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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.
Insights
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.
Objectives:
To describe the range of concurrent cardiac malformations in biliary atresia (BA) while providing a functional framework of risk.
Methods:
Demographic and variables were collected from a prospectively maintained single-centre database. Infants were grouped according to a cardiac functional framework (A=acyanotic, B=cyanotic and C=insignificant shunt). Primary outcome was set as clearance of jaundice (bilirubin ≤20 μmol/L) following Kasai portoenterostomy (KPE). Native liver survival and overall actuarial survival were compared with a date-matched control infant with BA (n=77). P value <0.05 was regarded as significant.
Results:
524 infants with histologically confirmed BA were treated between January 1999 and December 2018, 37 (7%) had a concurrent cardiac anomaly (A: n=23 (62%), B: n=10 (27%), C: n=4 (11%)). Infants with biliary atresia splenic malformation (BASM) or cat-eye syndrome (CES) contributed over half of the cases (21/37; 57%).Overall, 20 (54%) infants cleared jaundice (vs 50/77 (65%) controls; p=0.2), but with higher mortality compared with the non-cardiac controls (15/37 (40%) vs 3/77 (4%); HR 15.5 (95% CI 5.5 to 43.4); p<0.00001). Infants requiring cardiac intervention in the first year of life (n=15) were more likely to clear jaundice (6/7 vs 2/8; p=0.04) and had a trend towards higher survival (6/7 vs 3/8; p=0.1) when KPE followed cardiac surgery. Yet, the type of cardiac pathology did not impact clearance of jaundice or mortality.
Conclusion:
We propose the term cardiac-associated biliary atresia (CABA) as a high-risk group. We believe that restorative cardiac surgery should precede KPE wherever possible to improve outcome.
