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肥厚型心肌病的右心室受累:当前文献中的证据和意义。

Right ventricular involvement in hypertrophic cardiomyopathy: evidence and implications from current literature.

机构信息

Institute for Experimental Medical Research, Oslo University Hospital, University of Oslo, Oslo, Norway.

KG Jebsen Cardiac Research Centre, University of Oslo, Oslo, Norway.

出版信息

Scand Cardiovasc J. 2021 Aug;55(4):195-204. doi: 10.1080/14017431.2021.1901979. Epub 2021 Mar 24.

DOI:10.1080/14017431.2021.1901979
PMID:33759664
Abstract

. 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. 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. 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. 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

. 在现行指南中,肥厚型心肌病(HCM)定义为左心室(LV)肥厚。在 HCM 患者中,右心室(RV)较少受到关注。我们希望提供一份关于 HCM 中 RV 受累的当前文献综述。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。根据 GRADE 系统评估文章的质量。我们确定了 35 篇关于 HCM 中 RV 受累的原始文章。基于这些出版物,RV 肥厚发生在 28-44%的 HCM 患者中,具体取决于肥厚的截止值和评估方法。组织学研究显示 RV 中的结构变化与 LV 心肌细胞肥厚和排列紊乱以及纤维化相同。这些变化在 RV 中与 LV 相似,但程度较轻。我们讨论了 HCM 如何通过类似于 LV 的原发性受累或继发于 LV 功能障碍引起的血流动力学效应来影响 RV。HCM 中的 RV 功能障碍与更高的死亡率相关,部分原因是室性心动过速和心脏性猝死的风险增加。HCM 中 RV 受累的证据有限。应将 RV 的多模态成像评估纳入 HCM 患者的检查中,并应进一步探讨将 RV 功能纳入风险分层算法的附加值。

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