Dudell G G, Evans M L, Krous H F, Spicer R L, Lamberti J J
Division of Neonatology, Children's Hospital, San Diego, CA 92123.
Pediatrics. 1993 Feb;91(2):403-10.
Common pulmonary vein atresia is a rare form of cyanotic congenital heart disease in which the pulmonary veins join to form a blind confluence that does not communicate with the heart or the major systemic veins. Twenty-one cases have been reported since the lesion was first described in 1962; only two patients with this lesion have survived. Over a 4-year period, common pulmonary vein atresia was diagnosed in five newborns referred to the San Diego Regional Extracorporeal Membrane Oxygenation Program. All five improved dramatically as a result of venoarterial bypass. Congenital heart disease was diagnosed at autopsy in the initial case and by cardiac ultrasound and/or catheterization in the others. Surgical repair was attempted in three neonates; all three required continued extracorporeal membrane oxygenation support postoperatively because of pulmonary hypertension and severe pulmonary parenchymal disease. One infant died of respiratory insufficiency at 3 months of age. The other two survived and were discharged from the hospital. The diagnostic and therapeutic dilemmas posed by this lesion and the life-saving potential for extracorporeal membrane oxygenation in this rapidly fatal cardiac anomaly are the bases of this report.
共同肺静脉闭锁是一种罕见的青紫型先天性心脏病，其肺静脉汇合形成一个盲端，不与心脏或主要体静脉相通。自1962年首次描述该病变以来，已报告21例；仅有两名患有此病变的患者存活。在4年期间，圣地亚哥地区体外膜肺氧合项目转诊的5例新生儿被诊断为共同肺静脉闭锁。由于动静脉旁路，所有5例病情均显著改善。最初1例通过尸检诊断为先天性心脏病，其他病例通过心脏超声和/或心导管检查诊断。3例新生儿尝试进行手术修复；由于肺动脉高压和严重的肺实质疾病，所有3例术后均需要持续的体外膜肺氧合支持。1例婴儿在3个月时死于呼吸功能不全。另外2例存活并出院。本报告基于该病变带来的诊断和治疗困境以及体外膜肺氧合对这种快速致命性心脏畸形的挽救生命潜力。
