Wasserman Evan, Wulff Luis, Bandyopadhyay Debabrata, Restrepo-Jaramillo Ricardo, Qureshi Raheel, Herazo-Maya Jose, Islam Monirul
University of South Florida, Geisinger Health, United States.
Respir Med Case Rep. 2025 Jul 1;57:102250. doi: 10.1016/j.rmcr.2025.102250. eCollection 2025.
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.
一名70岁男性因进行性呼吸困难和运动诱发的晕厥发作入院。他既往有射血分数保留的心力衰竭（HFpEF）和肺动脉高压（PAH）病史。他接受了安立生坦、西地那非和司来帕格治疗。由于症状进展，他接受了右心导管检查（RHC）。与之前相比，结果显示平均肺动脉压（mPAP）有所改善，但肺毛细血管楔压（PCWP）恶化。因此，他停用了司来帕格，继续使用其他药物。尽管如此，他的症状仍继续恶化。静息超声心动图显示左心室（LV）收缩功能正常，右心室（RV）扩张且功能障碍；未检测到房间隔分流。在负荷超声心动图检查期间，他发生了一次晕厥，伴有低血压和氧饱和度下降。图像证实运动峰值时右心室显著扩张，同时室间隔向左膨出，导致左心室流出道（LVOT）受压——“反向伯恩海姆现象”。休息时右心室大小和功能迅速恢复。随后，患者的PAH治疗升级，加用了静脉注射曲前列尼尔，这导致运动能力改善和晕厥发作消失。重复负荷超声心动图显示急性运动诱发的右心室扩张和室间隔膨出消失，未压迫左心室流出道。
