Lopez Leo, Cohen Meryl S, Anderson Robert H, Redington Andrew N, Nykanen David G, Penny Daniel J, Deanfield John E, Eidem Benjamin W
Division of Pediatric Cardiology, Children's Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, New York, United States of America.
Cardiol Young. 2010 Dec;20 Suppl 3:107-12. doi: 10.1017/S1047951110001150.
The long-term outcome of patients with congenitally malformed hearts involving abnormal right ventricular morphology and haemodynamics is variable. In most instances, the patients are at risk for right ventricular failure, in part due to morphological differences between the right and left ventricles and their response to chronic volume and pressure overload. In patients after repair of tetralogy of Fallot, and after balloon valvotomy for valvar pulmonary stenosis, pulmonary regurgitation is the most significant risk factor for right ventricular dysfunction. In patients with a dominant right ventricle after Fontan palliation, and in those with systemic right ventricles in association with surgically or congenitally corrected transposition, the right ventricle is not morphologically capable of dealing with chronic exposure to the high afterload of the systemic circulation. In patients with Ebstein's malformation of the tricuspid valve, the degree of atrialisation of the right ventricle determines how well the right ventricle will function as the pump for the pulmonary vascular bed.
患有先天性心脏畸形且右心室形态和血流动力学异常的患者的长期预后各不相同。在大多数情况下，患者存在右心室衰竭的风险，部分原因是右心室和左心室之间的形态差异以及它们对慢性容量和压力过载的反应。在法洛四联症修复术后以及肺动脉瓣狭窄球囊瓣膜切开术后的患者中，肺动脉反流是右心室功能障碍的最重要危险因素。在Fontan姑息术后右心室占优势的患者以及与手术或先天性矫正型大动脉转位相关的体循环右心室患者中，右心室在形态上无法应对长期暴露于体循环的高后负荷。在三尖瓣埃布斯坦畸形患者中，右心室心房化的程度决定了右心室作为肺血管床泵的功能状况。
