Fetal single ventricle journey to first postnatal procedure: A multicentre UK cohort study.

Lillitos, P.J.; Nolan O.; Cave D.G.W.; Lomax C.; Barwick S.; Bentham J.R.; Seale A.N.

Fetal single ventricle journey to first postnatal procedure: A multicentre UK cohort study.

All Authors

Lillitos, P.J.

Nolan O.

Cave D.G.W.

Lomax C.

Barwick S.

Bentham J.R.

Seale A.N.

LTHT Author

Lillitos, Peter
Cave, Daniel
Barwick, Shuba
Bentham, James

LTHT Department

Congenital Cardiac Services
Cardiology
Cardio-Respiratory

Publication Date

2024

Item Type

Article

Abstract

Objectives UK single ventricle (SV) palliation outcomes after first postnatal procedure (FPP) are well documented. However, survival determinants from fetal diagnosis to FPP are lacking. To better inform parental-fetal counselling, we examined factors favouring survival at two large UK centres. Design Retrospective multicentre cohort study. Setting Two UK congenital cardiac centres: Leeds and Birmingham. Patients SV fetal diagnoses from 2015 to 2021. Main outcome measures Survival from fetal diagnosis with intention to treat (ITT) to birth and then FPP. Maternal, fetal and neonatal risk factors were assessed. Results There were 666 fetal SV diagnoses with 414 (62%) ITT. Of ITT, 381 (92%) were live births and 337 (81%) underwent FPP. Survival (ITT) to FPP was notably reduced for severe Ebstein's 14/22 (63.6%), unbalanced atrioventricular septal defect 32/45 (71%), indeterminate SV 3/4 (75%), mitral atresia 8/10 (80%) and hypoplastic left heart syndrome 127/156 (81.4%). Biventricular pathway was undertaken in five (1%). After multivariable adjustment, prenatal risk factors for mortality were increasing maternal age (OR 1.05, 95% CI 1.0 to 1.1), non-white ethnicity (OR 2.6, 95% CI 1.4 to 4.8), extracardiac anomaly (OR 6.34, 95% CI 1.8 to 22.7) and hydrops (OR 7.39, 95% CI 1.2 to 45.1). Postnatally, prematurity was significantly associated with mortality (OR 6.3, 95% CI 2.3 to 16.8). Conclusions Around 20% of ITT fetuses diagnosed with SV will not reach FPP. Risk varies according to the cardiac lesion and is significantly influenced by the presence of an extracardiac anomaly, fetal hydrops, ethnicity, increasing maternal age and gestation at birth. These data highlight the need for fetal preprocedure data to be used in conjunction with procedural outcomes for fetal counselling.