Fragata José, Magalhães Manuel, Baquero Luis, Trigo Conceição, Pinto Fátima, Fragata Isabel
Department Cardiothoracic Surgery, Congenital Heart Program, Hospital de Santa Marta and Chair of Surgery, FCM, NOVA University, Lisbon, Portugal.
World J Pediatr Congenit Heart Surg. 2013 Jan;4(1):44-9. doi: 10.1177/2150135112460250.
Partial anomalous pulmonary venous connections (PAPVCs) are a heterogeneous group of congenital heart lesions in which at least one pulmonary vein will drain into the systemic venous system. The consequences are a variable left-to-right hemodynamic shunt and more rarely pulmonary artery hypertension. Often, PAPVC occurs in association with other congenital cardiac malformations. Surgical correction is most often advisable and is generally straightforward and simple to achieve. Historically, some repairs have included incision across the junction of the superior vena cava with the right atrium, which can lead to late arrhythmias. The Warden technique avoids incision across the atriocaval junction. Neonates and infants with Scimitar syndrome represent the most challenging subset of patients with PAPVC.
部分性肺静脉异位连接(PAPVCs)是一组异质性先天性心脏病变,其中至少有一条肺静脉引流至体静脉系统。其后果是出现程度不一的左向右血流动力学分流,较少见的是肺动脉高压。PAPVC常常与其他先天性心脏畸形合并存在。手术矫正通常是可取的,而且一般操作直接、简单。过去,一些修复手术包括切开上腔静脉与右心房的交界处,这可能导致晚期心律失常。沃登技术避免切开房室交界处。弯刀综合征的新生儿和婴儿是PAPVC患者中最具挑战性的亚组。