Sheehan A, Ward O C, Duff D F, Denham B, Neligan M, Wood A
Department of Cardiology, Our Lady's Hospital for Sick Children, Dublin.
Ir Med J. 1990 Jun;83(2):67-9.
Between January 1976 and December 1987 42 children with Down syndrome and congenital heart disease underwent cardiac surgery. Four children had two operations. Age at the time of surgery ranged from 11 days to 14 years. The commonest operative procedure was repair of a patent ductus arteriosus. Four patients died post-operatively, two following repair of a complete atrio-ventricular canal defect (CAVD), one following correction of tetralogy of Fallot in association with a CAVD, and a fourth following closure of ventricular septal defect and atrial septal defect. The mortality for those who had open heart surgery was 13.3% and for the series as a whole the mortality was 6.6% over a period of follow-up ranging from two months to four years. A relatively conservative approach has been adopted with regard to surgery, based on the shorter natural expectation of life in Down syndrome, the complexity of many of the cardiac lesions involved and the recognition of the frequency of early intellectual deterioration in Down patients.
1976年1月至1987年12月期间，42名患有唐氏综合征和先天性心脏病的儿童接受了心脏手术。4名儿童接受了两次手术。手术时的年龄从11天到14岁不等。最常见的手术操作是动脉导管未闭修复术。4例患者术后死亡，2例死于完全性房室通道缺损（CAVD）修复术后，1例死于法洛四联症合并CAVD矫正术后，第4例死于室间隔缺损和房间隔缺损闭合术后。接受心脏直视手术的患者死亡率为13.3%，在为期两个月至四年的随访期内，整个系列的死亡率为6.6%。鉴于唐氏综合征患者预期寿命较短、所涉及的许多心脏病变复杂以及认识到唐氏患者早期智力衰退的频率，在手术方面采取了相对保守的方法。
