Best Predictor for Mortality from Biliary Atresia Liver Transplantation Candidates –Cardiomyopathy?

Briefly noted: A recent study (NM Gorgis et al. Hepatology 2019; 69: 1206-18, editorial 940-2 by Elizabeth Rand) indicates that cirrhotic cardiomyopathy (CCM) is very important factor for survival for biliary atresia (BA) patients requiring liver transplantation.

CCM was defined based on two-dimensional echocardiographic criteria: LV mass index ≥95 g/meter-squared or relative wall LV thickness of LV ≥0.42.

Key points:

  • Overall, 11 of 69 patients died, 4 while awaiting liver transplantation and 7 following transplantation.
  • 34 of 69 BA patients in this cohort had BA-CCM
  • All 11 who died had BA-CCM compared with no deaths in the 35 patients without CCM.

My take: Severe BA-CCM needs to be examined further; if severe, it may merit changing allocation policy.

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Joshua Tree National Park

Cirrhosis and Cardiac Function

Briefly noted: M Izzy, J Oh, KD Watt. Hepatology 2018; 68: 2008-2015.  This concise review discusses the outcome of cirrhotic cardiomyopathy after liver transplantation.

Key point: “Although it is often believed that cirrhotic cardiomyopathy resolves post-LT, the data, albeit limited, do not support this postulation…diastolic function may not improve post-transplant and may actually worsen. Improvement in systolic function was suggested by only two of six studies.”

Related blog post: Cholecardia

This figure from Hepatology November cover depicts a cirrhotic liver restricting the heart filling during diastole. (From Wiley Online Library -free access)

The heart connection

Cardiac changes with biliary atresia (BA) are surprisingly common– Gastoenterology 2011; 141: 1264-72.  In this study, 48 patients with BA, listed for liver transplantation, underwent echocardiography between 2004-2010.  The median age was 8 months.  Significant increases in left ventricle wall thickness (23% increase) and mass (51% increase) were noted; in addition, functional changes were noted as there was an increase in LV shortening fraction (8% increase).  Features of ‘cirrhotic cardiomyopathy’ were evident in 72% of infants (29/40).  The authors conclude that these heart changes likely contribute to prolongation of posttransplant hospitalization.

Additional References:

  • -J Am Coll Cardiol 2010; 56: 539-49
  • -J Hepatol 2010; 53: 179-90.