OUTCOME OF LIVER-TRANSPLANTATION IN PATIENTS WITH HEMOCHROMATOSIS

Recent preliminary reports suggest a poor outcome of orthotopic liver transplantation for patients with hemochromatosis. We analyzed an inst itutional experience with orthotopic liver transplantation for hemochr omatosis, 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. M ean age was 53 yr (range, 42 to 62 yr), and eight of nine patients wer e men. The diagnosis of hemochromatosis was based on transferrin satur ation > 62% and hepatic iron index > 2.0. Only two patients were known to have hemochromatosis before liver transplantation. All nine patien ts underwent standard cardiac evaluation before transplantation, and n o patient had detectable pre-existing cardiac disease. One patient had a major operative cardiac complication as a result of pulmonary embol ism and made a full recovery. Postoperatively, congestive heart failur e developed in three patients and four patients had arrhythmias. One p atient is undergoing phlebotomy for post-transplant cardiac complicati ons from hemochromatosis. Two patients had primary hepatic tumors in t he 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 s urvival 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 ot her adult liver transplant recipients (p < 0.01). In five of seven pat ients, post-transplant liver biopsies revealed hepatic iron accumulati on. We conclude that: (1) the survival of patients with hemochromatosi s after liver transplantation is decreased when compared with other re cipients; (2) the outcome after orthotopic liver transplantation for h emochromatosis cannot be predicted by standard pretransplant assessmen t, including cardiac evaluation; (3) cardiac, infectious and malignant complications account for excess morbidity and mortality post-transpl ant; and (4) confirmation of hemochromatosis and phlebotomy therapy pr etransplant might reduce cardiac complications after liver transplanta tion.