Quantifying additional procedures in functionally single ventricle disease: a national cohort study

Qi Huang*, Deborah Ridout, Victor Tsang, Nigel Drury, Timothy J Jones, Hannah Bellsham-Revell, Elena Hadjicosta, Anna Seale, Chetan Mehta, Christina Pagel, Sonya Crowe, Ferran Espuny-Pujol, Rodney C G Franklin, Kate L Brown

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

Background
Given their importance as a metric for health care evaluation, this study’s aim was to evaluate the rates for surgical and catheter reinterventions amongst children with functionally single ventricle (f-SV) congenital heart disease (CHD) undergoing staged palliation.

Methods
We undertook a retrospective cohort study of children born with f-SV CHD between 2000-2018 in England and Wales, using the national registry, with survival ascertained in 2020. Competing risk analysis was used to describe the incidence of additional procedures that occurred first, over follow-up, accounting for competing events of death or transplant.

Results
Of 56,039 patients who received an intervention for CHD, 3,307 (5.9%) had f-SV. The largest diagnostic subcategories were hypoplastic left heart syndrome 1276 (38.8%), tricuspid atresia 440 (13.4%), and double inlet left ventricle 322 (9.8%). Over a median follow-up of 5.4 (IQR: 0.8-10.8) years, 921 (27.9%) patients had at least one additional off-pathway surgery and 1,293 (39.1%) had at least one additional catheter interventions. The cumulative incidence of additional surgery at 6-months after stage one was 17.6% (95% CI: 16.2%-19.0%), 2-years after stage two was 8.3% (7.2%-9.5%) and 5-years after stage three was 8.4% (7.0%-9.9%). The cumulative incidence of additional catheter at 6-months after stage one was 18.0% (16.6%-19.4%), 2-years after stage two was 14.7% (13.3%-16.2%) and 5-years after stage three was and 23.7% (21.5%-26.0%).

Conclusions
It is important to quantify additional procedures for children with f-SV disease, to inform parents and health professionals, potentially facilitating the development of interventions that aim to reduce these important adverse outcomes.
Original languageEnglish
Journal Annals of Thoracic Surgery Short Reports
Early online date20 Dec 2023
DOIs
Publication statusE-pub ahead of print - 20 Dec 2023

Bibliographical note

Funding Sources
This study was funded by the British Heart Foundation (Project Grant No. PG/17/88/33401).

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