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Clinical Consequences of Cardiomyopathy in Children With Biliary Atresia Requiring Liver Transplantation

Noelle M Gorgis1, Curtis Kennedy1, Fong Lam1

  • 1Section of Pediatric Critical Care, Baylor College of Medicine, Houston, TX.

Hepatology (Baltimore, Md.)
|August 5, 2018
PubMed

Insights

New echocardiographic criteria define cirrhotic cardiomyopathy (CCM) in children with biliary atresia (BA). This pediatric CCM definition identifies patients at high risk for serious adverse events and death during liver transplant (LT).

Area of Science:

  • Pediatric Cardiology
  • Hepatology
  • Transplant Medicine

Background:

  • Cirrhotic cardiomyopathy (CCM) is a known complication of end-stage liver disease but is poorly understood in pediatric populations.
  • Biliary atresia (BA) is a leading indication for pediatric liver transplantation (LT), often associated with significant morbidity.
  • Lack of objective criteria hinders the diagnosis and management of CCM in children with BA.

Purpose of the Study:

  • To establish objective two-dimensional echocardiographic (2DE) criteria for defining CCM in children with BA (BA-CCM).
  • To correlate the presence of BA-CCM with liver transplant outcomes, including serious adverse events and mortality.
  • To assess the predictive value of echocardiographic parameters for adverse outcomes compared to existing scores like PELD.

Main Methods:

  • Retrospective cohort analysis of pediatric patients with BA listed for LT.
  • Utilized receiver operating characteristic (ROC) curve analysis to determine optimal 2DE cut-off values for left ventricular (LV) geometrical parameters.
  • Proposed a working definition for BA-CCM based on LV mass index (LVMI) and relative wall thickness.

Main Results:

  • A significant proportion of children with BA (49%) met the proposed criteria for BA-CCM.
  • BA-CCM was associated with increased multiorgan dysfunction, need for mechanical/vasopressor support, and prolonged ICU/hospital stays.
  • BA-CCM was strongly linked to waitlist deaths, post-transplant mortality, and serious adverse events (P < 0.01).
  • LVMI demonstrated superior predictive ability for adverse outcomes compared to the PELD score.

Conclusions:

  • The proposed 2DE criteria effectively identify BA-CCM in children with BA awaiting LT.
  • BA-CCM is a significant predictor of morbidity and mortality in this vulnerable pediatric population.
  • 2DE screening for BA-CCM can inform clinical decision-making for prioritizing and managing pediatric LT candidates.

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