Yoshimura Naoki, Yamaguchi Masahiro, Ohashi Hidetaka, Oshima Yoshihiro, Oka Shigeteru, Yoshida Masahiro, Murakami Hirohisa, Tei Teruo
Department of Cardiothoracic Surgery, Kobe Children's Hospital, Japan.
J Thorac Cardiovasc Surg. 2003 Nov;126(5):1417-26. doi: 10.1016/s0022-5223(03)01035-3.
Management strategy for pulmonary atresia with intact ventricular septum is controversial. We treated patients with this anomaly according to a management protocol based on our quantitative assessment of right ventricular morphology (index of right ventricular development and right ventricle-tricuspid valve index). In the present study, we reviewed a 20-year experience of the surgical treatment at our institution to define guidelines for selecting the appropriate type of surgical procedure.
Between April 1981 and June 2002, 45 consecutive patients with pulmonary atresia with intact ventricular septum underwent surgical treatment. Open transpulmonary valvotomy was performed in 27 patients and Blalock-Taussig shunt in 18 patients as the initial palliative procedure. Three patients who underwent a successful transpulmonary valvotomy alone in the neonatal period required no further operation. Definitive repair was performed in 32 patients. Biventricular repair was performed on 19 patients, one and a half ventricular repair in 3, and Fontan-type operation in 10.
There were 1 early and 2 late deaths before the definitive operation in patients who underwent transpulmonary valvotomy. Two patients who had coronary artery interruption died 3 months and 13 years after the initial Blalock-Taussig shunt. There were 1 early and 2 late deaths after the definitive operation. Actuarial survival, including noncardiac death, was 91.1% at 5 years after the initial procedure and 81.5% at 10 years.
We treated patients with pulmonary atresia with intact ventricular septum according to a management protocol based on our quantitative assessment of right ventricular morphology with good results.
室间隔完整的肺动脉闭锁的治疗策略存在争议。我们根据对右心室形态的定量评估（右心室发育指数和右心室-三尖瓣指数）制定的管理方案来治疗此类异常患者。在本研究中，我们回顾了本机构20年的手术治疗经验，以确定选择合适手术方式的指导原则。
1981年4月至2002年6月，45例连续的室间隔完整的肺动脉闭锁患者接受了手术治疗。27例患者行开放性经肺动脉瓣膜切开术，18例患者行Blalock-Taussig分流术作为初始姑息性手术。3例在新生儿期单独成功行肺动脉瓣膜切开术的患者无需进一步手术。32例患者进行了根治性修复。19例患者进行了双心室修复，3例进行了一个半心室修复，10例进行了Fontan类手术。
接受肺动脉瓣膜切开术的患者在根治性手术前有1例早期死亡和2例晚期死亡。2例冠状动脉中断的患者在初次Blalock-Taussig分流术后3个月和13年死亡。根治性手术后有1例早期死亡和2例晚期死亡。包括非心脏死亡在内的实际生存率在初次手术后5年时为91.1%，10年时为81.5%。
我们根据基于对右心室形态定量评估的管理方案治疗室间隔完整的肺动脉闭锁患者，取得了良好的效果。
