Advertisement
Canadian Journal of Cardiology

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

Published:August 04, 2022DOI:https://doi.org/10.1016/j.cjca.2022.07.018

      Abstract

      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.

      Résumé

      Contexte

      Il existe peu de données sur le rôle des indices du fonctionnement hépatorénal dans l’établissement du pronostic des patients ambulatoires atteints d’une cardiopathie congénitale. L’objectif de cette étude était de déterminer la prévalence de l’insuffisance hépatorénale, les facteurs de risque de la maladie et les facteurs pronostiques à considérer, mesurés à l’aide du modèle d'hépatopathie en phase terminale excluant le rapport international normalisé (MELD-XI; de l'anglais : Model for End-Stage Liver Disease Excluding International Normalised Ratio), chez des adultes atteints d’une cardiopathie congénitale.

      Méthodologie

      Dans cette étude rétrospective menée auprès de patients atteints d’une cardiopathie congénitale ayant subi un bilan métabolique complet (2003-2019), la dysfonction hépatorénale correspondait à un score MELD-XI de 11 à 15 pour la forme légère ou modérée et de > 15 pour la forme grave.

      Résultats

      Parmi les 4 977 patients, 1 376 (28 %) présentaient une dysfonction hépatorénale (légère ou modérée : n = 935 [19 %]; grave : n = 441 [9 %]). La dysfonction hépatorénale était plus courante chez les personnes ayant subi une intervention de Fontan ou ayant un ventricule unique non opéré (46 %) et chez celles présentant une insuffisance cardiaque droite (31 %). Le score MELD-XI au départ était associé à la mortalité toutes causes confondues (rapport de risques instantanés [RRI] : 1,27; intervalle de confiance [IC] de 1,21 à 1,33; p < 0,001) après ajustement pour l’âge, le sexe et les lésions cardiaques congénitales. Chez 3 864 patients pour lesquels les scores MELD-XI ont été recueillis en série, on a observé une augmentation temporelle de ce score, ce qui était associé à un risque accru de décès (RRI : 1,24; IC de 1,15 à 1,36, augmentation par unité du score MELD-XI; p = 0,004), quel que soit le score au départ. Dans le sous-groupe de 1 856 patients qui ont subi une intervention chirurgicale ou par cathéter, il y avait une réduction postopératoire du score MELD-XI, qui était associée à un plus faible risque de décès (RRI : 0,94; IC de 0,90 à 0,98, diminution par unité de MELD-XI; p = 0,008), quel que soit le score MELD-XI au départ.

      Conclusions

      La dysfonction hépatorénale était courante chez les adultes atteints d’une cardiopathie congénitale. Tant le score MELD-XI au départ que son évolution temporelle étaient associés à des issues cliniques et pourraient donc être utilisés pour surveiller la réponse thérapeutique aux interventions et la détérioration de l’état clinique.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Canadian Journal of Cardiology
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Samsky M.D.
        • Patel C.B.
        • DeWald T.A.
        • et al.
        Cardiohepatic interactions in heart failure: an overview and clinical implications.
        J Am Coll Cardiol. 2013; 61: 2397-2405
        • Rangaswami J.
        • Bhalla V.
        • Blair J.E.A.
        • et al.
        American Heart Association Council on the Kidney in Cardiovascular Disease and Council on Clinical Cardiology. Cardiorenal syndrome: classification, pathophysiology, diagnosis, and treatment strategies: a scientific statement from the American Heart Association.
        Circulation. 2019; 139: e840-e878
        • Heuman D.M.
        • Mihas A.A.
        • Habib A.
        • et al.
        MELD-XI: a rational approach to “sickest first” liver transplantation in cirrhotic patients requiring anticoagulant therapy.
        Liver Transpl. 2007; 13: 30-37
        • Kamath P.S.
        • Wiesner R.H.
        • Malinchoc M.
        • et al.
        A model to predict survival in patients with end-stage liver disease.
        Hepatology. 2001; 33: 464-470
        • Kim M.S.
        • Kato T.S.
        • Farr M.
        • et al.
        Hepatic dysfunction in ambulatory patients with heart failure: application of the MELD scoring system for outcome prediction.
        J Am Coll Cardiol. 2013; 61: 2253-2261
        • Chen Y.
        • Liu Y.X.
        • Seto W.K.
        • et al.
        Prognostic value of hepatorenal function by modified Model for End-stage Liver Disease (MELD) score in patients undergoing tricuspid annuloplasty.
        J Am Heart Assoc. 2018; 7e009020
        • Hawkins R.B.
        • Young B.A.C.
        • Mehaffey J.H.
        • et al.
        Investigators for the Virginia Cardiac Services Quality Initiative. Model for End-Stage Liver Disease score independently predicts mortality in cardiac surgery.
        Ann Thorac Surg. 2019; 107: 1713-1719
        • Inohara T.
        • Kohsaka S.
        • Shiraishi Y.
        • et al.
        West Tokyo Heart Failure Registry Investigators. Prognostic impact of renal and hepatic dysfunction based on the MELD-XI score in patients with acute heart failure.
        Int J Cardiol. 2014; 176: 571-573
        • Adams E.D.
        • Jackson N.J.
        • Young T.
        • DePasquale E.C.
        • Reardon L.C.
        Prognostic utility of MELD-XI in adult congenital heart disease patients undergoing cardiac transplantation.
        Clin Transplant. 2018; 32e13257
        • Assenza G.E.
        • Graham D.A.
        • Landzberg M.J.
        • et al.
        MELD-XI score and cardiac mortality or transplantation in patients after Fontan surgery.
        Heart. 2013; 99: 491-496
        • Konno R.
        • Tatebe S.
        • Sugimura K.
        • et al.
        Prognostic value of the Model for End-Stage Liver Disease Excluding INR score (MELD-XI) in patients with adult congenital heart disease.
        PLoS One. 2019; 14e0225403
        • Egbe A.C.
        • Miranda W.R.
        • Dearani J.
        • Kamath P.S.
        • Connolly H.M.
        Prognostic role of hepatorenal function indexes in patients with Ebstein anomaly.
        J Am Coll Cardiol. 2020; 76: 2968-2976
        • Marelli A.J.
        • Mackie A.S.
        • Ionescu-Ittu R.
        • Rahme E.
        • Pilote L.
        Congenital heart disease in the general population: changing prevalence and age distribution.
        Circulation. 2007; 115: 163-172
        • Egbe A.
        • Miranda W.
        • Connolly H.
        • Dearani J.
        Haemodynamic determinants of improved aerobic capacity after tricuspid valve surgery in Ebstein anomaly.
        Heart. 2021; 107: 1138-1144
        • Rudski L.G.
        • Lai W.W.
        • Afilalo J.
        • et al.
        Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography.
        J Am Soc Echocardiogr. 2010; 23: 685-713
        • Dimopoulos K.
        • Diller G.P.
        • Koltsida E.
        • et al.
        Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease.
        Circulation. 2008; 117: 2320-2328
        • Ali A.M.
        • Dawson S.J.
        • Blows F.M.
        • et al.
        Comparison of methods for handling missing data on immunohistochemical markers in survival analysis of breast cancer.
        Br J Cancer. 2011; 104: 693-699
        • Grimm J.C.
        • Magruder J.T.
        • Do N.
        • et al.
        Modified Model for End-Stage Liver Disease Excluding INR (MELD-XI) score predicts early death after pediatric heart transplantation.
        Ann Thorac Surg. 2016; 101: 730-735
        • Egbe A.
        • Miranda W.R.
        • Connolly H.M.
        • et al.
        Temporal changes in liver stiffness after Fontan operation: results of serial magnetic resonance elastography.
        Int J Cardiol. 2018; 258: 299-304
        • Egbe A.C.
        • Miranda W.R.
        • Veldtman G.R.
        • Graham R.P.
        • Kamath P.S.
        Hepatic venous pressure gradient in fontan physiology has limited diagnostic and prognostic significance.
        CJC Open. 2020; 2: 360-364
        • Possner M.
        • Gordon-Walker T.
        • Egbe A.C.
        • et al.
        Hepatocellular carcinoma and the Fontan circulation: clinical presentation and outcomes.
        Int J Cardiol. 2021; 322: 142-148
        • Wu F.M.
        • Kogon B.
        • Earing M.G.
        • et al.
        Alliance for Adult Research in Congenital Cardiology (AARCC) Investigators. Liver health in adults with Fontan circulation: a multicentre cross-sectional study.
        J Thorac Cardiovasc Surg. 2017; 153: 656-664
        • Zomer A.C.
        • Vaartjes I.
        • van der Velde E.T.
        • et al.
        Heart failure admissions in adults with congenital heart disease; risk factors and prognosis.
        Int J Cardiol. 2013; 168: 2487-2493
        • Diller G.P.
        • Kempny A.
        • Alonso-Gonzalez R.
        • et al.
        Survival prospects and circumstances of death in contemporary adult congenital heart disease patients under follow-up at a large tertiary centre.
        Circulation. 2015; 132: 2118-2125
        • Oliver J.M.
        • Gallego P.
        • Gonzalez A.E.
        • et al.
        Pulmonary hypertension in young adults with repaired coarctation of the aorta: an unrecognised factor associated with premature mortality and heart failure.
        Int J Cardiol. 2014; 174: 324-329
        • Jain C.C.
        • Warnes C.A.
        • Egbe A.C.
        • et al.
        Hemodynamics in adults with the Shone complex.
        Am J Cardiol. 2020; 130: 137-142