Progression of systemic disease and reduced long-term survival in patients with cardiac amyloidosis undergoing heart transplantation. Follow-up results of a multicenter survey.

Amyloid heart disease has been considered a contraindication for cardiac transplant based on the hypothesis that it is a systemic disease and that amyloid deposition would occur in the cardiac allograft. A survey was sent to all of the US centers and a limited number of Canadian and European centers listed with the International Registry. Twenty-four centers responded, and data were ultimately provided for a total of 10 patients (3 men, 7 women, mean age 48 years, range 30-60 years) who were transplanted for cardiac amyloid. The diagnosis of cardiac amyloidosis was made histologically on endomyocardial biopsy and/or examination of the explanted heart. Additional documented organ involvement included liver (two of 10), rectal (three of 10), renal (two of 10), gingiva (two of 10), and tongue (one of 10), although invasive biopsies were not performed in a majority of patients. A specific amyloid protein was identified in eight patients (seven lambda, one kappa immunoglobulin light chain). Although four of the surviving nine patients (one perioperative death) developed recurrent amyloid deposition in the allograft, it was detected solely by electron microscopy in two of these and had no clinical significance. There was, however, a progressive risk of major organ involvement with organ function impairment in this group (22% at 12 months, 50% at 24 months, 66% at 48 months). Although the immediate and early postoperative outcomes were not dissimilar between this group and patients undergoing transplantation for other cardiac diseases, late survival was reduced (39% at 48 months) compared with the larger population, but differences were not statistically significant due to the small amyloid sample size (p = 0.16).(ABSTRACT TRUNCATED AT 250 WORDS)