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Published on: June 15, 2020
Cohort study of intervened functionally univentricular heart in England and Wales (2000-2018)
Elena Hadjicosta1, Rodney Franklin2, Anna Seale3
1Clinical Operational Research Unit, Department of Mathematics, University College London, London, UK.
Insights
Nearly 80% of children with functionally univentricular heart (FUH) conditions survived 10 years after initial palliation. This study details treatment pathways and survival for complex congenital heart disease (CHD).
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Research
- Long-term Outcomes in FUH
Background:
- Long-term outcome data for complex congenital heart disease (CHD) is limited.
- Functionally univentricular heart (FUH) conditions require complex, staged interventions.
- Understanding treatment pathways and survival is crucial for improving patient care.
Purpose of the Study:
- To describe treatment pathways for functionally univentricular heart (FUH) conditions.
- To determine survival rates for patients with FUH, excluding hypoplastic left heart syndrome.
- To provide long-term outcome data for complex CHD.
Main Methods:
- Retrospective cohort study using the National Congenital Heart Diseases Audit (2000-2018).
- Primary outcome: mortality, ascertained from the Office for National Statistics (2020).
- Analysis included 1557 patients with FUH, detailing diagnostic categories and surgical timing.
Main Results:
- 10-year survival rate after initial palliation was 77.2% (95% CI 75.0% to 79.2%).
- Higher mortality risks were associated with unbalanced atrioventricular septal defect (AVSD), atrial isomerism, low weight, critical illness, and comorbidities at initial palliation.
- Staged surgeries included initial palliation, cavopulmonary shunt, and Fontan procedure with median ages of 11.5 days, 9.2 months, and 56.2 months, respectively.
Conclusions:
- Despite complex and variable treatment pathways, approximately 8 out of 10 children with FUH survived to 10 years.
- Longer-term outcome analyses based on diagnosis can inform clinical practice and improve care for complex CHD.
- This study provides valuable insights into survival and treatment for FUH, aiding clinicians, patients, and parents.
Objective:
Given the paucity of long-term outcome data for complex congenital heart disease (CHD), we aimed to describe the treatment pathways and survival for patients who started interventions for functionally univentricular heart (FUH) conditions, excluding hypoplastic left heart syndrome.
Methods:
We performed a retrospective cohort study using all procedure records from the National Congenital Heart Diseases Audit for children born in 2000-2018. The primary outcome was mortality, ascertained from the Office for National Statistics in 2020.
Results:
Of 53 615 patients, 1557 had FUH: 55.9% were boys and 67.4% were of White ethnic groups. The largest diagnostic categories were tricuspid atresia (28.9%), double inlet left ventricle (21.0%) and unbalanced atrioventricular septal defect (AVSD) (15.2%). The ages at staged surgery were: initial palliation 11.5 (IQR 5.5-43.5) days, cavopulmonary shunt 9.2 (IQR 6.0-17.1) months and Fontan 56.2 (IQR 45.5-70.3) months. The median follow-up time was 10.8 (IQR 7.0-14.9) years and the 1, 5 and 10-year survival rates after initial palliation were 83.6% (95% CI 81.7% to 85.4%), 79.4% (95% CI 77.3% to 81.4%) and 77.2% (95% CI 75.0% to 79.2%), respectively. Higher hazards were present for unbalanced AVSD HR 2.75 (95% CI 1.82 to 4.17), atrial isomerism HR 1.75 (95% CI 1.14 to 2.70) and low weight HR 1.65 (95% CI 1.13 to 2.41), critical illness HR 2.30 (95% CI 1.67 to 3.18) or acquired comorbidities HR 2.71 (95% CI 1.82 to 4.04) at initial palliation.
Conclusion:
Although treatment pathways for FUH are complex and variable, nearly 8 out of 10 children survived to 10 years. Longer-term analyses of outcome based on diagnosis (rather than procedure) can inform parents, patients and clinicians, driving practice improvements for complex CHD.
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