右心-肺循环单位在心肌病和贮积病中的作用

Right Heart-Pulmonary Circulation Unit in Cardiomyopathies and Storage Diseases.

机构信息

Department of Cardiology, Luigi Vanvitelli University, Monaldi Hospital, AORN Ospedali dei Colli, Via Bianchi, Naples 80100, Italy.

出版信息

Heart Fail Clin. 2018 Jul;14(3):311-326. doi: 10.1016/j.hfc.2018.03.001.

DOI:10.1016/j.hfc.2018.03.001

PMID:29966629

Abstract

Cardiomyopathies (CM) are a heterogeneous group of muscle heart diseases, divided into 3 main categories (dilated, hypertrophic, and restrictive). In addition to these subgroups, athlete's heart and hypertensive cardiopathy are both the result of heart adaptation to increased loading conditions, making it possible to include them in the CM group. Right heart involvement is clear in some CM as arrhythmogenic CM, carcinoid syndrome, and endomyocardial fibrosis, whereas in others, like hypertrophic or dilated CM, it is known that the right heart has a prognostic impact but less clear is its pathogenic role.

摘要

心肌病（CM）是一组异质性的肌肉心脏疾病，分为 3 个主要类别（扩张型、肥厚型和限制型）。除了这些亚组之外，运动员心脏和高血压性心脏病都是心脏对增加的负荷条件适应的结果，因此可以将它们归入 CM 组。在一些心肌病中，如心律失常性心肌病、类癌综合征和心内膜心肌纤维化，右心受累是明确的，而在其他心肌病中，如肥厚型或扩张型心肌病，已知右心对预后有影响，但右心的致病作用则不太明确。