV could be affected by many diseases with different etiological, clinical and morphological features. Compared to other imaging diagnostic modalities, CMR allows better definition of LV morphology, function and tissue characterization, becoming essential for LV abnormalities diagnosis and follow-up. 753 An unexpected case of pediatric myocardial ischemia: Truncus Arteriosus Communis and coronary artery anomalies, a challenging case Sara Moscatelli Sara Moscatelli Universtià degli Studi di Genova & UO Cardiologia, Ospedale San Martino, Genova Gianluca Trocchio Gianluca Trocchio UO Cardiologia Pediatrica, Istituto Giannina Gaslini, Genova Nicola Stagnaro Nicola Stagnaro UO Radiologia, Istituto Giannina Gaslini Maria Derchi Maria Derchi UO Cardiologia Pediatrica, Istituto Giannina Gaslini, Genova Giulia Tuo Giulia Tuo UO Cardiologia Pediatrica, Istituto Giannina Gaslini, Genova Martino Cheli Martino Cheli UO Cardiologia Pediatrica, Istituto Giannina Gaslini, Genova Alessandra Siboldi Alessandra Siboldi UO Cardiologia Pediatrica, Istituto Giannina Gaslini, Genova Sara Bondanza Sara Bondanza UO Cardiologia Pediatrica, Istituto Giannina Gaslini, Genova Italo Porto Italo Porto Universtià degli Studi di Genova & UO Cardiologia, Ospedale San Martino, Genova Maurizio Marasini Maurizio Marasini UO Cardiologia Pediatrica, Istituto Giannina Gaslini, Genova Universtià degli Studi di Genova & UO Cardiologia, Ospedale San Martino, Genova UO Cardiologia Pediatrica, Istituto Giannina Gaslini, Genova UO Radiologia, Istituto Giannina Gaslini Introduction: Truncus Arteriosus Communis (TAC) is a rare (incidence of 0.03-0.056/1000 live births) congenital cardiac malformation in which a single common artery arises from the heart by means of a single semilunar truncal valve and supplies the systemic and pulmonary circulations. Pulmonary arteries originate from the common arterial trunk distal to the coronary arteries and proximal to the first brachiocephalic branch of the aortic arch. TAC typically overrides a large outlet ventricular septal defect. TAC is an important cause of death in infancy. This case concerns a patients affected by Type I (or Type A1 according to Van Praagh) TAC, which is anatomically constituted by a short common pulmonary artery segment that arises on the left side of the common trunk just above the truncal valve, that gives rise to separate left and right pulmonary arteries, and by an ascending aorta that arises from the right side of the common trunk. Clinical Case: A 9 y/o girl affected by Di George Syndrome was born with a prenatal diagnosis of TAC type I. In the 1st month of life she underwent surgical correction by positioning a valved conduit between the right ventricle (RV) and the pulmonary arteries (PA). At 4 y/o she developed a severe truncal valve regurgitation associated with a significant left ventricle (LV) dysfunction, and a new operation was performed: a mechanical prothesis was implanted and the conduit changed with a larger one; immediate post-operative course was complicated by low cardiac output syndrome and LV dysfunction; however, her hemodynamic condition and LV function gradually improved and she was discharged 1 month after hospitalization, in treatment with warfarin and ramipril. At 9 y/o during a follow-up visit, an enlarged, globose LV with a slightly reduction of systolic function, septum dyskinesia and hypokinesia of the anterolateral wall, was detected at the transthoracic echocardiography examination (TTE), and she was finally directed to our Centre. A cardiac catheterization was performed showing: normal prosthetic aortic valve function, intra prosthetic pulmonary stenosis that was treated with balloon valvuloplasty, and most of all a diffusely hypolplastic left coronary artery (LCA) with a 70% stenosis, abnormally originating from the anterior sinus of Valsava and presenting a posterior course around the prosthetic aortic valve; the right coronary artery was originating with a separate ostium. Furthermore, a stress TTE was performed, resulting positive for low threshold inducible myocardial ischemia. An uneventful 24 hours Holter EKG was obtained and therapy with propanolol was started. An exercise stress test (EST) was also performed resulting negative for inducible ischemia. Finally, a cardiac MRI documented subendocardial LGE in anterior, anterolateral and lateral LV wall, without evidence of recent myocardial ischemia. Considering the absence of a full convincing invasive myocardial revascularization strategy, type of coronary lesion, past surgical history and that a highly probable new reoperation will be needed also for changing again the RV to PA conduit, the absence of symptoms and signs of inducible cardiac ischemia, the absence of arrhythmias, it was decided to continue follow-up maintaining the patient on medical therapy. Conclusion: Congenital heart malformations can be associated with coronary anomalies, carrying additional risk for sudden cardiac death. In our case, the ratio between benefits and drawbacks of an invasive approach, together with the patient’s clinical history and perspectives, brought to the decision to ‘wait and see’. Nonetheless, because of the rarity of this association, each patient has a singular path. 816 Sudden cardiac death in infancy and congenital coronary artery anomalies Sara Moscatelli Sara Moscatelli Universita degli Studi di Genova, Ospedale San Martino, Genova Gianluca Trocchio Gianluca Trocchio UO Cardiologia Pediatrica, Istituto Giannina Gaslini, Genova Italo Porto Italo Porto Universita degli Studi di Genova, Ospedale San Martino, Genova Giuseppe Pomé Giuseppe Pomé UO Cardiochirurgia Pediatrica, Istituto Giannina Gaslini, Genova Maurizio Marasini Maurizio Marasini UO Cardiologia Pediatrica, Istituto Giannina Gaslini, Genova Universita degli Studi di Genova, Ospedale San Martino, Genova UO Cardiologia Pediatrica, Istituto Giannina Gaslini, Genova UO Cardiochirurgia Pediatrica, Istituto Giannina Gaslini, Genova Introduction: Congenital coronary artery anomalies (CCA) occur rarely.They are usually an incidental finding at transthoracic echocardiography (TTE), but diagnosis could be extremely challenging. CC1: A 3 months old male child without a family history of congenital heart diseases and SCD was admitted in our Hospital after a resuscitated cardiac arrest. The infant was in good health condition until he suddenly fell lifeless after feeding. EKG was normal; TTE showed normal cardiac anatomy, normal coronary arteries origin, trivial aortic regurgitation and a substantially preserved left ventricle (LV) function. The subsequent course was favourable. The day after he was was weaned from mechanical ventilation, he had a second cardiac arrest that was recorded by a Holter EKG showing ST segment depression, followed by severe bradycardia and complete atrio-ventricular block. TTE detected hypokinesia of the infero-lateral LV wall, iperechogenic spots in the LV endocardium and in the mitral valve (MV) papillary muscles, mild-moderate MV and aortic valve (AV) regurgitations. An obstruction of the left coronary artery (LCA) was suspected, but coronary angiography (CA) failed to identify anomalies. Considering the possibility of tecnical limitations in a low weight infant, and the strong evidence of an underlying ischaemic mechanism, the hypothesis of a LCA ostial stenosis wasnot abbandoned, and he underwent a open heart surgery (HS) that found a hypoplastic and stenotic LCA ostium. LCA ostial plasty with a pericardial patch was performed. There were no complications, postoperative course was regular and uneventful. Medical therapy with acetylsalicylic acid (ASA), captopril, carvedilol and furosemide was started. At 6 month F-UP no major LV and MV/AV functional sequelae were evident at TTE, and he remained asymptomatic. CC2: A female child who was followed since birth for a postnatal diagnosis of ostium secundum ASD and perimembranous VSD, underwent surgical correction at 9 months old. After, she had recurrent episodes of loss of consciousness and hypotension during or immediately after meals not associated with EKG alterations and experienced a cardiac arrest. CA did not show coronary stenosis or anomalies. So, these events were suspected to be related to an addominal trigger and surgery was undertaken. However, symptoms persisted and she was transferred to our Center for further investigations. She had other two episodes of cardiac arrest preceded by sinus tachycardia and ventricular arrhythmias and during one of these TTE documented a gradually worsening AV regurgitation followed by LV systolic dysfunction. Cardiac CT resulted normal and a new cardiac catheterization was achieved including ADP Test, hyperventilation test, stress-dobutamine test, nitrates test, and finally temporary ballon occlusion of the pulmonary artery. During these test, ST segment depression in V1-V6, D1, D2, aVL with simultaneous ST elevation in AVR was documented, and a simultaneous TTE confirmed previous findings. LCA ostial anomaly was suspected. During HS the surgeon discovered an endoluminal flap in the proximal part of the LCA that presented a slit-like orifice: an ostial plasty with an autologous saphenous vein graft was performed. The patient was discharged on medical therapy with ASA, captopril and carvedilol. At 3 y/o CA showed good surgical result, and no signs nor symptoms of myocardial ischemia were detected. Conclusion: CCA are rare, but these must be suspected in case of unexplainable pediatric aborted SCD, especially if related to feeding, and with an apparently unexplainable and aspecific TTE finding of AV regurgitation 33 Univentricular heart with Fontan circulation and pregnancy Laura Orrú Laura Orrú Pediatric Cardiology and Congenital Heart Diseases Saint Michael Hospital Brotzu Cagliari Assunta Merola Assunta Merola Pediatric Cardiology and Congenital Heart Diseases Saint Michael Hospital Brotzu Cagliari Monica Urru Monica Urru Pediatric Cardiology and Congenital Heart Diseases Saint Michael Hospital Brotzu Cagliari Ramona Stara Ramona Stara Pediatric Cardiology and Congenital Heart Diseases Saint Michael Hospital Brotzu Cagliari Federica Scano Federica Scano Pediatric Cardiology and Congenital Heart Diseases Saint Michael Hospital Brotzu Cagliari Enrica Marini Enrica Marini Pediatric Cardiology and Congenital Heart Diseases Saint Michael Hospital Brotzu Cagliari Michela Congia Michela Congia Pediatric Cardiology and Congenital Heart Diseases Saint Michael Hospital Brotzu Cagliari Andrea Marini Andrea Marini Pediatric Cardiology and Congenital Heart Diseases Saint Michael Hospital Brotzu Cagliari Alessandra Cadoni Alessandra Cadoni Pediatric Cardiology and Congenital Heart Diseases Saint Michael Hospital Brotzu Cagliari Donatella Manca Donatella Manca Pediatric Cardiology and Congenital Heart Diseases Saint Michael Hospital Brotzu Cagliari Sabrina Montis Sabrina Montis Pediatric Cardiology and Congenital Heart Diseases Saint Michael Hospital Brotzu Cagliari Roberto Tumbarello Roberto Tumbarello Pediatric Cardiology and Congenital Heart Diseases Saint Michael Hospital Brotzu Cagliari Pediatric Cardiology and Congenital Heart Diseases Saint Michael Hospital Brotzu Cagliari Women with congenital heart defects, thanks to diagnostic and therapeutic advances can reach fertile age and have a pregnancy: someone with a cardiovascular risk comparable to the general population, others with serious sequelae at high risk of maternal-Foetal complications. Patients with univentricular heart and Fontan circulation are included in the World Health Organization (WHO) pregnancy risk class III, having a significant increase in the risk of maternal morbidity and mortality. In these women, in fact, the atrium and the single ventricle have limited ability to adapt to haemodynamic changes, leading to the worsening of a possible cyanosis, acute heart failure and thromboembolism. CLINICAL CASE: S.P., affected by tricuspid atresia, underwent surgical palliation at 11 years old through Fontan procedure with a fenestrated extra-cardiac conduit. A few years later the fenestration was closed with a percutaneous device. She attended our outpatient clinic according to a regular six-months follow-up. At 20 years old the cardiac magnetic resonance (MRI) showed an ejection fraction (EF) of 67% and normally functioning Fontan circuit. Dynamic 24 hours ECG showed frequent ventricular ectopic beats, sometimes in short non-sustained runs (max 9 beats), hence Bisoprolol was prescribed. At 19 years old she had unplanned pregnancy interrupted with miscarriage at 10t8 week. At 26 years old she had a new unplanned pregnancy and came to our attention at week 6t8. The medical therapy included acetylsalicylic acid, an ace-inhibitor (ACE-I) and Bisoprolol. The ACE-I was immediately discontinued and Bisoprolol was replaced with Metoprolol. The echocardiography showed a single left ventricle with EF of 60% and normal diastolic function, a rudimental right ventricle and a large interventricular septal defect, a mild mitral regurgitation and a normally functioning Fontan circuit. During pregnancy the patient underwent clinical and echocardiographic monitoring every 2-3 weeks, monthly dynamic ECG and Foetal echocardiography with evidence of clinical and instrumental stability: Foetal growth charts were at low limits but constant in progression. Even without contraindications for the vaginal delivery, after multidisciplinary discussion and considering the patient's desire, a caesarean section was planned for the 39t8 week and was performed without complications. The patient was monitored in ICU for 24 hours after delivery, there were no arrhythmic events. A slow hydration was started to maintain the cardiac output. The patient was always in good clinical and hemodynamic compensation. The echocardiographic check after delivery was stable. The management of pregnancy in women with complex congenital heart defetcs must be multidisciplinary to minimize maternal risks and ensure Foetal well-being. The complexity of some cardiac defects requires the collaboration between pediatric cardiologists, gynecologists, obstetricians, psychologists, geneticists, cardiac surgeons and anesthesiologists in all the phases from conception to childbirth. 870 Role of cardiovascular magnetic resonance end-systolic 3D-SSFP sequence in repaired tetralogy of Fallot patients eligible for transcatheter pulmonary valve implantation Marco Alfonso Perrone Marco Alfonso Perrone Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Childreńs Hospital, IRCCS, Rome, Italy Francesco Romeo Francesco Romeo Division of Cardiology, University of Rome, “Tor Vergata”, Rome, Italy Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Childreńs Hospital, IRCCS, Rome, Italy Division of Cardiology, University of Rome, “Tor Vergata”, Rome, Italy Aim: To evaluate the usefulness of cardiovascular magnetic resonance (CMR) 3 D steady state free precession (SSFP) sequence acquired at end-systole (ES) in repaired Tetralogy of Fallot (rToF) patients eligible for percutaneous pulmonary valve implantation (PPVI). Methods: Between 2012 and 2018, 78 rToF patients were selected for pulmonary valve replacement (PVR) according to CMR criteria. CMR protocol included 3 D-SSFP sequence used to assess the right ventricle outflow tract (RVOT) diameters at three levels (pulmonary valve remnant, mid-portion, bifurcation) in mid-diastole (MD) or ES, RVOT length and coronary artery anatomy. In 20 rToF patients without indications for PVR (controls), 3 D SSFP sequence was acquired at both cardiac phases (MD and ES) to evaluate RVOT dimension throughout the cardiac cycle. Invasive balloon sizing was recorded in patients undergoing PPVI. The 3 D-SSFP sequence was performed in MD on 39 patients and in ES on other 39, of whom 26 patients met the criteria for PPVI. Results: PPVI was unsuccessful in ten patients (38%), mainly due (80% of cases) to significant size discrepancy at PV remnant and bifurcation levels (p = 0.019 and 0.037 respectively) between the measurements by 3 D-SSFP in MD and those by the balloon size in systole. Significant RVOT size difference between MD and ES was present at mid-portion and bifurcation levels in the PVR candidate group, and at all three-levels in the control group (all p < 0.001). Conclusion: ES 3 D-SSFP sequence is able to quantify RVOT dilation in rToF patients at its maximum expansion, thus improving selection of PPVI candidates. 588 Management of pregnancy and delivery in women with heart diseases: a single Centre experience Elettra Pomiato Elettra Pomiato Division of Cardiology, AOUI Verona, University of Verona, Verona, Italy Maria Antonia Prioli Maria Antonia Prioli Division of Cardiology, AOUI Verona, University of Verona, Verona, Italy Giovanni Zanconato Giovanni Zanconato Division of Obstetrics And Gynecology, AOUI Verona, University of Verona, Verona, Italy Costanza Muraro Costanza Muraro Division of Obstetrics And Gynecology, AOUI Verona, University of Verona, Verona, Italy Valentina Benetti Valentina Benetti Division of Cardiology, AOUI Verona, University of Verona, Verona, Italy Stefania Milesi Stefania Milesi Division of Anesthesia and Intensive Care, AOUI Verona, Verona, Italy Giulia Poretti Giulia Poretti Division of Cardiac Surgery, AOUI Verona, University of Verona, Verona, Italy Mara Pilati Mara Pilati Division of Cardiology, AOUI Verona, University of Verona, Verona, Italy Camilla Sandrini Camilla Sandrini Division of Cardiology, AOUI Verona, University of Verona, Verona, Italy Flavio Ribichini Flavio Ribichini Division of Cardiology, AOUI Verona, University of Verona, Verona, Italy Division of Cardiology, AOUI Verona, University of Verona, Verona, Italy Division of Obstetrics And Gynecology, AOUI Verona, University of Verona, Verona, Italy Division of Cardiac Surgery, AOUI Verona, University of Verona, Verona, Italy Division of Anesthesia and Intensive Care, AOUI Verona, Verona, Italy Background: Pregnancy in women with congenital heart disease is becoming nowadays more frequent, but many concerns still remain about safety of the pregnancy for the mother and her baby. Material and methods: We collected grown-up congenital heart disease women who had experienced one or more pregnancies, followed or referred at our Center between 2004 and 2018. Different pregnancies in the same woman were treated as independent events. The population was divided according to mWHO classification. We analyzed outcome of pregnancy, cardiovascular complications of the mother during it, time and mode of delivery, obstetric events and offspring outcome. Results and discussion: 178 pregnancies occurred in 104 women at our GUCH-Unit. Mean age at delivery was 32 years. In 94 (90.4%) women the defect was discovered before pregnancy, in 10 women (9.6%) it was discovered during (5; 4.8%) or after (5; 4.8%) the pregnancy. Baseline mWHO Class was I in 58 (32.6%) pregnancies, II in 43 (24.2%) pregnancies, II-III in 60 (33.7%), III in 17 (9.5%) pregnancies. Cardiovascular maternal adverse events, obstetric events, rate of C-section, mean duration of pregnancy, offspring complications for each mWHO Class are resumed in Table 1. On the basis of the raising of cardiac complications according to mWHO Class, we divided the pregnancies in 2 subgroups: Group 1 encompassed mWHO I and II; Group 2 comprehended mWHO II-III and III. OR with confidence interval at 95% of cardiovascular maternal adverse events, obstetric events, rate of C-section, offspring complications are shown in Table 2. In our population the raise of mWHO Class matched with statistically significant raise of cardiovascular events and of C-sections. Abortions, obstetric and offspring complications were more frequent in more elevated mWHO Classes but the difference was not statistically significant. Conclusion: Pregnancy in women with congenital heart disease is possible and is generally well tolerated for both the mother and the baby, but a clear pre-conceptional counselling before gestation and regular multidisciplinary risk-assessments during pregnancy are essential. 393 Echocardiographic assessment of right ventricular strain in patients with pulmonary hypertension: comparison with other parameters of right ventricular systolic function Morena Rossi Morena Rossi Dipartimento Cardiovascolare Ospedale di Circolo - Universita’ degli Studi dell’Insubria, Varese Chiara Giuntini Chiara Giuntini Dipartimento Cardiovascolare Ospedale di Circolo - Universita’ degli Studi dell’Insubria, Varese Francesca Ceriani Francesca Ceriani Dipartimento Cardiovascolare Ospedale di Circolo - Universita’ degli Studi dell’Insubria, Varese Roberta Giani Roberta Giani Dipartimento Cardiovascolare Ospedale di Circolo - Universita’ degli Studi dell’Insubria, Varese Gaia Telli Gaia Telli Dipartimento Cardiovascolare Ospedale di Circolo - Universita’ degli Studi dell’Insubria, Varese Michele Golino Michele Golino Dipartimento Cardiovascolare Ospedale di Circolo - Universita’ degli Studi dell’Insubria, Varese Andrea Imporzani Andrea Imporzani Dipartimento Cardiovascolare Ospedale di Circolo - Universita’ degli Studi dell’Insubria, Varese Mariangela Lattanzio Mariangela Lattanzio Dipartimento Cardiovascolare Ospedale di Circolo - Universita’ degli Studi dell’Insubria, Varese Roberto De Ponti Roberto De Ponti Dipartimento Cardiovascolare Ospedale di Circolo - Universita’ degli Studi dell’Insubria, Varese Dipartimento Cardiovascolare Ospedale di Circolo - Universita’ degli Studi dell’Insubria, Varese Introduction: Transthoracic echocardiography is a non-invasive, available and low-cost imaging test and in patients with pulmonary hypertension (PH) is performed to the detection of the effects of PH on the heart, to estimate the pulmonary arterial pressure and to suppose the causes of this pathophysiological disorder. Speckle tracking echocardiography is a quantitative ultrasound technique that describes myocardial deformation (strain) during the cardiac cycle. The right ventricular (RV) myocardial strain analysis could represent a sensitive tool for an earlier detection of RV disfunction and a determinant for risk assessment in this set of patients. Objectives: The aim of our study is to evaluate the role of the speckle tracking echocardiography describing RV performance in patients with PH through 1) a correlation analysis between the strain data and two common parameters of RV function, commonly used in clinical practice and 2) evaluation of the inter-operator reproducibility of these data. Methods: We enrolled 15 patients with PH; a transthoracic echocardiogram was performed with the sampling of the traditional parameters of RV function: the tricuspid annular plane systolic excursion (TAPSE) and the fractional area change (FAC). Two different operators calculated the RV free wall peak of systolic longitudinal strain (obtained by averaging the basal, middle and apical lateral peak systolic strains): during an off-line analysis. Results: RV free wall peak of systolic longitudinal strain and the two traditional and validated parameters of right ventricular function showed good correlation: TAPSE (with r coefficient = -0.7) and FAC (with r coefficient = -0.66). The RV free wall peak of systolic longitudinal strain was pathological in 60% of patients, while TAPSE and FAC were altered only in 33% and 27% of cases, respectively. Furthermore, an excellent correlation emerged between the values obtained by the two different operators (coefficient r = 0.93), confirming a good reproducibility of the speckle-tracking echocardiography. No significant correlation was obtained between the variables under study and the anatomical data of the end-diastolic and end-systolic RV areas. Conclusion: From these preliminary data we could affirm that in the population of patients with PH the speckle tracking echocardiography is a reproducible, non-invasive, low-cost index of the right ventricular function and strain data are complementary to those traditionally used in diagnosis and follow-up of these patients. It could also be configured as a more sensitive method compared to TAPSE and FAC in identifying an initial dysfunction of the right ventricle in this population. 854 Pathogenic role of folates cycle related endothelial dysfunction in interatrial defects leading to cryptogenic stroke Luca Sgarra Luca Sgarra Department of Biomedical Sciences and Human Oncology - Section of Pharmacology, Medical School, University of Bari “Aldo Moro”, Bari, Italy Alessandro Santo Bortone Alessandro Santo Bortone Department of Emergency and Organ Transplantation - Section of Interdepartmental Interventional Cardiology, Medical School, University of Bari “Aldo Moro”, Bari, Italy Maria Assunta Potenza Maria Assunta Potenza Department of Biomedical Sciences and Human Oncology - Section of Pharmacology, Medical School, University of Bari “Aldo Moro”, Bari, Italy Tommaso Acquaviva Tommaso Acquaviva Department of Emergency and Organ Transplantation - Section of Interdepartmental Interventional Cardiology, Medical School, University of Bari “Aldo Moro”, Bari, Italy Emanuela De Cillis Emanuela De Cillis Department of Emergency and Organ Transplantation - Section of Interdepartmental Interventional Cardiology, Medical School, University of Bari “Aldo Moro”, Bari, Italy Celeste Coco Celeste Coco Department of Biomedical Sciences and Human Oncology - Section of Pharmacology, Medical School, University of Bari “Aldo Moro”, Bari, Italy Massimo Grimaldi Massimo Grimaldi “Miulli” Regional Hospital - Section of Electrophysiology, Acquaviva delle Fonti, Italy Marco Matteo Ciccone Marco Matteo Ciccone Department of Emergency and Organ Transplantation - Section of Cardiovascular Disease, Medical School, University of Bari “Aldo Moro”, Bari, Italy Monica Montagnani Monica Montagnani Department of Biomedical Sciences and Human Oncology - Section of Pharmacology, Medical School, University of Bari “Aldo Moro”, Bari, Italy Department of Biomedical Sciences and Human Oncology - Section of Pharmacology, Medical School, University of Bari “Aldo Moro”, Bari, Italy Department of Emergency and Organ Transplantation - Section of Interdepartmental Interventional Cardiology, Medical School, University of Bari “Aldo Moro”, Bari, Italy “Miulli” Regional Hospital - Section of Electrophysiology, Acquaviva delle Fonti, Italy Department of Emergency and Organ Transplantation - Section of Cardiovascular Disease, Medical School, University of Bari “Aldo Moro”, Bari, Italy Aim: To evaluate the correlation, in terms of severity, between MTHFR genotype, interatrial septum (IAS) phenotype and the degree of endothelial dysfunction (expressed by L-arginine/asymmetric dimethylarginine (ADMA) ratio) in subject with a history of stroke. Methods and Results: L-arginine, ADMA, homocysteine levels and MTHFR genotype were evaluated. IAS phenotype was assessed by transcranial Doppler coupled with transesophageal echocardiography. Of 57 patients, 10 had a septum integrum (SI), 38 carried a patency of foramen ovale (PFO), and 9 a ostium secundum (OS). 16 patients carried the 677 T/T genotype, 13 the 677 C/T + 1298A/C double heterozygosis, 6 the 677 C/T heterozygosis, 5 the 1298 C/C mutation, and 15 patients the 1298 A/C or wild-type MTHFR. The highest the severity of genotype mutation, the more compromised was the interatrial septum according a spectrum septum integrum->large and short PFO tunnel->tight and long PFO tunnel->ostium secundum defect (Fig.1). L-Arginine/ADMA ratio differs across septum phenotype (p < 0.005 – Graph 2 ) and was higher in SI than in PFO or OS patients and (p < 0.05, p < 0.01, respectively). Nonetheless L-Arginine/ADMA ratio differs across MTHFR genotypes (p < 0.0001 – Graph. 1) and resulted lower in the 677 T/T and in the 677 C/T + 1298 A/C subgroups than in 1298 A/C and WT healthiest subgroup (p < 0.0001, p < 0.05, respectively). A negative correlation was found between the L-Arginine/ADMA ratio and the PFO tunnel length/height ratio (p < 0.05; r = - 0.37; R2 = 0.14 – Graph. 3). OS patients carried the most dysfunctional MTHFR genotypes whereas SI patients the least ones; in PFO subjects, the tunnel lenght/height ratio was proportional to the MTHFR-related L-Arginine/ADMA ratio. Conclusions: Impaired activity of MTHFR is associated with low L-Arginine/ADMA ratio; in turn, L-arginine/ADMA ratio correlates with IAS phenotype, along a severity spectrum encompassing SI, PFO with short/large tunnel, PFO with long/tight tunnel, and OS. Genetic MTHFR defects may underlie endothelial dysfunction-related IAS abnormalities, and predispose to cryptogenic stroke. 138 Thromboembolic pulmonary hypertension: a shared therapeutic diagnostic path Lucia Soriente Lucia Soriente Cardiologia Intensiva AOU San Giovanni di Dio e Ruggi d’Aragona, Plesso Ruggi, Salerno Roberto Ascoli Roberto Ascoli Cardiologia Intensiva AOU San Giovanni di Dio e Ruggi d’Aragona, Plesso Ruggi, Salerno Francesco Vigorito Francesco Vigorito Emodinamica AOU San Giovanni di Dio e Ruggi d’Aragona, Salerno Valeria Visco Valeria Visco Clinica Cardiologica AOU San Giovanni di Dio e Ruggi d’Aragona, Salerno Samanta Setola Samanta Setola Clinica Cardiologica AOU San Giovanni di Dio e Ruggi d’Aragona, Salerno Rosanna Matturro Rosanna Matturro Clinica Cardiologica AOU San Giovanni di Dio e Ruggi d’Aragona, Salerno Rossella Benvenga Rossella Benvenga Clinica Cardiologica AOU San Giovanni di Dio e Ruggi d’Aragona, Salerno Alberto Gigantino Alberto Gigantino Cardiologia Intensiva AOU San Giovanni di Dio e Ruggi d’Aragona, Plesso Ruggi, Salerno Carmine Vecchione Carmine Vecchione Clinica Cardiologica AOU San Giovanni di Dio e Ruggi d’Aragona, Salerno Alessandro Vatrella Alessandro Vatrella Clinica di Malattie dell Apparato Respiratorio AOU San Giovanni di Dio e Ruggi d’Aragona, Salerno Giuseppe Di Maso Giuseppe Di Maso Cardiologia AOU San Giovanni di Dio e Ruggi d’Aragona, Plesso G. Fucito, Mercato San Severino-Salerno Vincenzo Capuano Vincenzo Capuano Cardiologia AOU San Giovanni di Dio e Ruggi d’Aragona, Plesso G. Fucito, Mercato San Severino-Salerno Francesco Ferrara Francesco Ferrara Cardiologia AOU San Giovanni di Dio e Ruggi d’Aragona. Plesso Santa Maria Incoronata dell’Olmo, Cava dei Tirreni, Salerno Eduardo Bossone Eduardo Bossone Cardiologia Riabilitativa Azienda Ospedaliera di Rilievo Nazionale Cardarelli, Napoli Cardiologia AOU San Giovanni di Dio e Ruggi d’Aragona, Plesso G. Fucito, Mercato San Severino-Salerno Cardiologia AOU San Giovanni di Dio e Ruggi d’Aragona. Plesso Santa Maria Incoronata dell’Olmo, Cava dei Tirreni, Salerno Cardiologia Intensiva AOU San Giovanni di Dio e Ruggi d’Aragona, Plesso Ruggi, Salerno Clinica Cardiologica AOU San Giovanni di Dio e Ruggi d’Aragona, Salerno Clinica di Malattie dell Apparato Respiratorio AOU San Giovanni di Dio e Ruggi d’Aragona, Salerno Emodinamica AOU San Giovanni di Dio e Ruggi d’Aragona, Salerno Cardiologia Riabilitativa Azienda Ospedaliera di Rilievo Nazionale Cardarelli, Napoli Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) is the only class of pulmonary hypertension that can be treated by pulmonary endoarteriectomy (PEA), thus it is relevant that all cases of CTEPH are early detected and evaluated in an Expert Centre. Clinical suspicion of CTEPH should be high in patients with previous episodes of venous thromboembolism and/or pulmonary embolism, furthermore the severity of the onset clinical situation allows to make a prognostic stratification in the short and medium term. Patients with a main diagnosis of pulmonary embolism should have a unified outpatient follow-up pathway regardless of the areas of admission (cardiology, pneumology, internal medicine, surgery and intensive care unit) in order to monitor anticoagulant therapy, reducing the possibility of bleeding and preventing new thromboembolism events. Methods: The algorithm for the diagnosis and management of CTEPH adopted by our University Hospital expected i:n patients with symptoms an echocardiogram 3 months after the acute episode that it is followed by a pulmonary ventilator and perfusional scintigraphy and/or chest CT angiography if there is the suspect of pulmonary hypertension. If suggestive signs of CTEPH are found, the patient is referred to an PEA Expert Centre for surgical assessment. Discussion: PEA allows the majority of patients to restore a normal quality of life; long-term survival of patients having surgery is comparable with that of the general population with extremely low operating mortality rates (2-3%). For patients with persistence or recurrence of pulmonary hypertension or judged inoperable, is adopted a specific therapy. In the event that maximum medical therapy is ineffective, the patient is evaluated for lung transplantation. Moreover, for inoperable patients in medical treatment there is the possibility of a new experimental angioplasty of the pulmonary arteries. Conclusions: The shared diagnostic therapeutic pathway is proving to be a useful tool in ensuring appropriate performance and spending restraint. The choice and monitoring of process and outcome indicators according to the pathway verify the goodness of the pathway and ensure continuous improvement. 853 Effects of sildenafil on right ventricular systolic function in pulmonary arterial hypertension Marisa Talarico Marisa Talarico Università di Modena e Reggio Emilia Cristina Rolando Cristina Rolando Università di Modena e Reggio Emilia Umberto Stefanelli Umberto Stefanelli Università di Modena e Reggio Emilia Francesca Coppi Francesca Coppi Università di Modena e Reggio Emilia Anna Vittoria Mattioli Anna Vittoria Mattioli Università di Modena e Reggio Emilia Giuseppe Boriani Giuseppe Boriani Università di Modena e Reggio Emilia Rosario Rossi Rosario Rossi Università di Modena e Reggio Emilia Andrea Pennacchioni Andrea Pennacchioni Università di Modena e Reggio Emilia Università di Modena e Reggio Emilia Introduction: In pulmonary arterial hypertension (PAH) the right ventricle (RV) adaptation to effort the increased afterload of the pulmonary circulation depends on the RV ability to increase its systolic performance. RV function remains the main determinant of prognosis in these patients. The objective of our study is whether in-chronic Sildenafil therapy affects RV systolic performance assessed invasively by right heart catheterization (RHC). Materials and Methods: We enrolled in the Policlinico of Modena 84 consecutive patients affected by PAH of different etiologies who underwent a baseline and at 6-months RHC after the initiation or adjunction of Sildenafil in therapy. Primary end-points were: 1) Change in RV stroke volume (RVSV), in pulmonary vascular resistance (PVR), both assessed by right heart catheterization (RHC), from baseline to 6 months. Results: In our population, we showed at 6-months a significative improvement of RVSV and reduction in PVR (+16.6 mL, p < 0.0001; 37% reduction, p < 0.0001). Moreover, we observed an improvement of cardiac index and a reduction of mean arterial pressure (mPAP) (+ 0.41, p < 0.01; -8.32, p < 0.001). In a subgroup of 34 patients, we performed RV ventriculography during RHC and we found an improvement in RV ejection fraction at 6-months (+12.8, p < 0.0001). Conclusion: In our PAH population, Sildenafil therapy had a direct and positive impact on RV systolic function and other hemodynamic parameters assessed by RHC. 264 When you see it. Evelina Toscano Evelina Toscano IRCCS Ospedale San Raffaele Milano - Unita’ Terapia Intensiva Coronarica Vittorio Pazzanese Vittorio Pazzanese IRCCS Ospedale San Raffaele Milano - Unita’ Terapia Intensiva Coronarica Matteo Pagnesi Matteo Pagnesi IRCCS Ospedale San Raffaele Milano - Unita’ Terapia Intensiva Coronarica Francesco Ancona Francesco Ancona IRCCS Ospedale San Raffaele Milano - Unita’ Terapia Intensiva Coronarica Marco Ancona Marco Ancona IRCCS Ospedale San Raffaele Milano - Emodinamica e Cardiologia Interventistica Luca Angelo Ferri Luca Angelo Ferri IRCCS Ospedale San Raffaele Milano - Emodinamica e Cardiologia Interventistica Savino Altizio Savino Altizio IRCCS Ospedale San Raffaele Milano - Unita’ Terapia Intensiva Coronarica Matteo Montorfano Matteo Montorfano IRCCS Ospedale San Raffaele Milano - Emodinamica e Cardiologia Interventistica Eustachio Agricola Eustachio Agricola IRCCS Ospedale San Raffaele Milano - Laboratorio di Ecocardiografia Federico Pappalardo Federico Pappalardo IRCCS Ospedale San Raffaele Milano - Unita’ Terapia Intensiva Coronarica Paolo Guido Camici Paolo Guido Camici IRCCS Ospedale San Raffaele Milano - Unita’ Terapia Intensiva Coronarica IRCCS Ospedale San Raffaele Milano - Unita’ Terapia Intensiva Coronarica IRCCS Ospedale San Raffaele Milano - Laboratorio di Ecocardiografia IRCCS Ospedale San Raffaele Milano - Emodinamica e Cardiologia Interventistica Case Description: A 46 years-old man was admitted to our emergency department (ED) for worsening dyspnoea and weight gain. His medical history consisted of psoriasis and previous hospitalization for pneumonia complicated by ARDS in 2007. Known cardiovascular risk factors were hypertension, obesity, and history of smoking. Upon arrival to the ED, the patient was tachi-dyspnoic, hypertensive, tachyarrhythmic in atrial fibrillation (AF), with clinical and radiographic signs of pulmonary congestion. ECG: AF with high ventricular rate (155 b.p.m.), no signs of acute ischemia. Transthoracic echocardiography (TTE): severe biventricular dysfunction (left ventricular ejection fraction 25%), no significant mitral regurgitation (MR) or aortic regurgitation (AR), moderate tricuspid regurgitation, severe pulmonary hypertension (sPAP 60 mmHg). The patient was immediately admitted to the Intensive Care Unit; IV diuretic, non-invasive mechanical ventilation, heparin, and digoxin were prescribed. Due to sub-optimal rate-control, once performed transesophageal echocardiography, we preceded to electric cardioversion and restoration of sinus rhythm. After few days, once introduced and up-titrated anti-remodelling medical therapy (metoprolol and ramipril), TTE was repeated: dilated left ventricle with recovery of systolic function, E/E’ <12, minimum MR, minimum AR, moderate-severe dilation of the right ventricle and pulmonary trunk, moderate right ventricular (RV) dysfunction, severe pulmonary hypertension. According to these data, we suspected pre-capillary PH, with acute decompensated RV failure during high-rate AF. According to ESC guidelines, we decided to perform computed tomography (CT) scan for lung and heart. CT scan revealed patent ductus arteriosus (PDA), with significant left-to-right shunt. After Heart Team discussion, percutaneous closure of PDA was indicated and performed. An Amplatzer PDA occluder I 20-14-12mm was implanted through femoral approach. Discussion: In patients with PH, correct diagnosis and treatment are keys to improve patient’s symptoms and prognosis. Congenital defects are very uncommon in adults without any prior medical history, especially in western countries. Percutaneous approach for grown-up congenital heart disease is very intriguing, since most of the procedures are less invasive than traditional surgery. In our case, percutaneous closure of PDA was technically feasible, and procedural success was achieved. At 3 months follow-up the patient is asymptomatic without reported adverse events. 155 A dyspnoea with surgical indication Valeria Visco Valeria Visco Dipartimento di Medicina, Chirurgia e Odontoiatria - Università degli Studi di Salerno Lucia Soriente Lucia Soriente A.O.U. San Giovanni di Dio e Ruggi d’Aragona, Salerno Chiara Aliberti Chiara Aliberti Medicina e Chirurgia, Università Cattolica del Sacro Cuore Samanta Setola Samanta Setola Dipartimento di Medicina, Chirurgia e Odontoiatria - Università degli Studi di Salerno Roberto Ascoli Roberto Ascoli A.O.U. San Giovanni di Dio e Ruggi d’Aragona, Salerno Saverio Ferrara Saverio Ferrara A.O.U. San Giovanni di Dio e Ruggi d’Aragona, Salerno Rodolfo Citro Rodolfo Citro A.O.U. San Giovanni di Dio e Ruggi d’Aragona, Salerno Tiziana Attisano Tiziana Attisano A.O.U. San Giovanni di Dio e Ruggi d’Aragona, Salerno Antonio Longobardi Antonio Longobardi A.O.U. San Giovanni di Dio e Ruggi d’Aragona, Salerno Michele Ciccarelli Michele Ciccarelli Dipartimento di Medicina, Chirurgia e Odontoiatria - Università degli Studi di Salerno Andrea Maria D´Armini Andrea Maria D´Armini Dipartimento di Scienze Clinico-Chirurgiche, Diagnostiche e Pediatriche - Università degli Studi di Pavia Dipartimento di Medicina, Chirurgia e Odontoiatria - Università degli Studi di Salerno A.O.U. San Giovanni di Dio e Ruggi d’Aragona, Salerno Medicina e Chirurgia, Università Cattolica del Sacro Cuore Dipartimento di Scienze Clinico-Chirurgiche, Diagnostiche e Pediatriche - Università degli Studi di Pavia Background: Dyspnoea affects up to 25% of the patients observed in a clinic; it can result from multiple underlying conditions and without an effective etiological treatment it is a short-term negative prognostic factor. Clinical case: The patient S.G., 70 years, arrived at our cardiology clinic for worsening dyspnoea, which had been present for 2 years. In patient history: previous removal of right pulmonary echinococcus cysts, deep vein thrombosis of the right popliteal vein, high blood pressure and previous smoking habit. Normal cardiac and thoracic physical examination, but evidence of lower limb edemas; electrocardiogram showed sinus rhythm and incomplete right bundle block. Echocardiography showed signs of pulmonary hypertension (PAPS 65 mmHg) without left ventricle disease. The patient performed chest CT angiography that documented bilateral pulmonary thromboembolism and started oral anticoagulants. Laboratory tests showed: hyperhomocysteinemia with heterozygous mutation of MTHFR and absence of autoimmune disease. Spirometry documented a medium degree airway obstruction, not reversible after administration of b2-adrenergic bronchodilator; DLCO 79% of the standard. A.B.G.: marked hypoxemia with normocapnia (pH 7.45, PaCO2 32.7 mmHg, PaO2 58.7 mmHg). After 3 months of oral anticoagulant therapy, the TTE showed persistence of dilation of the right chambers with D-shape left ventricle and small circle pressures still severely increased, while the echocolordoppler of lower limbs was normal. Lung scintigraphy showed multiple defects, compatible with bilateral pulmonary thromboembolism; chest CT angiography showed marked indirect lung signs of chronic thromboembolism disease, vascular asymmetry and diffuse mosaic perfusion aspects with scars of previous pulmonary infarctions, without alterations of the pulmonary interstitial tissue: chronic thromboembolic disease was confirmed with thrombotic material already in the main branches. Consequently, the patient was referred to a centre experienced in pulmonary endarterectomy (PEA), where he had right catheterization which concluded for severe pre-capillary pulmonary hypertension (pulmonary artery pressure 101/56/28 mmHg, wedge 5 mmHg, RVP 1028 dyne*sec*cm-5) and significant reduction of cardiac index (2 l/min/m2). Therefore, filter placement was made in inferior vena cava at the subrenal level through right jugular vein; then, was performed bilateral PEA. At scintigraphic control was obtained perfusion improvement and right catheterization showed: 55% reduction in lung pressures (25 vs 56 mmHg), 85% of RVP (154 vs 1028 dyne*sec*cm-5) and an increased cardiac index (2.6 vs 2 l/min/m2). Discussion and conclusion: Chronic thromboembolic pulmonary hypertension (CTEPH) is a pathological condition linked to unabsorbed and fibrous organization of the thrombus within the pulmonary artery lumen following an acute pulmonary embolism and PEA is the gold standard for restoring cardiorespiratory function to a state of near-normality. In the case of our patient, the instrumental examinations performed showed the excellent success of the surgery with the interruption of home O2 therapy. The challenge remains the clinical suspicion for an early diagnosis with multidisciplinary approach, because in more than 50% of cases the episode of acute pulmonary embolism goes unnoticed. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2019. For permissions please email: journals.permissions@oup.com. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model)
TI - Congenital Heart Disease and Diseases of the Pulmonary Circulation
JF - European Heart Journal Supplements
DO - 10.1093/eurheartj/suz251
DA - 2019-12-01
UR - https://www.deepdyve.com/lp/oxford-university-press/congenital-heart-disease-and-diseases-of-the-pulmonary-circulation-4rk74mQPps
SP - J76
VL - 21
IS - Supplement_J
DP - DeepDyve
ER -