Godart F, Houyel L, Lacour-Gayet F, Serraf A, Sousa-Uva M, Bruniaux J, Petit J, Piot J D, Binet J P, Conte S, Planché C
Department of Pediatric Cardiac Surgery, Marie Lannelongue Hospital, Paris Sud University, Le Plessis Robinson, France.
Ann Thorac Surg. 1996 Jul;62(1):136-42. doi: 10.1016/0003-4975(96)00276-7.
The operative management of absent pulmonary valve syndrome remains controversial regarding the need for pulmonary valve implantation and remodeling of pulmonary arteries. Moreover, symptomatic infants are considered to have a poor prognosis. This retrospective report summarizes the experience of a single institution.
Between May 1977 and May 1995, 37 consecutive patients underwent repair of absent pulmonary valve syndrome. Patients were divided into two groups according to age at operation: group A (10 infants less than 1 year old) and group B (27 patients older than 1 year). Mean age at operation was 5 +/- 4 months in group A and 72 +/- 42 months in group B. Initially, repair consisted of ventricular septal defect closure and relief of right ventricular outflow tract obstruction combined with pulmonary valve implantation. More recently, the concept of treatment has evolved with pulmonary arterioplasty without pulmonary valve insertion, except in patients with elevated pulmonary artery pressure.
Of the 37 patients, 34 had successful repair. The overall in-hospital mortality rate was 8% (two deaths in group A and one in group B). No hemodynamic data were correlated with operative death. Death was associated with longer extracorporeal circulation time (p = 0.005) and longer aortic cross-clamping time (p = 0.019). In fact, these were clearly related to more complex anatomy (p = 0.001): multiple ventricular septal defects in 1, left pulmonary artery arising from the ductus in another, and left pulmonary artery arising from the aorta in the remainder. Follow-up was available in 22 of the 34 survivors. Mean follow-up time was 30 +/- 47 months in group A and 38 +/- 33 months in group B. All but 1 had no restriction of exercise, and most of them had pulmonary incompetence of Doppler echocardiography. One developed severe exercise intolerance because of pulmonary valve stenosis (xenograft), leading to uneventful reoperation 123 months after initial repair. One infant died suddenly of complete atrioventricular block 3 months after repair. The late mortality rate was 5%.
Surgical treatment of absent pulmonary valve syndrome should include pulmonary arterioplasty to reduce bronchial obstruction, with no need for pulmonary valve insertion. This procedure is feasible and is recommended especially in markedly symptomatic infants.
关于肺动脉瓣缺如综合征的手术治疗，在是否需要植入肺动脉瓣以及肺动脉重塑方面仍存在争议。此外，有症状的婴儿被认为预后较差。本回顾性报告总结了一家机构的经验。
1977年5月至1995年5月期间，37例连续患者接受了肺动脉瓣缺如综合征修复术。根据手术时年龄将患者分为两组：A组（10例1岁以下婴儿）和B组（27例1岁以上患者）。A组手术时平均年龄为5±4个月，B组为72±42个月。最初，修复包括室间隔缺损闭合、右心室流出道梗阻解除以及肺动脉瓣植入。最近，治疗理念有所发展，除肺动脉压力升高的患者外，采用肺动脉成形术而不植入肺动脉瓣。
37例患者中，34例修复成功。总体住院死亡率为8%（A组2例死亡，B组1例死亡）。没有血流动力学数据与手术死亡相关。死亡与体外循环时间延长（p = 0.005）和主动脉阻断时间延长（p = 0.019）有关。事实上，这些明显与更复杂的解剖结构相关（p = 0.001）：1例有多发性室间隔缺损，另1例左肺动脉起源于动脉导管，其余患者左肺动脉起源于主动脉。34例幸存者中有22例获得随访。A组平均随访时间为30±47个月，B组为38±33个月。除1例患者外，所有患者运动均不受限，且大多数患者经多普勒超声心动图检查有肺动脉瓣关闭不全。1例因肺动脉瓣狭窄（异种移植物）出现严重运动不耐受，在初次修复后123个月进行了顺利的再次手术。1例婴儿在修复后3个月因完全性房室传导阻滞突然死亡。晚期死亡率为5%。
肺动脉瓣缺如综合征的外科治疗应包括肺动脉成形术以减轻支气管梗阻，无需植入肺动脉瓣。该手术可行，尤其推荐用于有明显症状的婴儿。
