肺动脉闭锁合并室间隔缺损的临床病理分析

Pulmonary atresia with ventricular septal defect:an analysis of 233 cases

目的研究肺动脉闭锁合并室间隔缺损的解剖类型和血流动力学改变,并探讨其适宜的手术方式。方法回顾性分析233例肺动脉闭锁合并室间隔缺损的造影结果,分析肺血来源、肺动脉发育情况和合并畸形及其与手术预后的影响。结果233例中中央肺动脉存在,肺血单纯由未闭的动脉导管供应者112例(48.1%),其中1例为双侧动脉导管(0.5%);中央肺动脉和大的主-肺动脉侧支血管(MAPCA)均存在者104例(44.6%);无中央肺动脉,仅有MAPCA供应肺血者17例(7.3%)。肺动脉闭锁部位以右室流出道和瓣膜闭锁最多见(48.1%)。侧支血管的来源包括直接的主动脉-肺侧支动脉,间接的主动脉-肺侧支动脉和支气管动脉。合并畸形包括心脾综合征、房室连接不一致、心室大动脉连接不一致、多发室间隔缺损、右位主动脉弓、房间隔缺损、左上腔静脉残存、内脏异位症、上下心室、肺静脉异位引流和冠状动脉起源异常等。结论肺动脉闭锁合并室间隔缺损患者肺血来源多样化,肺动脉发育程度不一,并可合并多种心内、心外畸形,影响手术方法的选择和手术结果。

Objectives To analyze the anatomy and morphology of the pulmonary circulation of pulmonary atresia with ventricular septal defect（PA/VSD）and to study the eligible surgical approach. Methods The findings of angiography in 233 cases of PA/VSD were retrospectively studied including the type of systemic-pulmonary collateral vessels and the development of native pulmonary arteries and associated anomalies, also the influence on the consequence of operation. Results Native pulmonary arteries（NPA）were seen in 112 cases（48.1%）, and 1 case with double patent ductus arteriosus. Pulmonary blood flow was provided by both NPA and major aortopulmonary collateral arteries（MAPCA（s））in 104 cases, with only MAPCA（s）and without NPA in 17 cases. The atresia occurred at infundibular and pulmonary valve was the most common type（48.1%）. The systemic pulmonary collaterals originated from direct aortopulmonary, indirect aortopulmonary collaterals, or true bronchial arteries. The associated anomalies of PA/VSD included splenic syndrome, the atrioventricular connection discordance, the ventriculo-arterial connection discordance, multi-ventricular septal defect, right aortic arch, atrial septal defect, left superior vena cava, heterotaxia, crisscrossed heart, anomalous pulmonary venous connection and coronary arteries malformation. Conclusions The source of pulmonary blood-flow, the development of pulmonary artery, and extremely heterogeneous cardiopulmonary malformation in PA/VSD to a large extent determine the surgical approach and overall outcome.