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目的 总结成人复杂型主动脉缩窄合并其他心内畸形的临床特点以及外科治疗方式.方法 成人复杂型主动脉缩窄合并心内畸形患者6例,年龄(43.8±10.6)岁.合并的心内畸形包括升主动脉瘤3例、主动脉瓣关闭不全4例、主动脉瓣狭窄1例、二尖瓣关闭不全2例、二尖瓣狭窄1例、中-重度三尖瓣关闭不全1例、冠心病1例、动脉导管未闭2例和室间隔缺损1例.解剖外旁路转流术包括升主动脉-胸降主动脉旁路人造血管术4例和升主动脉-腹主动脉旁路人造血管术2例.同期手术包括主动脉瓣置换术3例、主动脉根部置换术2例、二尖瓣置换术1例、二尖瓣成形2例、冠状动脉旁路移植术1例、动脉导管未闭缝合术1例和室间隔缺损修补术1例.结果 6例患者均无手术死亡,术后无脑部和脊髓等神经系统并发症.其中1例接受二次开胸止血,该例患者同时出现术后急性肾功能不全,经透析后肾功能恢复正常;其余患者术后恢复满意.患者均获随访,最长随访时间6年,患者均存活,血压均恢复正常,上下肢血压差小于20 mmHg,人造血管保持血流通畅,均无明显不适症状.结论 对成人复杂型主动脉缩窄合并其他心内畸形患者,一期同时治疗心内畸形联合解剖外旁路主动脉转流术是安全、可行的.“,”Objective To summarize the clinical characteristic and surgical management of complex coarctation of aorta associated with intracardiac abnormalities.Methods Data of six adult cases aged (43.8 ± 10.6) years old with complex coarctation of aorta associated with intracardiac abnormalities were retrospectively reviewed.The intracardiac abnormalities included ascending aortic aneurysm in 3 cases,aortic valve insufficiency in 4 cases,aortic valve stenosis in 1 case,mitral valve insufficiency in 2 cases,mitral valve stenosis in 1 case,moderate-severe tricuspid valve insufficiency in 1 case,coronary artery stenosis in 1 case,patent ductus arteriosurn(PDA) in 2 cases,and ventricular septal defect (VSD)in 1 case.Extraanatomic aortic bypass included ascending-descending aortic graft bypass in 4 cases and ascending-abdominal aortic graft bypass in 2 cases.At the same time,simultaneous procedures were performed,which included aortic valve replacement in 3 cases,aortic root replacement in 2 cases,mitral valve replacement in 1 case,mitral valve repair in 2 cases,coronary artery bypass grafting in 1 case,PDA repair in 1 case,VSD repair in 1 case.Results The surgeries were performed without motality and central nervous system complication.One patient received reexploration for hemostasis because of anastomosis bleeding,who suffered from acute renal failure and completely recovered from dialysis.Blood pressures of all cases returned to normal after operation with blood pressure gradient between the upper and lower limbs less than 20 mmHg and patent flow in aortic grafts.Conclusion In the patients with complex coarctation of aorta associated with intracardiac abnormalities,simultaneous management of intracardiac abnormalities combined with extraanatomic aortic bypass is safe and feasible.