Cavalini J F, Aiello V D, Guedes de Souza P, Trevisan I V, Marcial M B, Ebaid M
Division of Pediatric Cardiology, Instituto do Coração do Hospital das Clínicas da FMUSP, Avenida Dr. Enéas de Carvalho Aguiar, 44, CEP 05403-000, São Paulo, SP-Brazil.
Pediatr Cardiol. 1998 Nov-Dec;19(6):490-4. doi: 10.1007/s002469900367.
Two cases of double outlet right ventricle with restrictive ventricular septal defect are described. This is an uncommon presentation that causes left ventricular dysfunction because of left ventricular outflow tract obstruction. The presence of an intact atrial septum leads to severe pulmonary hypertension, which tends to aggravate the right ventricular output. In the presence of a normal left ventricle, the authors suggest the possibility of enlargement of the ventricular septal defect in order to perform a biventricular repair. The association of a supramitral valve ring in both cases, and the isolation of the left subclavian artery and an aortopulmonary fenestration in one of these cases, are also discussed. In addition we explore factors that cause restrictive ventricular septal defects as well as the mechanisms that may lead to spontaneous closure of ventricular septal defect in a double outlet right ventricle.
本文描述了两例右心室双出口合并限制性室间隔缺损的病例。这是一种不常见的表现形式，由于左心室流出道梗阻导致左心室功能障碍。完整的房间隔的存在会导致严重的肺动脉高压，这往往会加重右心室输出。在左心室正常的情况下，作者建议扩大室间隔缺损以进行双心室修复的可能性。还讨论了两例病例中均存在二尖瓣上瓣膜环，以及其中一例病例中左锁骨下动脉的分离和主肺动脉窗的情况。此外，我们探讨了导致限制性室间隔缺损的因素以及右心室双出口中室间隔缺损可能自发闭合的机制。
