Second Department of Medicine, University of Toyama, Toyama 930-0194, Japan.
Medicina (Kaunas). 2023 Feb 18;59(2):401. doi: 10.3390/medicina59020401.
Combined cases of hypertrophic obstructive cardiomyopathy (HOCM) and pulmonary arterial hypertension (PAH) are rare and have a management dilemma. Although preload is crucial in the management of HOCM, anti-PAH agents dramatically change the preload, leading to improving or worsening heart failure in patients with HOCM. We had a 74-year-old woman with Sjogren-syndrome-associated PAH. Her heart failure worsened following the initiation of anti-PAH agents due to an incremental preload on the left ventricle, whereas HOCM clinically developed following the termination of anti-PAH agents and progressing anorexia due to the progression of the left ventricular outflow obstruction. Careful monitoring of the left ventricular outflow obstruction during initiation/termination of anti-PAH agents and medical intervention to the HOCM are highly recommended.
肥厚型梗阻性心肌病（HOCM）和肺动脉高压（PAH）合并病例较为罕见，治疗管理存在困境。尽管前负荷在 HOCM 的管理中至关重要，但抗 PAH 药物会显著改变前负荷，导致 HOCM 患者心力衰竭改善或恶化。我们曾有一位 74 岁的女性患有干燥综合征相关的 PAH。由于左心室前负荷增加，她在开始使用抗 PAH 药物后心力衰竭恶化，而 HOCM 在抗 PAH 药物停药后临床发作，并由于左心室流出道梗阻加重导致进行性厌食。强烈建议在开始/停止使用抗 PAH 药物期间仔细监测左心室流出道梗阻，并对 HOCM 进行医疗干预。
