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Outcome of liver transplantation in patients with hemochromatosis
Author(s) -
Farrell Frank J.,
Nguyen Marie,
Woodley Susan,
Imperial Joanne C.,
GarciaKennedy Richard,
Man Kevin,
Esquivel Carlos O.,
Keeffe Emmet B.
Publication year - 1994
Publication title -
hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.488
H-Index - 361
eISSN - 1527-3350
pISSN - 0270-9139
DOI - 10.1002/hep.1840200221
Subject(s) - medicine , hemochromatosis , transplantation , liver transplantation , hereditary hemochromatosis , heart failure , complication , gastroenterology , phlebotomy , heart transplantation , surgery
Recent preliminary reports suggest a poor outcome of orthotopic liver transplantation for patients with hemochromatosis. We analyzed an institutional experience with orthotopic liver transplantation for hemochromatosis, focusing on factors contributing to increased morbidity and mortality. Between March 1988 and October 1992, nine of 249 adults (3.6%) undergoing orthotopic liver transplantation had hemochromatosis. Mean age was 53 yr (range, 42 to 62 yr), and eight of nine patients were men. The diagnosis of hemochromatosis was based on transferrin saturation>62% and hepatic iron index>2.0. Only two patients were known to have hemochromatosis before liver transplantation. All nine patients underwent standard cardiac evaluation before transplantation, and no patient had detectable pre‐existing cardiac disease. One patient had a major operative cardiac complication as a result of pulmonary embolism and made a full recovery. Postoperatively, congestive heart failure developed in three patients and four patients had arrhythmias. One patient is undergoing phlebotomy for post‐transplant cardiac complications from hemochromatosis. Two patients had primary hepatic tumors in the explant liver. There were four deaths caused by multiorgan failure with congestive heart failure (1), infection (2), and/or malignancy (2). Five patients are alive 3 to 25 mo post‐transplant. The actuarial survival of the nine patients was 53% at 25 mo vs. 89% for 18 age‐ and sex‐matched control transplant recipients (p=0.1) and 81% for all other adult liver transplant recipients (p<0.01). In five of seven patients, post‐transplant liver biopsies revealed hepatic iron accumulation. We conclude that: (1) the survival of patients with hemochromatosis after liver transplantation is decreased when compared with other recipients; (2) the outcome after orthotopic liver transplantation for hemochromatosis cannot be predicted by standard pretransplant assessment, including cardiac evaluation; (3) cardiac, infectious and malignant complications account for excess morbidity and mortality post‐transplant; and (4) confirmation of hemochromatosis and phlebotomy therapy pretransplant might reduce cardiac complications after liver transplantation. (Hepatology 1994;20:404–410.)