Merrill W H, Stewart J R, Hammon J W, Boucek R J, Bender H W
Ann Thorac Surg. 1986 Sep;42(3):264-8. doi: 10.1016/s0003-4975(10)62731-2.
Pulmonary valve dysplasia is a distinct pathological entity consisting of markedly thickened, deformed, and largely immobile pulmonary valve leaflets. The clinical features and surgical management of 21 consecutive patients operated upon between 1975 and 1985 were reviewed. Mean age at operation was 26.7 months (range: 1 month to 9.5 years). Initial surgical intervention in 3 patients consisted of closed valvotomy or systemic pulmonary artery shunt. One of these patients subsequently had a partial pulmonary valvectomy; the other 2 had total valvectomy and pulmonary annuloplasty. Two patients had partial valvectomy as a primary procedure, and 1 has subsequently required total valvectomy and annuloplasty. Sixteen patients initially had total pulmonary valvectomy, 4 with a simultaneous transannular outflow tract patch. One patient subsequently required a transannular outflow tract patch. Therefore, definitive repair consisted of partial valvectomy in 2 patients, total valvectomy in 19, and a transannular outflow tract patch in 9 patients with a hypoplastic pulmonary annulus. There were no operative deaths, and all patients are asymptomatic at a mean follow-up of 37.9 months. Five patients who underwent total valvectomy were catheterized postoperatively. The mean residual gradient was 25 mm Hg. Pulmonary valvectomy appears to provide both effective relief of stenosis and long-term asymptomatic status in patients with pulmonary valve dysplasia. Total excision of all valve leaflets is recommended. Careful consideration should be given to a simultaneous transannular outflow tract patch if the annulus is hypoplastic.
肺动脉瓣发育异常是一种独特的病理实体，其特征为肺动脉瓣叶明显增厚、变形且大多活动受限。回顾了1975年至1985年间连续接受手术的21例患者的临床特征及手术治疗情况。手术时的平均年龄为26.7个月（范围：1个月至9.5岁）。3例患者最初的手术干预包括闭式瓣膜切开术或体肺分流术。其中1例患者随后接受了部分肺动脉瓣切除术；另外2例进行了全瓣膜切除术及肺动脉瓣环成形术。2例患者将部分瓣膜切除术作为主要手术，其中1例随后需要进行全瓣膜切除术及瓣环成形术。16例患者最初接受了全肺动脉瓣切除术，4例同时进行了跨瓣环流出道补片修补术。1例患者随后需要进行跨瓣环流出道补片修补术。因此，确切的修复方法包括2例患者行部分瓣膜切除术，19例患者行全瓣膜切除术，9例肺动脉瓣环发育不良的患者行跨瓣环流出道补片修补术。无手术死亡病例，所有患者在平均37.9个月的随访期内均无症状。5例接受全瓣膜切除术的患者术后接受了心导管检查。平均残余压差为25 mmHg。肺动脉瓣切除术似乎能有效缓解肺动脉瓣发育异常患者的狭窄并使其长期保持无症状状态。建议彻底切除所有瓣膜叶。如果瓣环发育不良，应仔细考虑同时进行跨瓣环流出道补片修补术。
