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

Abstract

ObjectiveTo investigate the relationship between ethnicity and health outcomes among fetuses and infants with congenital left heart obstruction (LHO).DesignA 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).ResultsCritical 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.ConclusionHLHS 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.

Factors influencing the choice-of-care pathway and survival in the fetus with hypoplastic left heart syndrome in New Zealand: a population-based cohort study

“…[2][3][4] In New Zealand, the incidence of HLHS is 0.22/1000 births-similar to that in northern Europe when maternal ethnicity is European, and lower when maternal ethnicity is Māori or Pacific. 5 Mortality from the first stage procedure to the second stage procedure was 17% 2 between 1992 and 2014, similar to that reported elsewhere. 4 6 7 Antenatal detection of HLHS provides an opportunity for prenatal counselling.…”

“…There may also be differences in parental values and religious beliefs across ethnic groups. 5 23 In one study maternal education explained a large percentage of the difference in outcome seen between ethnic groups in infants with HLHS. 23 The NZ deprivation index combines a number of social and economic variables that are likely to impact access to healthcare and other resources.…”

“… 5 The cause of this inequity is unclear but could relate to access to healthcare, parental and family values, implicit bias or, given the heterogeneity of HLHS, differences in disease severity. 5 Several recent reports have highlighted the impact of access on decision-making in this condition. 8 9 A better understanding of this process has the potential to impact the future design of maternal, fetal and paediatric cardiac programmes in areas where there is inequity in healthcare delivery.…”

Transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia: A Swedish national cohort study

“…11 In a recent national population-based study from New Zealand, Cloete et al reported 56% survival to 1 year of age in hypoplastic left heart syndrome with intention to treat. 12 The improved survival between the early and late birth period was mainly explained by improved survival between stages I and II. Improved survival over time was frequently reported in the early era of Norwood surgery.…”

Social Determinants of Disparities in Mortality Outcomes in Congenital Heart Disease: A Systematic Review and Meta-Analysis

“…A large SR-MA by Paradies et al has suggested that racial discrimination is a significant determinant of physical and mental health outcomes in the United States ( 80 ). While most of the studies in this review are from the United States, this finding is likely to be true in other societies, as is suggested from studies included in our review from countries such as Panama ( 24 ) and New Zealand ( 27 ).…”