Objectives:
The incidence of cardiovascular malformations in Down syndrome (DS) is well described however there remains little data on spectrum, management and outcome. We aimed to describe this in a defined population over 22 years.

Methods:
Data were obtained on all live-births and live-births with DS from 1985-2006 in the Northern region of the UK (population 3 million) with all congenital heart disease referred to a single centre.

Results:
In 1985-2006 there were 754,486 live-births. 821 were live-born with DS (1.08 per 1000 with no change in live birth prevalence over time). 343(42%) had a cardiovascular malformation (Figure 1). One patient had pulmonary atresia/intact ventricular septum and one had an unbalanced complete atrioventricular septal defect requiring completion of Fontan circulation. 21% had two or more abnormalities, the commonest additional being a persistent ductus arteriosus.

In 1985-1995, 101/163(62%) had surgery of which 30 died (30% operated on in this time), 14 within 30 days post-operatively. 17(5%) patients in the total group developed pulmonary hypertension. In 1996-2006, 129/180(72%) had surgery of which 7 died (5% operated on during this time), 2 within 30 days. There was 1 Fontan completion and 2 cardiac transplants.

1 year survival in DS with cardiac diagnosis improved from 78% in 1985-1995 to 91% in 1996-2006(Figure 2).

Conclusions:
Although the spectrum of cardiovascular malformations in DS has remained unchanged there has been a reduction in post-operative mortality. Other treatment modalities such as single ventricle palliation and transplantation are offered. Long term survival has improved over time.