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In 1910, Hippolyte Bernheim (1840-1919) () described 10 patients with signs and symptoms of right-sided heart failure, and necropsy in each disclosed a thick left ventricular free wall and ventricular septum with the latter bulging into the right ventricular cavity (1). He titled this original article "Venous asystole in hypertrophy of the left heart with associated stenosis of the right ventricle." In that article Bernheim included two drawings, one of a normal heart and one with a thickened left ventricular wall with the thick ventricular septum protruding toward the right ventricular cavity (). In 1915, Bernheim published a similar article (2), this time titling it "Right ventricular stenosis caused by displacement of the septum in eccentric hypertrophy of the left ventricle and resulting venous asystole."
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Figure 1.
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Figure 2.
During the next century, a number of articles appeared describing "the Bernheim syndrome." Although Bernheim described 10 cases in his original article, most subsequent articles have been only case reports (3-11), with few exceptions (1, 12-22). Most subsequent articles were necropsy studies, and most supported the concept of the Bernheim syndrome's being a real entity. There were exceptions. Evans and White (12) () studied 33 patients at necropsy with considerable left ventricular hypertrophy (heart weight >750 g) and then reviewed their medical records to determine whether signs and symptoms of right-sided heart failure were isolated or simply preceded signs of left-sided heart failure (dyspnea). These authors found
no instance ... of isolated early signs or symptoms of right-sided failure ... [and] concluded from ... [their] analysis, as well as from prior experience, that ... [they had] yet to encounter any unquestionable case of so-called Bernheim's syndrome ... [and that] it would appear sensible to drop this designation.
In 1955, Selzer and colleagues (6) () described a 62-year-old man with severe hypertension (blood pressure 260/140 mm Hg) hospitalized because of nausea, hepatomegaly, distended neck veins, and clear lung fields. Right-sided cardiac catheterization disclosed identical peak systolic pulmonary arterial and right-ventricular...