(1867)
Ueber den Einfluss der Stenose der Conus arteriosus, des Ostium pulmonale und der Pulmonalarterie auf Entstehung von Tuberkulose, 4
W. Yater, M. Shapiro (1937)
CONGENITAL DISPLACEMENT OF THE TRICUSPID VALVE (EBSTEIN'S DISEASE): REVIEW AND REPORT OF A CASE WITH ELECTROCARDIOGRAPHIC ABNORMALITIES AND DETAILED HISTOLOGIC STUDY OF THE CONDUCTION SYSTEM*Annals of Internal Medicine, 11
(1927)
Eine seltene Missbildung der Trikuspidalklappe (‘Ebsteinsche Krankheit’), 266
(1876)
Therapie des Diabetes mellitus, insbesondere über die Anwendung des salicylsauren Natron bei demselben, 13
L. Soloff, H. Stauffer, J. Zatuchni (1951)
EBSTEIN'S DISEASE: REPORT OF THE FIRST CASE DIAGNOSED DURING LIFEThe American Journal of the Medical Sciences, 222
(1866)
Ueber einen sehr seltenen Fall von Insufficienz der Valvula tricuspidalis, bedingt durch eine angeborene hochgradige Missbildung derselben, 7
Mann Rj, Lie Jt (1979)
The life story of Wilhelm Ebstein (1836-1912) and his almost overlooked description of a congenital heart disease.Mayo Clinic Proceedings, 54
(1913)
Ueber eine besondere Form von Entwicklungsstörung der Trikuspidalklappe, 214
(1900)
Congenital malformations of the heart as illustrated by the specimens in the pathological museum of the Johns Hopkins Hospital, 11
(1907)
Wilhelm Ebstein's Arbeiten aus den Jahren 1859–1906, 89
Abstract In 1866, Wilhelm Ebstein published a scholarly description of a tricuspid valve anomaly with dilation of the right atrium and patent foramen ovale that bears his name. However, his original report was almost overlooked. Despite a wide range of publications on the history of cardiac pathology and cardiac surgery, the international literature provides only scarce information regarding the personality of Wilhelm Ebstein and his original description of the anomaly that bears his name. In this article, we present biographical data of Wilhelm Ebstein and discuss how his original description of autopsy findings correlates with our current knowledge of this congenital disorder. It is the excellent correlation of Ebstein's pathologic findings with clinical notes of his colleague and Ebstein's hypotheses of the pathophysiology that made his publication a landmark in the description of a new entity. In addition, Ebstein's report provided a strong basis for the development of repair techniques for this rare anomaly 100 years later. Ebstein's malformation, Tricuspid valve anomaly, Biographical data 1 Introduction Ebstein's scholarly description of the tricuspid valve abnormality was published in 1866 [1]. However, his report was almost overlooked. Despite a wide range of publications on the history of cardiac pathology and cardiac surgery, the international literature provides only one article focusing on the personality of Wilhelm Ebstein [2]. In this article, we present further biographical data of Wilhelm Ebstein and revisit his original report in a view of our current knowledge of this congenital disorder. 2 The life of Wilhelm Ebstein (1836–1912) Wilhelm Ebstein (Fig. 1) was born on November 27, 1836, at Jauer, Silezia, Poland, to Louis and Amalie Ebstein. His father was a merchant in this small town. In 1855, Wilhelm Ebstein commenced his medical study at the University of Breslau (now Wroclaw). He spent only a few months there, as he, still in 1855, moved to the much more prestigious University of Berlin. Here, he attended the medical clinic of many talented and devoted teachers, such as Traube, von Langenbeck, von Graefe, Du Bois-Reymond, Romberg, and Schoenlein. One of his most influential teachers was Rudolf Virchow (1821–1902), who returned from Würzburg to Berlin in 1856. Under Virchow's charismatic leadership, a pathologic institute was constructed on the grounds of the Charité Hospital. Ebstein devotedly attended Virchow's thorough and in-depth pathologic demonstrations. It is without doubt that Ebstein's later accurate description of the cardiac anomaly that bears his name, was closely associated with Virchow's methodology in pathology. On July 11, 1859, Ebstein graduated with a medical degree from Berlin. In 1861, he was appointed assistant physician to the Allerheiligen (All Saints’) Hospital at Breslau. In 1869, he qualified as ‘Privatdozent’, which gave him the right to lecture at the University. His career was interrupted by the Franco-Prussian war. Upon his return from the French campaign, in 1870, he was appointed medical officer to the state almshouses at Breslau. By late 1874, he was called to Göttingen (Fig. 2) as Professor of Medicine. He remained in Göttingen for the rest of his life and died there on October 22, 1912, of cerebral apoplexy. It is in Göttingen that he acquired international fame for numerous contributions to medicine. He became best known for his work on the pathology and therapy of metabolic diseases, such as diabetes mellitus, gout, obesity, alkaptonuria, cystinuria, and pentosuria [3,4]. Ebstein was a prolific writer. He published 272 articles. However, only 12 of them were related to cardiovascular diseases. Ironically, Ebstein's description of a congenital heart condition with which his name is most readily identified in the current era, was almost overlooked. This work was performed early in his career and was largely ignored until after his death. Fig. 1 Open in new tabDownload slide Wilhelm Ebstein (1836–1912). Fig. 1 Open in new tabDownload slide Wilhelm Ebstein (1836–1912). Fig. 2 Open in new tabDownload slide The Ernst August Hospital, University of Göttingen, was constructed in 1851. Wilhelm Ebstein was appointed as Professor of Medicine at this location in 1874. Fig. 2 Open in new tabDownload slide The Ernst August Hospital, University of Göttingen, was constructed in 1851. Wilhelm Ebstein was appointed as Professor of Medicine at this location in 1874. 3 Ebstein's description of Ebstein's anomaly On June 28, 1864, a 19-year-old laborer, Joseph Prescher, was admitted to the medical service of the Allerheiligen Hospital at Breslau. The history and physical examination, as documented by Dr Kornfeld, the attending physician, were very thorough and are reported in Ebstein's original article [1]. Prescher had experienced increasing dyspnea and palpitation since childhood. On physical examination, the patient was emaciated; he had extreme cyanosis of the face and neck and “marked jugular venous pulsations synchronous with the heart beat”. The sternum was completely dull to percussion from the beginning of the third rib to the xiphoid process. To the left of the sternum, cardiac dullness could not be differentiated from left lung dullness. Over the entire area of cardiac dullness, one could hear both heart sounds, which were incorporated into a murmur that started in systole and extended into diastole. This murmur was maximal at the base of the heart and was transmitted over the precordium, particularly to the right. The second sound in the pulmonary area was not accentuated. Based on these clinical findings, Kornfeld made a diagnosis of congenital cardiac defect. The patient died 8 days later on July 6, 1864. The next day, Ebstein performed an autopsy. The detailed and accurate description of his findings, illustrated by excellent drawings by his colleague Dr Wyss, are of great historical interest: “The right heart was opened from the entrance of the superior vena cava along the lateral border of the right atrium and right ventricle. The right atrium was extremely dilated and the pectinate muscles were well developed. The Eustachian valve was normally developed; the valve of the coronary sinus, or Thebesian valve, however, was absent. The fossa ovalis in the atrial septum was not completely closed. In the valve of the foramen ovale there were multiple openings....” [1]. Ebstein's examination of the right ventricle demonstrated the following findings: “The tricuspid valve was extremely abnormal in appearance. A membrane originated from a normally developed annulus fibrosus and was related to both the anterior and posterior walls of the right ventricle and blended with the posterior half of the endocardium of the ventricular septum. This membrane, together with markedly opacified and thickened posterior half of the ventricular endocardium, formed a sac. This sac, completely closed inferiorly and to the right, was opened by our incision. The sac was related to the rest of the endocardium, that is, the inner surface of the right ventricle in the following manner. From the outer surface of the membrane, both short and long, thin and thick chordae tendineae took their origin. They merged with papillary muscles, which in turn inserted on the inner wall of the right ventricle by means of either single or ramifying insertions. These chordae tendineae and papillary muscles were almost completely missing on the upper right marginal border and on the posterior part of the right ventricle; on the other hand, they were numerous in its lower and anterior portions. The membrane itself had the appearance and consistency of a fibrous sheet. It was white and glistening, in some areas very thin and diaphanous, in others somewhat thicker. It had multiple fenestrations, particularly in its lower half.... Fifteen millimeters below the annulus fibrosus, and directly under the membranous portion of the ventricular septum, a malformed leaflet originated from the endocardium. The wide base of the triangular-shaped leaflet was superior and the tip inferior. Attached to it were numerous chordae tendineae, some of which were long, thin, and tough. Most of these originated from its posterior surface, but some also from the tip of the cusp. The chordae tendineae were primarily attached to the endocardium, but a few also to a papillary muscle. This was located at the center of the ventricular septum and projected into the ventricular cavity. Into this 4 cm long papillary muscle, the anterior part of the previously mentioned membrane was inserted by means of a small upper and wide lower limb. In this manner a long oval aperture was formed, which measured 4 cm from top to bottom and 3 cm from back to front. It allowed the only entrance into the otherwise completely closed right conus arteriosus...... The right ventricular cavity was greatly enlarged. Its musculature had a thickness of 3–4 mm. The pulmonary valve and the pulmonary artery were normal....” [1]. In 1866, immediately after publishing his case report [1], Ebstein also presented it to the Schlesischen Gesellschaft für vaterländische Kultur at Breslau. His presentation was in the Verhandlungen of this Society. In 1867, Lebert, a professor of medicine at Breslau, probably was the first to refer to Ebstein's work [5]. The second case of the anomaly was described in Marxen's thesis, entitled ‘Ein seltener Fall von Anomalie der Tricuspidalisklappe’ (1886) [6]. The first description of Ebstein's anomaly in English language literature was in 1900 by MacCallum [7], then assistant and later Professor of pathology at Johns Hopkins University in Baltimore, MD. However, the eponym ‘Ebstein's disease’ was not used by these authors. Arnstein [8] is credited for introducing it to the German literature in 1927. In 1937, Yater and Shapiro [9] first used this eponym in the English language literature; the latter were the first to examine the patient by both radiography and electrocardiography. The latter investigators commented that “it would appear impossible to make the diagnosis during life” [9]. In 1951, Soloff and colleagues first reported a patient in whom the diagnosis was made during life, using cardiac catheterization and angiocardiography [10]. 4 Comment Ebstein concluded that the cardiac pathology as observed in the underlying case consisted of three components: (1) a severe malformation of the tricuspid valve; (2) absence of the Thebesian valve; (3) a patent foramen ovale. He considered the tricuspid valve anomaly to be the most important feature and established it as an error of embryogenesis, that is, a failure of delamination of the tricuspid valve leaflets. In a thorough and beautiful fashion, Ebstein described how the proximal anterior leaflet partially blended with the ventricular endocardium and how the remainder of the anterior leaflet was tethered by multiple chordae to the ventricular endocardium. In reviewing the existing literature on congenital tricuspid malformations, he concluded that his case was unique. Ebstein also concluded that the absence of the Thebesian valve played no role in the pathophysiologic condition. Although Ebstein attributed the patent foramen ovale to the incompetent tricuspid valve, he failed to realize that the opening in the atrial septum would allow a right-to-left shunt and thus cause cyanosis. This was the only major flaw in his analysis. In conclusion, Ebstein's original detailed description of the anomaly that was named after him was a continuation of the outstanding teaching and research in pathology of his mentor and teacher, Virchow. The excellent correlation of Ebstein's pathologic findings with Kornfeld's clinical notes and his hypothesis of the pathophysiology of Ebstein's anomaly make his publication a landmark in the description of a new entity. In addition, it provided a strong basis for the development of repair techniques for this rare anomaly 100 years later. The authors are immensely grateful to Dr Gordon K. Danielson, Mayo Clinic, Rochester, MN for his valuable advice and encouragement. References [1] Ebstein W. . Ueber einen sehr seltenen Fall von Insufficienz der Valvula tricuspidalis, bedingt durch eine angeborene hochgradige Missbildung derselben , Arch Anat Physiol , 1866 , vol. 7 (pg. 238 - 254 ) OpenURL Placeholder Text WorldCat [2] Mann R.J. , Lie J.T. . The life story of Wilhelm Ebstein (1836–1912) and his almost overlooked description of a congenital heart disease , Mayo Clin Proc , 1979 , vol. 54 (pg. 197 - 204 ) Google Scholar PubMed OpenURL Placeholder Text WorldCat [3] Ebstein E. . Wilhelm Ebstein's Arbeiten aus den Jahren 1859–1906 , Dtsch Arch Klin Med , 1907 , vol. 89 (pg. 367 - 378 ) OpenURL Placeholder Text WorldCat [4] Ebstein W. . Therapie des Diabetes mellitus, insbesondere über die Anwendung des salicylsauren Natron bei demselben , Berl Klin Wochenschr , 1876 , vol. 13 (pg. 337 - 340 ) OpenURL Placeholder Text WorldCat [5] Lebert H. . Ueber den Einfluss der Stenose der Conus arteriosus, des Ostium pulmonale und der Pulmonalarterie auf Entstehung von Tuberkulose , Berl Klin Wochenschr , 1867 , vol. 4 (pg. 233 - 235 ) OpenURL Placeholder Text WorldCat [6] Heigel A. . Ueber eine besondere Form von Entwicklungsstörung der Trikuspidalklappe , Virchows Arch (Pathol Anat) , 1913 , vol. 214 (pg. 301 - 319 ) Google Scholar Crossref Search ADS WorldCat [7] MacCallum W.G. . Congenital malformations of the heart as illustrated by the specimens in the pathological museum of the Johns Hopkins Hospital , Johns Hopkins Hosp Bull , 1900 , vol. 11 (pg. 69 - 71 ) OpenURL Placeholder Text WorldCat [8] Arnstein A. . Eine seltene Missbildung der Trikuspidalklappe (‘Ebsteinsche Krankheit’) , Virchows Arch (Pathol Anat) , 1927 , vol. 266 (pg. 247 - 254 ) Google Scholar Crossref Search ADS WorldCat [9] Yater W.M. , Shapiro M.J. . Congenital displacement of the tricuspid valve (Ebstein's disease): review and report of a case with electrocardiographic abnormalities and detailed histologic study of the conduction system , Ann Intern Med , 1937 , vol. 11 (pg. 1043 - 1062 ) Google Scholar Crossref Search ADS WorldCat [10] Soloff L.A. , Stauffer H.M. , Zatuchni J. . Ebstein's disease: report of the first case diagnosed during life , Am J Med Sci , 1951 , vol. 222 (pg. 554 - 561 ) Google Scholar Crossref Search ADS PubMed WorldCat © 2001 Elsevier Science B.V. All rights reserved. Elsevier Science B.V.
European Journal of Cardio-Thoracic Surgery – Oxford University Press
Published: Nov 1, 2001
Keywords: Ebstein's malformation Tricuspid valve anomaly Biographical data
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