Congenital left heart obstruction: ethnic variation in incidence and infant survival

OBJECTIVE: To investigate the relationship between ethnicity and health outcomes among fetuses and infants with congenital left heart obstruction (LHO). DESIGN: A retrospective population-based review was conducted of fetuses and infants with LHO including all terminations, stillbirths and live births from 20 weeks' gestation in New Zealand over a 9-year period. Disease incidence and mortality were analysed by ethnicity and by disease type: hypoplastic left heart syndrome (HLHS), aortic arch obstruction (AAO), and aortic valve and supravalvular anomalies (AVSA). RESULTS: Critical LHO was diagnosed in 243 fetuses and newborns. There were 125 with HLHS, 112 with AAO and 6 with isolated AVSA. The incidence of LHO was significantly higher among Europeans (0.59 per 1000) compared with Māori (0.31 per 1000; p<0.001) and Pacific peoples (0.27 per 1000; p=0.002). Terminations were uncommon among Māori and Pacific peoples. Total case fatality was, however, lower in Europeans compared with other ethnicities (42% vs 63%; p=0.002) due to a higher surgical intervention rate and better infant survival. The perinatal and infant mortality rate was 82% for HLHS, 15% for AAO and 2% for AVSA. CONCLUSION: HLHS carries a high perinatal and infant mortality risk. There are ethnic differences in the incidence of and mortality from congenital LHO with differences in mortality rate suggesting inequities may exist in the perinatal management pathway.