SUCCESSFUL INTERMEDIATE-TERM OUTCOME FOR PATIENTS WITH CARDIAC AMYLOIDOSIS UNDERGOING HEART-TRANSPLANTATION - RESULTS OF A MULTICENTER SURVEY

  • 1 July 1990
    • journal article
    • research article
    • Vol. 9  (4) , 346-350
Abstract
Amyloid heart disease has been considered a contraindication for heart transplant on the basis of the hypothesis that it is a systemic disease and that amyloid deposition would occur in the cardiac allograft. Despite these concerns, several centers have performed heart transplantation for amyloidosis in a limited number of cases. A survey was sent to all of the U.S. centers and a limited number of European centers listed with the Registry of the International Society for Heart Transplantation. Nineteen centers responded, and data were provided for a total of seven patients (three men and four women, with a mean age of 46 years, range, 30 to 60 years) who had transplants for cardiac amyloid. The diagnosis of cardiac amyloidosis was made histologically on endomyocardial biopsy and/or examination of the explanted heart. Additional organ involvement included liver (two cases), rectal (two cases), renal (one case), gingiva (one case), and tongue (one case). A specific amyloid protein was identified in five patients (four lambda, one kappa Ig light chain). Two patients developed recurred amyloid in their allograft seen by electron microscopy at 31/2 and 4 months, respectively. One patient developed progressive diastolic dysfunction, but systolic function was preserved. This patient died 13 months after transplantation as a result of progressive liver infiltration with amyloid. One patient died immediately after operation. Five patients are alive and fully rehabilitated 32 .+-. 12 months after transplant. On the basis of this small series, some patients with cardiac amyloidosis can undergo heart transplant with good intermediate-term results.

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