主-肺动脉间隔缺损手术21例临床分析

Surgical treatment of aortopulmonary septal defect in 21 cases

目的 总结 2 1例先天性主 -肺动脉间隔缺损 (APSD)术前诊断和外科治疗的经验、教训及疗效。方法 1979年 11月至 2 0 0 0年 2月收治APSD病儿 2 1例中男 12例 ,女 9例 ;平均 (6 5± 3.8)岁。按Richardson分型 ,I型 10例、II型 10例、III型 1例。 12例合并其他心脏畸形 ,4例曾诊断为动脉导管未闭 ,有左后外侧开胸史 ,其中 3例为误诊。 14例采用补片修补缺损 ,4例直接缝合 ,2例心外结扎 ,1例行人工血管右肺动脉 -主肺动脉连接术。结果 术前确诊 11例 ,误诊、漏诊 10例。术后早期死亡 3例 ,晚期死亡 1例。 15例随访 1个月～ 15年 ,疗效满意。结论 APSD是一种易被误诊和漏诊的少见先天性心脏病。提高对本病的认识、仔细的超声检查、结合升主动脉造影和超高速CT检查有助于确诊。及早手术 。

Objective: To review the experience of diagnosis and surgical treatment of aortopulmonary septal defect (APSD). Methods: Twenty one patients with APSD, aged(6 5±3 8) years, underwent operative repair from November 1979 to February 2000. According to Richardson classification system, type I APSD was present on 10 patients, whereas type II and III were found in 10 and 1, respectively. Twelve patients were associated with other cardiac defects. Four patients had a history of left posterolateral thoracotomy, in 3 of whom an incorrect diagnosis of patent ductus arteriosus (PDA) was made. Results: Correct diagnosis of APSD was made in 10 cases before operation and 11 were misdiagnosed. Operative technique included patch of the defect in 14 patients, direct suture in 4 and ligation in 2. In one patient with type III APSD right pulmonary artery was harvested from the ascending aorta and anastomosed to the main pulmonary artery using an artificial conduct. There were three operative deaths with a mortality rate of 14 3% . The caused of death was postoperative pulmonary hypertension. The late death occurred in one patient. All survivors had clinical improvement at 1 month to 15 years follow up. Conclusions: APSD is a rare congenital heart disease and often misdiagnosed as PDA. Preoperative correct diagnosis can be made by careful examination of echocardiography, ascending aortic angiography and spiral CT. Early surgical treatment and intensive care of pulmonary hypertension postoperatively may improve the result of operation.