Regression of Advanced Liver Fibrosis After Heart Transplantation in a Patient With Prior Fontan Surgery for Complex Congenital Heart Disease
2018; Lippincott Williams & Wilkins; Volume: 11; Issue: 11 Linguagem: Inglês
10.1161/circheartfailure.117.003754
ISSN1941-3297
AutoresJudith Bouchardy, Philippe Meyer, Patrick Yerly, C Blanche, Roger Hullin, Emiliano Giostra, Sylviane Hanquinet, Laura Rubbia‐Brandt, Nicole Sekarski, René Prêtre, Maurice Beghetti,
Tópico(s)Cardiac Structural Anomalies and Repair
ResumoHomeCirculation: Heart FailureVol. 11, No. 11Regression of Advanced Liver Fibrosis After Heart Transplantation in a Patient With Prior Fontan Surgery for Complex Congenital Heart Disease Free AccessCase ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplementary MaterialsFree AccessCase ReportPDF/EPUBRegression of Advanced Liver Fibrosis After Heart Transplantation in a Patient With Prior Fontan Surgery for Complex Congenital Heart Disease Judith Bouchardy, MD, Philippe Meyer, MD, Patrick Yerly, MD, Coralie Blanche, MD, Roger Hullin, MD, Emiliano Giostra, MD, Sylviane Hanquinet, MD, Laura Rubbia-Brandt, MD, Nicole Sekarski, MD, René Prêtre, MD and Maurice Beghetti, MD Judith BouchardyJudith Bouchardy Judith Bouchardy, MD, HUG, Service de Cardiologie, Rue Gabrielle-Perret-Gentil 4, 1211 Geneva, Switzerland. Email E-mail Address: [email protected] Division of Cardiology, Lausanne University Hospital, Switzerland. (J.B., P.Y., R.H.) Division of Cardiology, Geneva University Hospital, Switzerland (J.B., P.M., C.B.) , Philippe MeyerPhilippe Meyer Division of Cardiology, Geneva University Hospital, Switzerland (J.B., P.M., C.B.) , Patrick YerlyPatrick Yerly Division of Cardiology, Lausanne University Hospital, Switzerland. (J.B., P.Y., R.H.) , Coralie BlancheCoralie Blanche , Roger HullinRoger Hullin Division of Cardiology, Lausanne University Hospital, Switzerland. (J.B., P.Y., R.H.) , Emiliano GiostraEmiliano Giostra Division of Gastro-Enterology and Hepatology, Geneva University Hospital, Switzerland (E.G.) , Sylviane HanquinetSylviane Hanquinet , Laura Rubbia-BrandtLaura Rubbia-Brandt Radio-pediatric Unit, Division of Radiology, Geneva University Hospital, Switzerland (S.H.) Division of Pathology, Geneva University Hospital, Switzerland (L.R.-B.) , Nicole SekarskiNicole Sekarski Pediatric Cardiology Unit, Lausanne University Hospital, Switzerland. (N.S.) Centre Universitaire Romand de Cardiologie et Chirurgie Cardiaque Pédiatrique, Lausanne University Hospital, Switzerland. (N.S., R.P., M.B.) Division of Pathology, Geneva University Hospital, Switzerland (L.R.-B.) Centre Universitaire Romand de Cardiologie et hirurgie Cardiaque Pédiatrique, Geneva University Hospital, Switzerland (N.S., R.P., M.B.) , René PrêtreRené Prêtre Centre Universitaire Romand de Cardiologie et Chirurgie Cardiaque Pédiatrique, Lausanne University Hospital, Switzerland. (N.S., R.P., M.B.) Division of Cardiac Surgery, Lausanne University Hospital, Switzerland. (R.P.) Centre Universitaire Romand de Cardiologie et hirurgie Cardiaque Pédiatrique, Geneva University Hospital, Switzerland (N.S., R.P., M.B.) and Maurice BeghettiMaurice Beghetti Centre Universitaire Romand de Cardiologie et Chirurgie Cardiaque Pédiatrique, Lausanne University Hospital, Switzerland. (N.S., R.P., M.B.) Division of Cardiology, Geneva University Hospital, Switzerland (J.B., P.M., C.B.) Division of Pathology, Geneva University Hospital, Switzerland (L.R.-B.) Paediatric Cardiology Unit, Department of Child and Adolescent, Geneva University Hospital, Switzerland (M.B.) Centre Universitaire Romand de Cardiologie et hirurgie Cardiaque Pédiatrique, Geneva University Hospital, Switzerland (N.S., R.P., M.B.) Originally published16 Nov 2018https://doi.org/10.1161/CIRCHEARTFAILURE.117.003754Circulation: Heart Failure. 2018;11:e003754Palliative Fontan surgery offers enhanced survival1 and quality of life for patients with univentricular heart. Despite reported long-term survival,1 progressive failure of the Fontan circulation is expected, characterized by ventricular dysfunction and systemic complications, such as Fontan-associated liver disease (FALD). Heart transplantation (HTx) might be the only potential treatment. We report the case of a patient with Fontan failure and advanced liver dysfunction with histological and functional regression of advanced liver fibrosis after HTx.Case PresentationA 20-year-old male patient was followed at the adult congenital heart disease outpatient clinic after Fontan palliation (extra-cardiac tunnel) at the age of 10 years for complex congenital heart disease. After an unremarkable pediatric course, he presented with an inaugural episode of heart failure at the age of 19 years. He progressively developed chronic Fontan circulation failure because of severe systolic ventricular dysfunction and atrioventricular valve insufficiency (see hemodynamic data in Table I in the Data Supplement). We decided to evaluate him for HTx because of progressive functional decline and development of ascites requiring recurrent hospitalizations. Despite normal liver enzymes and no frank signs of cirrhosis on abdominal computed tomography scan and hepatic ultrasound (Table II and Figure I in the Data Supplement), we performed a transcutaneous liver biopsy because of ascites and decreased synthetic liver function assessed by factor V activity but normal albumin. The patient was Child-Pugh class A. The Model for End-Stage Liver Disease and Model for End-Stage Liver Disease-XI scores, known to predict clinical outcomes, were respectively calculated at 9 and 11 (low mortality risk). No transhepatic gradient was measured, as the biopsy was done transcutaneously. Histology revealed extensive fibrosis with centrolobular to portal tract bridging fibrous septa, annular fibrosis with area of architectural nodular changes (Figure [A]), and confluent fibrosis with parenchymal alteration and dissociation (Figure [B]). The distribution of fibrosis was not typical for the so-called cardiac cirrhosis. Moderate sinusoidal dilatation was present (Figure [C]). Other causes of liver cirrhosis were excluded. The question of a combined heart-liver transplantation was raised. Considering clinically mildly advanced liver disease and potentially longer ischemic time for the transplanted liver because of the complex cardiac anatomy, the patient was listed for HTx only. The teams were prepared to list the patient for urgent liver transplantation if needed in the immediate postoperative course.Download figureDownload PowerPointFigure. Liver biopsies of >2.5 cm length before and after heart transplantation. Liver biopsy before transplantation illustrates (A) a cirrhotic pattern with extensive bridging and annular fibrosis and regenerative nodule (Masson's Fontana stain; B) large centrilobular perisinusoidal area of fibrosis with hepatocyte replacement (arrow) and alteration of trabecular and (C) area of moderate sinusoidal dilatation (hematoxylin and eosin stain). Liver biopsy after transplantation shows (D, E) absence of fibrosis and mostly regular lobular architecture (Masson's Fontana stain) and (F) no significant sinusoidal dilatation (hematoxylin and eosin stain).He was transplanted at the age of 24 years with an unremarkable postoperative course. After 18 months of follow-up free from any posttransplant complications, liver biopsy was repeated. The biopsy (Figure [D]) revealed regression of the fibrosis and of the architectural anomalies (Figure [D and E]) compatible with reversal of the cirrhotic process. Sinusoidal dilatation was no more present (Figure [F]).DiscussionFontan surgery for univentricular hearts has evolved since first performed by Fontan himself but the basic underlying physiology remains unchanged, with passive circulation driving the systemic venous return to the lungs. The gain in arterial blood oxygen saturation is made at the expense of elevated systemic venous and lymphatic pressures.Effects of chronically elevated right atrial pressure on hepatic function have increasingly been documented by liver biopsy2 and autopsy studies. FALD, defined as abnormalities in liver structure and function, including cirrhosis, ascites, synthetic dysfunction, hepatocarcinoma, and portal hypertension, results from Fontan circulation3 and its inevitable chronic venous and lymphatic congestion combined with reduced cardiac output. Laboratory testing may reveal mild cholestasis and elevation of transaminases with preserved or mildly reduced synthetic function. Noninvasive techniques such as liver elastography are part of the recommended screening for FALD but seem not to reliably reflect hepatic pathology.3 Liver biopsy demonstrates highly prevalent centrolobular hepatic fibrosis, portal fibrosis, and liver cirrhosis4 with poor correlation with clinical history, hemodynamic measurements, biochemical data, and clinical outcomes,4 as in our case. The degree of FALD is particularly crucial when discussing HTx.3Fontan operation remains a palliative procedure and failure of the Fontan circulation seems to inexorably develop presumably because of low cardiac output and chronic venous congestion. Ventricular dysfunction, already present in some children with Fontan procedure, can progress in adulthood. Standard medical therapy for heart failure has failed to demonstrate benefits.When decreased ejection fraction and increased end-diastolic ventricular pressures preclude Fontan revision, HTx provides the ultimate treatment option in the long-term. Recent studies demonstrate that adults with congenital heart disease have survival rates rivaling those of other patients and that Fontan surgery does not seem to be a risk factor for increased mortality. In adult undergoing cardiac surgery or HTx, liver dysfunction and cirrhosis are associated with significant morbidity and mortality. In the Fontan population, the risk of perioperative acute liver failure in the setting of preexisting liver damage remains high. Impact of FALD on posttransplant outcome is not well described.Cirrhosis is increasingly recognized to be a dynamic process. Our case nicely demonstrates that advanced liver fibrosis might regress post-HTx in selected Fontan patients with FALD.DisclosuresNone.FootnotesThe Data Supplement is available at https://www.ahajournals.org/doi/suppl/10.1161/CIRCHEARTFAILURE.117.003754.Judith Bouchardy, MD, HUG, Service de Cardiologie, Rue Gabrielle-Perret-Gentil 4, 1211 Geneva, Switzerland. Email judith.[email protected]chReferences1. d'Udekem Y, Iyengar AJ, Galati JC, Forsdick V, Weintraub RG, Wheaton GR, Bullock A, Justo RN, Grigg LE, Sholler GF, Hope S, Radford DJ, Gentles TL, Celermajer DS, Winlaw DS. Redefining expectations of long-term survival after the Fontan procedure: twenty-five years of follow-up from the entire population of Australia and New Zealand.Circulation. 2014; 130(11suppl 1):S32–S38. doi: 10.1161/CIRCULATIONAHA.113.007764LinkGoogle Scholar2. Schwartz MC, Sullivan LM, Glatz AC, Rand E, Russo P, Goldberg DJ, Rome JJ, Cohen MS. Portal and sinusoidal fibrosis are common on liver biopsy after Fontan surgery.Pediatr Cardiol. 2013; 34:135–142. doi: 10.1007/s00246-012-0402-9CrossrefMedlineGoogle Scholar3. Greenway SC, Crossland DS, Hudson M, Martin SR, Myers RP, Prieur T, Hasan A, Kirk R. Fontan-associated liver disease: implications for heart transplantation.J Heart Lung Transplant. 2016; 35:26–33. doi: 10.1016/j.healun.2015.10.015CrossrefMedlineGoogle Scholar4. Wu FM, Jonas MM, Opotowsky AR, Harmon A, Raza R, Ukomadu C, Landzberg MJ, Singh MN, Valente AM, Egidy Assenza G, Perez-Atayde AR. Portal and centrilobular hepatic fibrosis in Fontan circulation and clinical outcomes.J Heart Lung Transplant. 2015; 34:883–891. doi: 10.1016/j.healun.2015.01.993CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Dipchand A, Honjo O, Alonso-Gonzalez R, McDonald M and Roche S (2022) Heart Transplant Indications, Considerations, and Outcomes in Fontan Patients: Age-Related Nuances, Transplant Listing, and Disease-Specific Indications, Canadian Journal of Cardiology, 10.1016/j.cjca.2022.02.019, 38:7, (1072-1085), Online publication date: 1-Jul-2022. Hilscher M, Wells M, Venkatesh S, Cetta F and Kamath P (2022) Fontan‐associated liver disease, Hepatology, 10.1002/hep.32406, 75:5, (1300-1321), Online publication date: 1-May-2022. Broda C, Alonso-Gonzalez R, Ghanekar A, Gulamhusein A, McDonald M, Luk A, Kobulnik J, Billia F, Heggie J, Jariani M, Honjo O, Barron D, Hickey E and Roche S (2022) Fate of the liver in the survivors of adult heart transplant for a failing Fontan circulation, The Journal of Heart and Lung Transplantation, 10.1016/j.healun.2021.10.020, 41:3, (283-286), Online publication date: 1-Mar-2022. Téllez L, Rodríguez de Santiago E and Albillos A (2021) Fontan-Associated Liver Disease: Pathophysiology, Staging, and Management, Seminars in Liver Disease, 10.1055/s-0041-1732355, 41:04, (538-550), Online publication date: 1-Nov-2021. Rodriguez D, Mao C, Mahle W, Kanter K, Alazraki A, Braithwaite K, Rytting H, Caltharp S, Magliocca J and Romero R (2021) Pretransplantation and Post-Transplantation Liver Disease Assessment in Adolescents Undergoing Isolated Heart Transplantation for Fontan Failure, The Journal of Pediatrics, 10.1016/j.jpeds.2020.09.044, 229, (78-85.e2), Online publication date: 1-Feb-2021. Schleiger A, Kramer P, Salzmann M, Danne F, Schubert S, Bassir C, Müller T, Tacke F, Müller H, Berger F, Photiadis J and Ovroutski S (2020) Evaluation of Fontan failure by classifying the severity of Fontan-associated liver disease: a single-centre cross-sectional study, European Journal of Cardio-Thoracic Surgery, 10.1093/ejcts/ezaa310, 59:2, (341-348), Online publication date: 29-Jan-2021. Emamaullee J, Zaidi A, Schiano T, Kahn J, Valentino P, Hofer R, Taner T, Wald J, Olthoff K, Bucuvalas J and Fischer R (2020) Fontan-Associated Liver Disease, Circulation, 142:6, (591-604), Online publication date: 11-Aug-2020. Keung C, Zentner D, Gibson R, Phan D, Grigg L, Sood S and Nicoll A (2019) Fontan-associated liver disease: pathophysiology, investigations, predictors of severity and management, European Journal of Gastroenterology & Hepatology, 10.1097/MEG.0000000000001641, 32:8, (907-915), Online publication date: 1-Aug-2020. November 2018Vol 11, Issue 11 Advertisement Article InformationMetrics © 2018 American Heart Association, Inc.https://doi.org/10.1161/CIRCHEARTFAILURE.117.003754PMID: 30571198 Originally publishedNovember 16, 2018 KeywordsFontan procedureliver fibrosisPDF download Advertisement SubjectsCongenital Heart DiseasePrognosisTransplantation
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