Successful intermediate-term outcome for patients with cardiac amyloidosis undergoing heart transplantation: results of a multicenter survey.
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 recurrent amyloid in their allograft seen by electron microscopy at 3 1/2 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 transplantation with good intermediate-term results.
Hosenpud
J D
JD
Oregon Health Sciences University, Portland 97201.
Uretsky
B F
BF
Griffith
B P
BP
O'Connell
J B
JB
Olivari
M T
MT
Valantine
H A
HA
D000328:Adult; D000686:Amyloidosis / Q000401:mortality / Q000601:surgery*; D009202:Cardiomyopathies / Q000401:mortality / Q000601:surgery*; D003625:Data Collection; D005060:Europe; D005260:Female; D005500:Follow-Up Studies; D016027:Heart Transplantation / Q000401:mortality; D006801:Humans; D008297:Male; D008875:Middle Aged; D013997:Time Factors; D014481:United States