主-肺动脉间隔缺损的外科治疗(附11例报道)

Surgical management of aortopulmonary septal defect (11 cases report)

目的 总结 11例先天性主 肺动脉间隔缺损 (APSD)术前诊断的经验教训和外科治疗效果。方法 自 1992年 2月到 2 0 0 0年 2月我院共收治 11例先天性主 肺动脉间隔缺损患者 ,年龄 1.5～ 13岁 ,平均 (5 .9± 4.1)岁。其中Ⅰ型APSD7例 ,Ⅱ型 4例。合并右室双出口 ,主动脉瓣下隔膜 ,二尖瓣关闭不全 ,室间隔缺损 ,法鲁四联症和主动脉右弓右降 ,主动脉弓中断和动脉导管未闭各一例。 3例入院前曾误诊为动脉导管未闭 ,有左后外侧开胸史。 8例采用补片修补缺损 ,3例直接缝合。 10例经主动脉切口修补 ,1例经肺动脉切口修补。结果 术后早、晚期死亡各一例 ,分别为合并法鲁四联症 ,主动脉弓中断和动脉导管未闭的患者。 8例随访 1～ 78个月 ,平均 43.2个月 ,疗效满意。结论主 肺动脉间隔缺损是一种易被误诊为动脉导管未闭的少见先天性心脏病 ,早期手术治疗效果良好 。

Objective To review the experience in diagnosis and surgical treatment of aortopulmonary septal defect. Methods Eleven patients with aortopulmonary septal defect underwent operative repair from February 1992 to February 2000, in whom 7 had typeⅠ,4 had typeⅡ. Age ranged from 1.5 to 13 (mean 5.9±4.1) years. Six patients associated with other cardiac defects, including double outlets of right ventricle, subaortic valvular membranous ring, mitral valve insufficiency, vetricular septal defect, tetralogy of Fallot with right aortic arch, and interrupted aortic arch with patent ductus arteriosus, respectively. Three patients had a history of left posterolateral thoracotomy with the misdiagnosis of patent ductus arteriosus. Operative technique included patch of the defect in 8 patients using various approaches: transaortic in 7 and transpulmonary in 1, and direct suture in 3 patients through aortotomy. Results Two patients with complex, associated lesions died in the postoperative period: one with interrupted aortic arch and patent ductus arteriosus died of brain and lung complications on the 23rd day postoperatively, the other with tetralogy of Fallot died of pulmonary hypertention crisis on the first day after the operation. All survivors had good clinical improvement at a mean interval of 43.2 months follow up. Conclusions Aortopulmonary septal defect is a very rare congenital heart anomaly and an incorrect diagnosis of patent ductus arteriosus is easily made in this disease. Early surgical treatment can achieve satisfactory result, whereas the mortality rate of this disease is closely related to its associated complex heart lesions and severe pulmonary hypertension.