Department of Cardiology, Academic Medical Center, Meibergdreef 9, 1055 AZ, Amsterdam, The Netherlands.
Department of Anatomy, Embryology, and Physiology, Academic Medical Center, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands.
Cardiovasc Res. 2017 Oct 1;113(12):1509-1520. doi: 10.1093/cvr/cvx155.
Congenital heart disease is a major health issue, accounting for a third of all congenital defects. Improved early surgical management has led to a growing population of adults with congenital heart disease, including patients with defects affecting the right ventricle, which are often classified as severe. Defects affecting the right ventricle often cause right ventricular volume or pressure overload and affected patients are at high risk for complications such as heart failure and sudden death. Recent insights into the developmental mechanisms and distinct developmental origins of the left ventricle, right ventricle, and the outflow tract have shed light on the common features and distinct problems arising in specific defects. Here, we provide a comprehensive overview of the current knowledge on the development into the normal and congenitally malformed right heart and the clinical consequences of several congenital heart defects affecting the right ventricle.
先天性心脏病是一个主要的健康问题，占所有先天性缺陷的三分之一。早期手术管理的改进导致越来越多的先天性心脏病成年人，包括影响右心室的患者，这些患者通常被归类为严重。影响右心室的缺陷常导致右心室容量或压力超负荷，受影响的患者有心力衰竭和猝死等并发症的高风险。最近对左心室、右心室和流出道的发育机制和不同发育起源的深入了解，揭示了特定缺陷中常见的特征和不同的问题。在这里，我们提供了一个关于正常和先天性畸形右心发育的综合概述，以及几种影响右心室的先天性心脏病的临床后果。
