Reverse Bernheim Phenomenon - A True Enigma

A 70-year-old man was admitted with progressive dyspnea and exercise-induced syncopal episodes. His past medical history was significant for heart failure with preserved ejection fraction (HFpEF) and pulmonary arterial hypertension (PAH). He was treated with ambrisentan, sildenafil and selexipag. Due to progressive symptoms, he underwent right heart catheterization (RHC). Compared to his prior, this showed improvement in mean pulmonary arterial pressure (mPAP) but worsened pulmonary capillary wedge pressure (PCWP). He was therefore titrated off selexipag and continued on other medications. Despite this his symptoms continued to worsen. Resting echocardiography demonstrated normal left ventricular (LV) systolic function with right ventricle (RV) dilatation and dysfunction; no shunt across the interatrial septum was detected. During a stress echocardiogram he had an episode of syncope with associated hypotension and oxygen desaturation. The images confirmed dramatic RV dilatation at peak exercise along with leftward bulging of the septum leading to compression of the left ventricular outflow tract (LVOT) - "the reverse Bernheim phenomenon". The RV size and function recovered rapidly at rest. The patient's PAH therapy was subsequently escalated with addition of intravenous treprostinil, which led to improvement of exercise capacity and resolution of syncopal episodes. A repeat stress echocardiogram revealed resolution of the acute exercise-induced RV dilatation and septal bulging without compressing the LVOT.