048 Aortic Dilation in Patients With Turner Syndrome

Abstract

inals were observed to have a predisposition to Total Anomalous Pulmonary Venous Return (TAPVR). We sought to determine if a unique distribution of congenital heart disease exist in Manitoba. METHODS: Retrospective review of all pediatric patients diagnosed with any form of D-TGA and TAPVR from 1991-2010 in Manitoba was undertaken. Epidemiological parameters were collected. Ethnicity was collected on a self-identification basis: aboriginal (First Nation/Metis/Inuit) versus non-aboriginal. Birth rates were collected from statistics Canada. Odds ratio calculations using 5-year moving rates were used to compare groups. RESULTS: Ninety-three percent (93/100) of D-TGA patients were non-aboriginal with a incidence of 1 in 2866 live births compared to 1 in 7620 for Aboriginals (Odds Ratio 2.3, 95% CI 1.12-4.74, p 0.024). In TAPVR 59 % (29/57) of patients were aboriginal with an incidence of 1 in 1838 live births, compared to 1 in 10253 in non-aboriginals (Odds ratio 5.58, 95% CI 3.28-9.47), p 0.0001). In isolated TAPVR, without any complex concomitant abnormalities or heterotaxia syndromes, the aboriginal odds ratio was 8.57 (95% CI 4.4316.57, p 0.0001). CONCLUSION: Clinically and statistically, TAPVR was significantly more common in Manitoba aboriginals, compared to non-aboriginals. The occurrence of D-TGA was observed to be almost 2 times less likely in the Manitoban Aboriginal populace studied. This distinct pattern of congenital heart disease warrants future research to investigate the epidemiological factors and/or genetic etiology causing these observed ethnical differences.