ACUTE DILATIVE CARDIOMYOPATHY FOLLOWING LIVER-TRANSPLANTATION FOR IDIOPATHIC HEMOCHROMATOSIS

Liver transplantation was performed in a 29-year-old male suffering fr om end-stage liver disease due to idiopathic hemochromatosis. History, clinical examination, chest X-ray and ECG revealed no signs of cardia c dysfunction. Apart from intraabdominal bleedings necessitating relap arotomy on day 1, the immediate postoperative course was entirely unev entful and the patient enjoyed excellent graft function. Six days foll owing transplantation he developed rapidly progressive heart failure w hich, despite high-dose catecholamines, led to death only 24 hours lat er. Postmortem examination revealed acute dilative cardiomyopathy due to iron storage disease of the heart. Iron extraction from the heart i n order to meet postoperative iron requirements is discussed as a poss ible cause. From this observation it is concluded that echocardiograph ic examination and perhaps endomyocardial biopsy should be included in the pretransplant work-up of patients with idiopathic hemochromatosis .