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Liver fibrosis in patients with tetralogy of Fallot, an unrecognised complication?

Authors
  • Ravndal, Maren E H1, 2
  • Borgwardt, Lise3
  • Juul, Klaus1
  • Nielsen, Jon1
  • Borgwardt, Lotte4
  • Henriksen, Birthe M4
  • Willemoe, Gro L5
  • Jensen, Annette S6
  • Christensen, Vibeke B1
  • Kjær, Mette S7
  • 1 Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. , (Denmark)
  • 2 Department of Cardiothoracic Surgery, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. , (Denmark)
  • 3 Department of Clinical Physiology, Nuclear Medicine and PET, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. , (Denmark)
  • 4 Department of Diagnostic Radiology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. , (Denmark)
  • 5 Department of Pathology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. , (Denmark)
  • 6 Department of Cardiology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. , (Denmark)
  • 7 Department of Gastroenterology and Hepatology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. , (Denmark)
Type
Published Article
Journal
Cardiology in the Young
Publisher
Cambridge University Press
Publication Date
Nov 01, 2021
Volume
31
Issue
11
Pages
1796–1806
Identifiers
DOI: 10.1017/S1047951121000901
PMID: 33719984
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

Improved survival has led to a growing population of adults with congenital heart disease (CHD), followed by numerous reports of late complications. Liver disease is a known complication in some patients, with most studies focusing on Fontan associated liver disease. Whether liver disease also exists in other patients with CHD is not fully investigated. Elevated central venous pressure is considered pivotal in the development of liver disease in Fontan associated liver disease, and other patients with alterations in central venous pressure may also be at risk for developing liver fibrosis. We wanted to see if liver fibrosis is present in patients with tetralogy of Fallot. Many patients with tetralogy of Fallot have severe pulmonary regurgitation, which can lead to elevated central venous pressure. Patients with tetralogy of Fallot may be at risk of developing liver fibrosis. Ten patients (24-56 years) with tetralogy of Fallot and pulmonary regurgitation were investigated for liver fibrosis. All patients were examined with magnetic resonance elastography of liver, hepatobiliary iminodiacetic acid scan, indocyanine green elimination by pulse spectrophotometry, elastography via FibroScan, abdominal ultrasound including liver elastography, and blood samples including liver markers. Three out of ten patients had findings indicating possible liver fibrosis. Two of these had a liver biopsy performed, which revealed fibrosis stage 1 and 2, respectively. The same three patients had an estimated elevated central venous pressure in previous echocardiograms. Mild liver fibrosis was present in selected patients with tetralogy of Fallot and may be related to elevated central venous pressure.

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