Canadian Journal of Cardiology

Determinants and Prognostic Implications of Hepatorenal Dysfunction in Adults with Congenital Heart Disease

Published:August 04, 2022DOI:



      There are limited data on the prognostic role of hepatorenal function indices in ambulatory patients with congenital heart disease (CHD). The purpose of this study was to determine the prevalence, risk factors, and prognostic implications of hepatorenal dysfunction, as measured by model for end-stage liver disease excluding international normalized ratio (MELD-XI) score, in adults with CHD.


      Retrospective study of CHD patients with comprehensive metabolic panel (2003-2019). Mild/moderate and severe hepatorenal dysfunction were defined as MELD-XI 11-15 and >15 respectively.


      Of 4,977 patients, 1,376 (28%) had hepatorenal dysfunction (mild/moderate n=935[19%]; severe n=441[9%]). Hepatorenal dysfunction was most common in Fontan/unrepaired single ventricle (46%) and right heart disease (31%). Baseline MELD-XI was associated with all-cause mortality (HR 1.27, 1.21-1.33, p<0.001) after adjustment for age, sex, and congenital heart lesion.
      In 3,864 patients with serial MELD-XI data, there was a temporal increase in MELD-XI, and this was associated with an increased risk of mortality (HR 1.24, 1.15-1.36 per unit increase in MELD-XI, p=0.004), independent of baseline MELD-XI score. In the subset of 1,856 patients that underwent surgical/transcatheter interventions, there was a postoperative reduction in MELD-XI, and this was associated with a lower risk of mortality (HR 0.94, 0.90-0.98 per unit decrease in MELD-XI, p=0.008), independent of the baseline MELD-XI score.


      Hepatorenal dysfunction was common in adults with CHD. Both baseline MELD-XI score and temporal changes in MELD-XI score were associated with clinical outcomes, and hence could be used to monitor for deterioration in clinical status, and therapeutic response to interventions.

      Key Words


      CHD (congenital heart disease), RA (right atrium), RV (right ventricle), MELD-XI (model for end-stage liver disease excluding international normalized ratio), AUC (area under the curve), HR (hazard ratio), CI (confidence interval)
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