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

Background: 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 Normalised Ratio (MELD-XI) score, in adults with CHD. Methods: In this retrospective study of CHD patients with comprehensive metabolic panels (2003-2019), mild/moderate and severe hepatorenal dysfunction was defined as MELD-XI 11-15 and > 15, respectively. Results: Of 4977 patients, 1376 (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, CI 1.21-1.33; P < 0.001) after adjustment for age, sex, and congenital heart lesion. In 3864 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, CI 1.15-1.36, per unit increase in MELD-XI; P = 0.004), independently from the baseline MELD-XI score. In the subset of 1856 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, CI 0.90-0.98, per unit decrease in MELD-XI; P = 0.008), independently from the baseline MELD-XI score. Conclusions: Hepatorenal dysfunction was common in adults with CHD. Both baseline MELD-XI score and temporal changes in MELD-XI were associated with clinical outcomes, and therefore could be used to monitor therapeutic response to interventions and for deterioration in clinical status.