介入及手术联合矫治伴有体肺动脉侧支的肺血减少型先天性心脏病

目的评价介入及手术联合技术矫治伴有体肺动脉侧枝的肺血减少型先天性心脏病的临床应用价值。方法1992年11月至2003年10月，介人和手术联合治疗伴有体肺侧枝的肺血减少型先天性心脏病15例。12例侧支栓堵后行外科矫治术，3例外科矫治术后行侧支栓堵。心血管造影诊断体肺侧支血管46支。行介人栓堵35支，外科结扎体肺侧支1支，未予介人栓堵的体肺侧支10支。结果本组死亡4例，死亡率26．7％。侧支栓堵后行外科矫治术12例，术后死亡3例，死因分别是低心排综合征、肺出血、肺梗死。术后并发广泛肺部渗出、右心功能不全各1例；7例顺利痊愈。外科矫治术后行侧支栓堵3例，死亡1例，死因为栓堵术后残留粗大体肺侧支引起的充血性心力衰竭。1例矫治术后造影发现4支体肺侧支，栓堵其中3支，患儿痊愈出院；另1例矫治术后造影发现4支体肺侧支，栓堵2支体肺侧支，另外2支无法栓堵，予再次手术结扎后痊愈。结论介人栓堵体肺侧支与心内矫治手术联合治疗伴有体肺侧枝肺血减少型先天性心脏病能提高一期根治的成功率和减少手术对患儿的创伤。对于单独向肺段供血的大的体肺侧支，不宜介入栓堵，应行肺动脉融合术，以免术后肺梗死。

An integral approach for cyanotic congenital heart disease with major aortopulmonary collateral arteries

Objective To demonstrate the feasibility of combined collateral embolization with surgical repair for tetralogy of Fallot or pulmonary atresia with major aorta pulmonary collateral arteries. Methods The clinical, catheterization and surgical data of 15 such patients from November 1992 to September 2003 were analyzed retrospectively. Results All 15 patients underwent combined therapy of najor-aorto pulmonary collateral arteriesl (MAPCAs) occlusion and complete repair with a mortality of 26.7% . Of the 46 collateral arteries, 35 were occluded with a successful rate of 90.6%. Embolization was performed before corrective surgery in 12 patients. The causes of early death in three patients were low cardiac output syndrome, pulmonary hemorrhage, and pulmonary infarction respectively. Complications included extensive pulmonary infiltration in 1 patient and right ventricular dysfunction in another patient. The other 7 patients recovered smoothly with the arterial oxygen saturation greater than 97% at discharge. Embolization was performed after corrective surgery in 3 patients with postoperative congestive heart failure. One patient died of congestive heart failure. The symptoms and signs of heart failure was significently relieved in the other two cases. Conclusions In selective group of patients with dual pulmonary blood supply by both MAPCA and native pulmonary arteries, this combined therapy of collateral embolization and surgical repair simplifies complete surgical operation and enhances surgical results. It is necessary to minimize the error in distinguish sole pulmonary blood supply from dual pulmonary blood supply to avoid postoperative pulmonary infarction. Embolization after surgical correction is an important rescue measurement to congestive heart failure.