Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College; Beijing-China.
Anatol J Cardiol. 2021 Jul;25(7):476-483. doi: 10.5152/AnatolJCardiol.2021.98537.
Severe right ventricular hypertrophy (SRVH) in hypertrophic cardiomyopathy (HCM) is rare. We studied the clinical characteristics and prognosis of 36 patients with HCM and SRVH in a Chinese cohort.
Patients with HCM and SRVH were enrolled between 2013 and 2017. The clinical characteristics, treatment therapies, and clinical outcomes of the 36 patients were retrospectively studied and compared with those of 128 patients without SRVH.
Patients in the group with SRVH were younger than those in the group without SRVH (27.58±15.09 years vs 40.34±13.21 years, respectively; p<0.001). Patients with SRVH had more serious clinical symptoms and a higher New York Heart Association functional class than those without SRVH. Most patients in the group with SRVH exhibited diffuse RV hypertrophy, and 13 patients presented with biventricular outflow tract obstruction. Maximal left ventricular (LV) wall thickness (27.29±7.95 mm vs 24.33±5.85 mm, respectively; p=0.027) and LV outflow tract gradient (80.83±24.41 mm Hg vs 42.3±5.7 mm Hg, respectively; p=0.000) were significantly greater in patients with SRVH than in those without SRVH. A total of 30 patients in the group with SRVH underwent surgical correction. During a median follow-up period of 48 months, six patients with SRVH reached primary clinical endpoints (four sudden cardiac deaths, one heart failure-related death, and one heart transplantation), whereas only two deaths occurred in the patients without SRVH.
We conclude that patients with HCM and SRVH exhibit serious symptoms and have complex surgical requirements and poor clinical outcomes.
肥厚型心肌病（HCM）中严重右心室肥厚（SRVH）较为罕见。我们在中国人群中研究了 36 例 HCM 合并 SRVH 患者的临床特征和预后。
2013 年至 2017 年间，我们纳入了 HCM 合并 SRVH 的患者。回顾性研究了 36 例患者的临床特征、治疗方法和临床结局，并与 128 例无 SRVH 的患者进行了比较。
与无 SRVH 组相比，SRVH 组患者年龄更小（27.58±15.09 岁比 40.34±13.21 岁，p<0.001）。SRVH 组患者的临床症状更严重，纽约心脏协会心功能分级更高。大多数 SRVH 组患者表现为右心室弥漫性肥厚，13 例患者表现为双心室流出道梗阻。SRVH 组患者的左心室（LV）壁最厚处厚度（27.29±7.95 mm 比 24.33±5.85 mm，p=0.027）和 LV 流出道梯度（80.83±24.41 mm Hg 比 42.3±5.7 mm Hg，p=0.000）显著大于无 SRVH 组患者。共有 30 例 SRVH 患者接受了手术矫正。在中位数为 48 个月的随访期间，有 6 例 SRVH 患者达到了主要临床终点（4 例心源性猝死，1 例心力衰竭相关死亡，1 例心脏移植），而无 SRVH 组仅发生了 2 例死亡。
我们的结论是，HCM 合并 SRVH 的患者表现出严重的症状，且手术要求复杂，临床结局较差。
