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目的:介绍采用传统的外科手术与杂交技术治疗先天性重症孤立性右心室流出道梗阻11年的临床经验。方法:我院1998-01至2009-12治疗先天性重症孤立性右心室流出道梗阻小婴儿127例,年龄2天～12个月,平均年龄(5.7±0.3)个月,其中新生儿18例。127例患儿按照治疗方法和肺动脉瓣病变情况分组。将75例传统外科手术患儿,分为肺动脉瓣闭锁外科手术组(A1组,13例),重度肺动脉瓣狭窄(PS)外科手术组(A2组,62例);将52例杂交技术患儿,分为肺动脉瓣闭锁杂交技术组(B1组,25例),重度PS杂交技术组(B2组,27例)。并对住院和随访结果进行统计。结果:127例患儿住院死亡12例(A1组4例,A2组8例,B1、B2组均为0)。存活出院的115例患者中,可随访到100例患者,平均随访时间24.1±26.4(1～102)个月。杂交技术有5例远期死亡,传统外科手术无远期死亡。结论:开胸经心室肺动脉瓣球囊成形术治疗孤立性右心室流出道梗阻可提高围术期的生存率,降低并发症,为远期双心室矫治创造条件。同期加行改良体肺分流,对新生儿和右心室发育较差的患儿是重要选择策略。
Objective: To introduce the clinical experience of using traditional surgery and hybridization in the treatment of congenital severe isolated right ventricular outflow tract obstruction for 11 years. Methods: From January 1998 to December 2009, 127 small infants with obstructive congenital severe isolated right ventricular outflow tract obstruction were treated in our hospital from January to December 2009, with a mean age of (5.7 ± 0.3) months between 2 days and 12 months. Among them, 18 . 127 cases of children in accordance with the treatment and pulmonary valve disease grouping. Seventy-five children with conventional surgery were divided into three groups: group A1 (n = 13), group PS (group A2), and group A2 (n = 62) , Divided into pulmonary valve atresia hybridization group (B1 group, 25 cases), severe PS hybridization group (B2 group, 27 cases). And statistics of hospitalization and follow-up results. RESULTS: A total of 127 children were hospitalized in 12 patients (4 in group A1, 8 in group A2 and 0 in group B1 and B2). Of the 115 patients who survived the discharge, 100 patients were followed up with an average follow-up of 24.1 ± 26.4 (1 to 102) months. Five cases of long-term hybridization death, traditional surgery without long-term death. Conclusion: Thoracotomy and pulmonary valvuloplasty in the treatment of isolated right ventricular outflow tract obstruction can improve the perioperative survival rate, reduce complications and create conditions for long-term biventricular correction. Simultaneous addition of modified pulmonary bypass, neonatal and right ventricular dysplasia in children is an important selection strategy.