Objective To describe the long-term outcomes, treatment pathways and risk factors for patients diagnosed with hypoplastic left heart syndrome (HLHS) in England and Wales.
Methods The UK’s national audit database captures every procedure undertaken for congenital heart disease and updated life status for resident patients in England and Wales. Patients with HLHS born between 2000 and 2015 were identified using codes from the International Paediatric and Congenital Cardiac Code.
Results There were 976 patients with HLHS. Of these, 9.6% had a prepathway intervention, 89.5% underwent a traditional pathway of staged palliation and 6.4% of infants underwent a hybrid pathway. Patients undergoing prepathway procedures or the hybrid pathway were more complex, exhibiting higher rates of prematurity and acquired comorbidity. Prepathway intervention was associated with the highest in-hospital mortality (34.0%).
44.6% of patients had an off-pathway procedure after their primary procedure, most frequently stenting or dilation of residual or recoarctation and most commonly occurring between stage 1 and stage 2.
The survival rate at 1 year and 5 years was 60.7% (95% CI 57.5 to 63.7) and 56.3% (95% CI 53.0 to 59.5), respectively. Patients with an antenatal diagnosis (multivariable HR (MHR) 1.63 (95% CI 1.12 to 2.38)), low weight (<2.5 kg) (MHR 1.49 (95% CI 1.05 to 2.11)) or the presence of an acquired comorbidity (MHR 2.04 (95% CI 1.30 to 3.19)) were less likely to survive.
Conclusion Treatment pathways among patients with HLHS are complex and variable. It is essential that the long-term outcomes of conditions like HLHS that require serial interventions are studied to provide a fuller picture and to inform quality assurance and improvement.
- quality and outcomes of care
- Fontan physiology
- complex congenital heart disease
- congenital heart disease surgery
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Contributors KLB conceived the study. KLB, CP and MU designed and led the study. LR carried out the data analysis and wrote the first draft of the paper. RCF, IDS, MM, VT and CB provided clinical input and advice. All authors read and commented on the manuscript.
Funding This project was funded by the Great Ormond Street Children’s Charity (v1248). MU was in part supported by the National Institute for Health Research (NIHR) Collaboration for Leadership in Applied Health Research and Care North Thames at Bart’s Health NHS Trust. KLB, IDS, MM and VT were supported by the NIHR Research Biomedical Research Center at Great Ormond Street Hospital for Children NHS Foundation Trust and University College London.
Competing interests KLB and RCF sit on the steering committee of NCHDA.
Ethics approval The study was approved by the NCHDA Research Committee and the National Health Service (NHS) Healthcare Quality Improvement Partnership (study number 14CONG03).
Provenance and peer review Not commissioned; externally peer reviewed.