The myth of the Bernheim syndrome

Abstract

The Bernheim syndrome has been a topic of discussion for over a century. It has been reported to be caused by severe rightward movement of the ventricular septum resulting in compression of the right ventricular cavity leading to right-sided heart failure without pulmonary congestion. Hemodynamic findings have been described in a few patients with the so-called Bernheim syndrome. We describe a patient in whom the ventricular septum dramatically decreased the size of the right ventricular cavity and yet peak systolic pressures in both the right ventricle and pulmonary trunk were identical. Thus, it is difficult to view the Bernheim syndrome as a real entity.

Figure 1.

Portraits of (a) Hippolyte Bernheim, MD, (b) Paul D. White, MD, and (c) Arthur Selzer, MD.

Figure 2.

Illustration in Hippolyte Bernheim's original article (1): (a) Bernheim syndrome and (b) normal heart.

Figure 3.

Cross-section of the cardiac ventricles in a 62-year-old man reported by Selzer et al (6). LV indicates left ventricle; VS, ventricular septum; RV, right ventricle.

Figure 4.

Hemodynamic tracings from cardiac catheterization in our patient: (a) right atrium; (b) pulmonary artery; (c) right ventricle, and (d) pulmonary capillary wedge pressure, all in mm Hg.

Figure 5.

Explanted basal portion of the heart of our patient. The arrow shows a slit-like right ventricular (RV) cavity. The left ventricular (LV) cavity is severely dilated, and its wall is focally scarred. The ventricular septum (VS) protrudes severely toward the right ventricular cavity.

Figure 6.

Explanted 1-cm thick slices of the ventricles caudal to the basal portion of the heart in our patient. The arrows show the extremely small right ventricular (RV) cavity. LV indicates left ventricle; VS, ventricular septum.