Does Disease Severity Contribute to Disparity in Outcome Amongst Ethnic Groups in Patients with Hypoplastic Left Heart syndrome in New Zealand?

Abstract

Māori/Pacific Island patients with hypoplastic left heart syndrome (HLHS) have a later diagnosis, lower termination rates and higher mortality compared to other ethnic groups in New Zealand (NZ). Our aim was to determine if HLHS disease severity contributes to these outcome differences. Patients with HLHS diagnosed on a fetal or post-natal echocardiogram in NZ 2006 - 2015 were identified via a national database and divided into sub-groups (NZ European, Māori/Pacific Islander, and Asian). A fetal echocardiography risk score was calculated based on predictors of severity. Mitral stenosis/aortic atresia, hypoplastic aortic arch, intact/restrictive atrial septum, moderate-severe tricuspid regurgitation, and moderate-severe right ventricular dysfunction were each allocated one point. There were 125 patients with HLHS. Twenty-seven (22%) were excluded due to insufficient fetal data. All 27 were terminated or still-born. Results for included cases:Tabled 1NZ EuropeanMāori/PIAsianpTotal patients (98)60 (61%)29 (30%)9 (9%)Birth state0.002Termination of pregnancy32 (53%)3 (10%)5 (56%)Stillbirth2 (3%)2 (7%)2 (22%)Livebirth26 (43%)24 (83%)2 (22%)Risk score ≥219 (32%)20 (69%)1 (11%)0.02Survival at 1 year (% live birth)16 (62%)5 (21%)0 (0%)0.009Survival at 1 year if risk score ≥25 (26%)2 (10%)0 (0%)0.49 Open table in a new tab Disparity exists in health outcomes amongst ethnic groups in patients with HLHS in NZ. More severe disease in Māori/Pacific Island patients may in part account for differences in outcome.