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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.
- Cardiology
- Mortality
- Neonatology
- Paediatrics
Data availability statement
Data are available upon reasonable request. All data relevant to the study are included in the article or uploaded as supplementary information.
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Data availability statement
Data are available upon reasonable request. All data relevant to the study are included in the article or uploaded as supplementary information.
Footnotes
Correction notice This paper has been corrected since it was first published. The percentages in table 3, row 'Restrictive atrial septum in HLHS' have been corrected.
Contributors PJL: chief investigator and project lead; involved in project conception, method design, data collection and data analysis; main author of the manuscript; guarantor. ON: principal investigator at Birmingham; involved in method design, data collection and manuscript review. DGWC: method design, statistical analysis and manuscript review. CL: method design, data collection and manuscript review. SB: project conception, method design, data interpretation and manuscript review. JRB: method design, data interpretation and manuscript review. ANS: project supervisor; involved in project conception, method design, data interpretation and manuscript review.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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