Fontan-Associated Liver Disease

Author:

Emamaullee Juliet12,Zaidi Ali N.34,Schiano Thomas5,Kahn Jeffrey6,Valentino Pamela L.7,Hofer Ryan E.8,Taner Timucin910,Wald Joyce W.11,Olthoff Kim M.12,Bucuvalas John13,Fischer Ryan14

Affiliation:

1. Liver Transplant Center, Children’s Hospital–Los Angeles, CA (J.E.).

2. Department of Surgery (J.E.), Keck School of Medicine, University of Southern California, Los Angeles.

3. Mount Sinai Cardiovascular Institute (A.N.Z.), Recanati/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, NY.

4. The Children’s Heart Center (A.N.Z.), Recanati/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, NY.

5. Divisions of Hepatology (T.S.), Recanati/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, NY.

6. Division of Gastrointestinal and Liver Diseases (J.K.), Keck School of Medicine, University of Southern California, Los Angeles.

7. Section of Gastroenterology and Hepatology, Department of Pediatrics, Yale University School of Medicine, New Haven, CT (P.L.V.).

8. Departments of Anesthesiology and Perioperative Medicine (R.E.H.), Mayo Clinic, Rochester, MN.

9. Surgery (T.T.), Mayo Clinic, Rochester, MN.

10. Immunology (T.T.), Mayo Clinic, Rochester, MN.

11. Division of Cardiology, Department of Medicine (J.W.W.), University of Pennsylvania, Philadelphia, PA.

12. Department of Surgery (K.M.O.), University of Pennsylvania, Philadelphia, PA.

13. Pediatric Hepatology (J.B.), Recanati/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, NY.

14. Department of Gastroenterology, Liver Care Center, Children’s Mercy Kansas City, MO (R.F.).

Abstract

Surgical innovation and multidisciplinary management have allowed children born with univentricular physiology congenital heart disease to survive into adulthood. An estimated global population of 70 000 patients have undergone the Fontan procedure and are alive today, most of whom are <25 years of age. Several unexpected consequences of the Fontan circulation include Fontan-associated liver disease. Surveillance biopsies have demonstrated that virtually 100% of these patients develop clinically silent fibrosis by adolescence. As they mature, there are increasing reports of combined heart-liver transplantation resulting from advanced liver disease, including bridging fibrosis, cirrhosis, and hepatocellular carcinoma, in this population. In the absence of a transplantation option, these young patients face a poor quality of life and overall survival. Acknowledging that there are no consensus guidelines for diagnosing and monitoring Fontan-associated liver disease or when to consider heart transplantation versus combined heart-liver transplantation in these patients, a multidisciplinary working group reviewed the literature surrounding Fontan-associated liver disease, with a specific focus on considerations for transplantation.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Physiology (medical),Cardiology and Cardiovascular Medicine

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