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Cardiac-associated biliary atresia (CABA): a prognostic subgroup
Bashar Aldeiri1, Vangelis Giamouris2, Kuberan Pushparajah3,4
1Department of Paediatric Surgery, King's College Hospital NHS Foundation Trust, London, UK.
Archives of Disease in Childhood
|July 22, 2020
Summary
Infants with biliary atresia (BA) and heart defects face higher mortality. Cardiac surgery before Kasai portoenterostomy (KPE) may improve outcomes for this high-risk group, termed cardiac-associated biliary atresia (CABA).
Area of Science:
- Pediatric Surgery
- Cardiology
- Hepatology
Background:
- Biliary atresia (BA) is a serious condition affecting infants.
- Concurrent cardiac malformations can complicate BA treatment.
- A functional framework is needed to assess cardiac risk in BA.
Purpose of the Study:
- To detail the spectrum of cardiac anomalies in BA patients.
- To establish a risk stratification framework for cardiac involvement in BA.
- To evaluate the impact of cardiac malformations on BA treatment outcomes.
Main Methods:
- A single-center database prospectively collected data on 524 infants with BA.
- Infants were classified using a cardiac functional framework (A, B, C).
- Outcomes included jaundice clearance post-Kasai portoenterostomy (KPE) and survival rates.
Main Results:
- Seven percent (37/524) of BA infants had cardiac anomalies, with 57% linked to BA splenic malformation or cat-eye syndrome.
- Jaundice clearance was lower in infants with cardiac anomalies (54%) compared to controls (65%).
- Mortality was significantly higher in BA infants with cardiac anomalies (40%) versus controls (4%).
Conclusions:
- Cardiac-associated biliary atresia (CABA) represents a high-risk subgroup.
- Restorative cardiac surgery should ideally precede KPE to improve outcomes.
- This highlights the importance of integrated cardiac and liver management in BA.
