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

Part of: Advanced Imaging Tetralogy of Fallot (TOF)

Published online by Cambridge University Press:  15 March 2021

Maren E. H. Ravndal Open the ORCID record for Maren E. H. Ravndal [Opens in a new window] ,
Lise Borgwardt ,
Klaus Juul ,
Jon Nielsen ,
Lotte Borgwardt ,
Birthe M. Henriksen ,
Gro L. Willemoe ,
Annette S. Jensen ,
Vibeke B. Christensen  and
Mette S. Kjær
Maren E. H. Ravndal*
Affiliation:
Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark Department of Cardiothoracic Surgery, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
Lise Borgwardt
Affiliation:
Department of Clinical Physiology, Nuclear Medicine and PET, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
Klaus Juul
Affiliation:
Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
Jon Nielsen
Affiliation:
Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
Lotte Borgwardt
Affiliation:
Department of Diagnostic Radiology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
Birthe M. Henriksen
Affiliation:
Department of Diagnostic Radiology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
Gro L. Willemoe
Affiliation:
Department of Pathology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
Annette S. Jensen
Affiliation:
Department of Cardiology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
Vibeke B. Christensen
Affiliation:
Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
Mette S. Kjær
Affiliation:
Department of Gastroenterology and Hepatology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
*
Author for correspondence: Maren Emilie Hebeltoft Ravndal, MD, Department of Cardiothoracic Surgery, Copenhagen University Hospital, Rigshospitalet, Blegdamsvej 9, 2100, Copenhagen, Denmark. Tel: +45-35459631. E-mail: maren.emilie.hebeltoft.ravndal@regionh.dk
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Abstract

Objectives:

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.

Materials and methods:

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.

Results:

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.

Conclusions:

Mild liver fibrosis was present in selected patients with tetralogy of Fallot and may be related to elevated central venous pressure.

Keywords

Liver fibrosiscongenital heart diseaseliver complicationshepatocellular carcinomatetralogy of Fallot
Type
Original Article
Information
Cardiology in the Young , Volume 31 , Issue 11 , November 2021 , pp. 1796 - 1806
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Copyright
© The Author(s), 2021. Published by Cambridge University Press

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