Cardiac transplantation for amyloid heart disease: the United Kingdom experience.
BACKGROUND
Heart transplantation (TX) for cardiac amyloidosis is uncommon because of concern about progression of amyloid in other organs and the possibility of amyloid deposition in the donor heart.


METHODS
Records of all 24 patients with amyloid heart disease who have undergone TX in the United Kingdom were examined. Seventeen patients had AL amyloidosis (AL) and 7 had non-AL forms of amyloidosis (non-AL).


RESULTS
Survival of the 10 patients with AL who underwent TX but had no additional chemotherapy was 50%, 50%, and 20% at 1, 2, and 5 years, respectively; amyloid recurred in the grafts of these patients after a median of 11 months, and extra-cardiac amyloid deposition contributed to mortality in 70% of these patients. Survival of 7 patients with AL who also had chemotherapy was 71%, 71%, and 36% respectively and 2 patients remain alive. Survival of the 7 patients with non-AL was 86%, 86%, and 64% at 1, 2, and 5 years, respectively; 5 patients remain alive. One patient from this group had recurrence of amyloid in the graft at 60 months. Five-year survival for all 24 amyloid patients was 38%, compared to patients undergoing TX in the UK for other indications (n = 4,058) for whom it was 67% (p = 0.013).


CONCLUSIONS
Regardless of the use of adjunctive chemotherapy, the 5-year survival after TX for cardiac AL amyloidosis was less than that after TX for other indications, and progression of the systemic disease contributed substantially to the increased mortality. In contrast, the 5-year survival after TX for non-AL amyloid, combined as necessary with liver or kidney TX, was similar to that after TX in general.
Dubrey
Simon W
SW
The Imperial College School of Medicine, National Heart and Lung Institute, London, United Kingdom.
Burke
Margaret M
MM
Hawkins
Philip N
PN
Banner
Nicholas R
NR
D000206:Actuarial Analysis; D000686:Amyloidosis / Q000188:drug therapy / Q000401:mortality / Q000601:surgery*; D009202:Cardiomyopathies / Q000188:drug therapy / Q000401:mortality / Q000601:surgery*; D005260:Female; D016027:Heart Transplantation; D006801:Humans; D008297:Male; D008875:Middle Aged; D011182:Postoperative Care; D012008:Recurrence; D016019:Survival Analysis; D013997:Time Factors; D006113:United Kingdom