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Liver disease after the Fontan procedure: what the hepatologist needs to know

Hiral Shah1, Karen Kuehl, Averell H Sherker

  • 1Division of Gastroenterology and Hepatology, Department of Medicine, Washington Hospital Center, Washington, DC 20010, USA. hiral.shah@medstar.net

Journal of Clinical Gastroenterology
|March 11, 2010
PubMed

Insights

Congenital heart disease (CHD) survival has dramatically improved, with many patients now reaching adulthood. However, long-term complications like liver issues after the Fontan operation require integrated care from CHD specialists and hepatologists.

Area of Science:

  • Cardiology
  • Hepatology
  • Pediatric Surgery

Background:

  • Congenital heart disease (CHD) survival rates have significantly increased over the past 50 years.
  • A substantial population of adults with CHD now exists, with an estimated 80-85% reaching adulthood.
  • The Fontan operation is a primary surgical repair for univentricular heart physiology, leading to a growing adult CHD population.

Purpose of the Study:

  • To highlight the increasing prevalence of adults living with CHD.
  • To emphasize the emerging long-term morbidities, particularly liver complications, following the Fontan operation.
  • To advocate for collaborative care between CHD specialists and hepatologists for this patient group.

Main Methods:

  • Review of historical survival data for infants with CHD.
  • Analysis of current adult CHD population statistics in the United States.
  • Discussion of the established role and long-term sequelae of the Fontan operation.
  • Emphasis on the need for interdisciplinary management strategies.

Main Results:

  • Significant improvement in CHD survival, with a large adult population now living with the condition.
  • Increasing recognition of long-term complications, especially liver issues, in adults post-Fontan operation.
  • The Fontan operation remains a cornerstone for univentricular physiology but carries significant long-term risks.

Conclusions:

  • The management of adults with CHD requires specialized attention to long-term morbidities.
  • Liver complications are a significant concern in the growing adult CHD population post-Fontan.
  • Integrated care involving CHD specialists and hepatologists is crucial for optimizing outcomes in adults with CHD.

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