Reverse Bernheim Phenomenon as a Cause of Exercise-Induced Syncope
A 43-year-old male was admitted after 2 episodes of exercise-induced syncopal spells during a recreational soccer match with his 2 healthy children. The patient had been diagnosed with hepatosplenic hypertension caused by chronic schistosomiasis and had undergone elective splenectomy 10 years before. On physical examination, an increased pulmonic component of the second heart sound was noted.
Schistosomiasis is endemic in more than 70 tropical and subtropical countries, affecting up to 200 million people. It is caused by skin-penetrating trematode larvae worms. The disease goes through several stages, and morbidity is often associated with granuloma formation around the schistosome eggs trapped in host tissues. Hepatosplenic schistosomiasis is the most life-threatening form and the leading cause of schistosomiasis-related mortality, but pulmonary pathology may exist because ectopic migration of schistosome eggs can reach the pulmonary beds. Chronic pulmonary schistosomiasis can present as (1) asymptomatic cases with schistosomal eggs in the pulmonary beds, with or without granuloma formation, (2) granuloma formation with pulmonary hypertension, or (3) granuloma formation with pulmonary hypertension and cor pulmonale.1,2
Patients with severe pulmonary disease are prone to develop the reverse Bernheim phenomenon, a leftward shift of the interventricular septum secondary to right ventricular pressure and volume overload, which alters left ventricular volume as well as diastolic and systolic function. This phenomenon may occur in patients with both volume and pressure overload of the right ventricle. Twelve-lead ECG (Figure 1) showed sinus rhythm, right QRS axis deviation at −90 degrees with qR pattern in V1, tall R-waves in V1-V2, and deep S-waves in V4–V6 leads, suggestive of right ventricular hypertrophy. An echocardiogram obtained at rest showed a left ventricular ejection fraction of 0.6, left atrium of 35 mm, left ventricular end-diastolic volume of 44 mm, an intraventricular septum of 8 mm with an enlarged right ventricle of 35 mm, and pulmonary artery pressure of 70 mm Hg at rest (M-mode shown in Figure 1C). A dilated pulmonary artery of 28 mm and an aorta of 20 mm, an enlarged and hypertrophic right ventricle (Figure 1B), and a leftward bulging of the intraventricular septum toward the left ventricular cavity during systole were noted at magnetic resonance imaging (Figure 1D). These findings suggest severe pulmonary hypertension.
During a stress-echo treadmill test, the patient complained of dizziness and intense fatigue. Pulmonary artery pressure of 127 mm Hg and acute dilation of the right ventricle were then noted. Because pericardial stretch cannot occur immediately to compensate for the sudden increase of right ventricular volume, it can only ensue at the expense of left ventricular cavity. In the present case, a pronounced leftward bulging of the intraventricular septum caused the left ventricle to collapse (Figure 2). Syncope would probably have occurred as a consequence of left-ventricle obliteration if the stress test had been continued.
The online-only Data Supplement, consisting of a movie file, is available with this article at http://circ.ahajournals.org/cgi/content/full/114/23/e618/DC1.