Cardiovascular abnormalities in Down's syndrome: spectrum, management and survival over 22 years

Background The prevalence of cardiovascular anomalies in Down's syndrome is well described, but there are few data on spectrum, management and outcome. The authors aimed to provide this information for infants with Down's syndrome in a defined population over a 22-year period. Methods The regional paediatric cardiology database in Newcastle upon Tyne provided information on all cardiovascular anomalies, surgical treatment and outcome. Data was subdivided into two eras, 1985-1995 and 1996-2006, and surgical results and outcomes compared. Data on live births with Down's syndrome were obtained from the Northern Congenital Abnormality Survey (NorCAS). Denominator data on all live births in the region were obtained from UK Statistics. Results In 1985-2006 there were 754,486 live births in the population. 821 infants were live-born with Down's syndrome (1.09 per 1000 live births). 342 (42%) infants with Down's syndrome had a cardiovascular anomaly. The commonest anomaly was complete atrioventricular septal defect in 125 (37%) infants. Three patients had univentricular physiology. In 1985-1995, 101/163 (62%) infants had surgery with 30% mortality; in 1996-2006, 129/180 (72%) had surgery with 5% mortality. One patient underwent Fontan completion. There were two cardiac transplants for cardiomyopathy. One-year survival in Down's syndrome with a cardiovascular anomaly improved from 82% in 1985-1995 to 94% in 1996-2006. Conclusions The incidence of cardiovascular anomalies in Down's syndrome was 42%. There has been a significant reduction in postoperative mortality and improvement in 1-year survival. Treatment modalities such as single ventricle palliation and cardiac transplantation are now considered in these patients.