Morris C D, Menashe V D
Department of Medicine, Oregon Health Sciences University, Portland 97201.
JAMA. 1991 Dec 25;266(24):3447-52.
To determine long-term survival and the cause of death after repair of one of eight congenital heart defects in childhood.
Cohort study.
General community.
All Oregon residents with one of eight congenital heart defects, which was repaired surgically between 1958 and 1989 when the patient was aged 18 years or younger, including (1) tetralogy of Fallot; (2) isolated ventricular septal defect; (3) isolated atrial septal defect; (4) coarctation of the aorta; (5) aortic valvular stenosis; (6) pulmonary valvular stenosis; (7) transposition of the great arteries; and (8) patent ductus arteriosus. Follow-up of this cohort of 2701 individuals was obtained from 94%.
Mortality from cardiac and noncardiac causes.
Age at surgery and operative mortality have decreased significantly over the last 30 years. Late cardiac mortality at 25 years after surgery was 5% for tetralogy of Fallot and isolated ventricular septal defect, 10% for coarctation of the aorta, 17% for aortic stenosis, 5% for pulmonic stenosis, and less than 1% for patent ductus arteriosus; there were no late cardiac deaths after atrial septal defect repair. For transposition, late cardiac mortality was 15% at 15 years after the Mustard operation and was 2% at 10 years after the Senning operation.
Surgical repair of most congenital heart defects is associated with lingering cardiac mortality, particularly for aortic stenosis, coarctation, and transposition.
确定儿童期八种先天性心脏缺陷之一修复后的长期生存率及死亡原因。
队列研究。
普通社区。
所有俄勒冈州患有八种先天性心脏缺陷之一的居民，这些缺陷于1958年至1989年患者18岁及以下时接受手术修复，包括（1）法洛四联症；（2）孤立性室间隔缺损；（3）孤立性房间隔缺损；（4）主动脉缩窄；（5）主动脉瓣狭窄；（6）肺动脉瓣狭窄；（7）大动脉转位；（8）动脉导管未闭。对这2701名个体组成的队列进行随访，随访率为94%。
心脏和非心脏原因导致的死亡率。
在过去30年中，手术时的年龄和手术死亡率显著降低。法洛四联症和孤立性室间隔缺损术后25年的晚期心脏死亡率为5%，主动脉缩窄为10%，主动脉狭窄为17%，肺动脉狭窄为5%，动脉导管未闭小于1%；房间隔缺损修复术后无晚期心脏死亡病例。对于大动脉转位，Mustard手术后15年的晚期心脏死亡率为15%，Senning手术后10年为2%。
大多数先天性心脏缺陷的手术修复与持续的心脏死亡率相关，尤其是主动脉狭窄、主动脉缩窄和大动脉转位。
