Combined heart–liver transplantation in a case of haemochromatosis

Hereditary haemochromatosis results in multiorgan dysfunction secondary to iron overload. Haemojuvelin (HJV)-associated haemochromatosis, is a rapidly progressing form of haemochromatosis caused by mutation in the HJV that frequently results in heart and liver failure. Herein, we describe the succes...

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Bibliographic Details
Published in:BMJ case reports 2021-05, Vol.14 (5), p.e241508
Main Authors: Shubin, Andrew D, De Gregorio, Lucia, Hwang, Christine, MacConmara, Malcolm
Format: Article
Language:English
Subjects:
Abdomen
Adult
Arthritis
Biopsy
Cardiomyopathy
Case Report
Case reports
Female
Genes
Heart failure
Heart Transplantation
Hemochromatosis - complications
Histology
Humans
Hypotension
Iron Overload - etiology
Ischemia
Liver
Liver cirrhosis
Liver diseases
Liver Transplantation
Liver transplants
Mutation
Patients
Phlebotomy
Proteins
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Summary:Hereditary haemochromatosis results in multiorgan dysfunction secondary to iron overload. Haemojuvelin (HJV)-associated haemochromatosis, is a rapidly progressing form of haemochromatosis caused by mutation in the HJV that frequently results in heart and liver failure. Herein, we describe the successful treatment of a 39-year-old woman with decompensated heart failure and liver cirrhosis requiring extracorporeal membrane oxygenation who was successfully treated with combined heart–liver transplantation. Following her life-saving multiorgan transplantation, she was also noted to have rapid correction of her serum ferritin to normal levels. She remains healthy with excellent allograft function and normal iron and ferratin levels 4 years after the procedure. To our knowledge, this case is the first demonstration that combined heart–liver transplantation is a feasible option for patients with heart and liver failure secondary to HJV-associated haemochromatosis and indeed offers a long-standing corrective solution to treat this condition and restore physiologically normal iron metabolism.
ISSN:1757-790X
1757-790X
DOI:10.1136/bcr-2020-241508