Abstract

Objectives. In current guidelines, hypertrophic cardiomyopathy (HCM) is defined by hypertrophy of the left ventricle (LV). Less attention has been given to the right ventricle (RV) in patients with HCM. We wanted to provide an overview of current literature on RV involvement in HCM. Design. We performed a systematic search in PubMed and added additional articles by manual screening of references. The quality of the articles was assessed according to the GRADE system. Results. We identified 35 original articles on RV involvement in HCM. Based on these publications, RV hypertrophy occurs in 28–44% of HCM patients, depending on the cut-off value for hypertrophy and the method for assessment. Histological studies show the same structural changes in RV as are typically described in the LV cardiomyocyte hypertrophy and disarray, as well as fibrosis. These changes are similar, but less pronounced in the RV than in the LV. We discuss how HCM can impact the RV, either through a primary involvement similar to the LV or secondary to hemodynamic effects resulting from LV dysfunction. RV dysfunction in HCM is associated with higher mortality, partly due to an increased risk of ventricular tachycardia and sudden cardiac death. Conclusions. The evidence for RV involvement in HCM is limited. Multimodal imaging assessment of the RV should be included in the work-up of patients with HCM, and the added value of including RV function in the risk stratification algorithm should be further explored.

摘要

目标。在当前的指南中，肥厚型心肌病 (HCM) 的定义是左心室 (LV) 肥大。对 HCM 患者的右心室 (RV) 的关注较少。我们想提供关于 RV 参与 HCM 的当前文献的概述。设计。我们在 PubMed 中进行了系统搜索，并通过手动筛选参考文献添加了其他文章。文章的质量根据GRADE系统进行评估。结果。我们确定了 35 篇关于 RV 参与 HCM 的原创文章。根据这些出版物，28-44% 的 HCM 患者发生 RV 肥大，具体取决于肥大的临界值和评估方法。组织学研究显示 RV 的结构变化与 LV 心肌细胞肥大和紊乱以及纤维化中通常描述的相同。这些变化是相似的，但在 RV 中不如在 LV 中明显。我们讨论了 HCM 如何影响 RV，通过类似于 LV 的主要受累或继发于 LV 功能障碍导致的血流动力学影响。HCM 的 RV 功能障碍与较高的死亡率相关，部分原因是室性心动过速和心源性猝死的风险增加。结论。RV 参与 HCM 的证据有限。RV 的多模态成像评估应包括在 HCM 患者的检查中，并应进一步探索将 RV 功能纳入风险分层算法的附加值。