儿童左冠状动脉异常起源于肺动脉的手术治疗效果及随访

目的：总结左冠状动脉异常起源于肺动脉（ ALCAPA）的手术效果和随访结果。方法回顾性分析20例ALCAPA患儿的临床资料。术前左室射血分数（ EF）8％～65％，其中16例EF＜40％。20例患儿均在中低温体外循环下，移植左冠状动脉至升主动脉，其中8例行急诊手术，其余12例为亚急诊手术。6例同期行二尖瓣成形术。结果住院死亡3例（15％），1例为术后低心排血量综合征，2例死于术后顽固性室颤。失访1例，另16例存活患儿无院外死亡，随访3～67个月。术前EF＜40％的患儿中有61．5％（8／13）在术后3～6个月期间EF升至50％以上；术前EF＜40％、随访1年以上的患儿中有90．9％（10／11） EF升至50％以上。结论外科手术移植左冠状动脉至升主动脉是治疗ALCAPA的有效方法。术后低心排血量综合征和恶性心律失常是其主要死亡原因。患儿如能安全度过围术期，心功能有望恢复正常，远期预后良好。

Surgical treatment and follow-ups of anomalous origin of the left coronary artery from the pulmonary artery in children

Objective To analyze the surgical treatment and follow-up outcomes of anomalous origin of the left coronary artery from the pulmonary artery ( ALCAPA) in children.Methods 20 cases of ALCAPA were retrospectively analyzed, including 8 boys and 12 girls, aged from 2 months to 13 years.The preoperative ejection fraction ( EF) of left ventricular ranged from 8%to 65%, and 16 children presented with EF<40%.All 20 patients were operated by direct reimplantation of the left coronary artery into the ascending aorta using moderate or deep hypothermia cardiopulmonary by-pass.Surgery was undertaken on an urgent basis in 8 patients and on a semi-elective basis in the remaining 12 children. Concurrent mitral valvoplasty was performed in 6 children.Results The hospital mortality was 15%( 3/20 ) .One case was due to postoperative low cardiac output, and the other two patients died from refractory ventricular fibrillation.One pa-tient was lost to follow-up.The remaining 16 patients were asymptomatic during follow-ups of 3 to 67 months.EF was elevated from preoperative <40% to over 50% in 61.5% ( 8/13 ) patients within 3 to 6 months after surgery and in 90.9%(10/11) patients 1 year after surgery.Conclusions Implantation of the left coronary artery from the pulmonary artery into aorta is an effective surgical treatment for ALCAPA.Low cardiac output and malignant ventricular arrhythmia are two main causes of death after ALCAPA surgery.The heart function is expected to recover if the patients can carry though the perioperative period, and good outcomes can be achieved after the surgery of ALCAPA.