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Long‐term outcome in patients treated with combined heart and liver transplantation for familial amyloidotic cardiomyopathy
Author(s) -
Nelson Lærke M.,
Penninga Luit,
Sander Kaare,
Hansen Peter B.,
Villadsen Gerda E.,
Rasmussen Allan,
Gustafsson Finn
Publication year - 2012
Publication title -
clinical transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.918
H-Index - 76
eISSN - 1399-0012
pISSN - 0902-0063
DOI - 10.1111/ctr.12053
Subject(s) - medicine , amyloidosis , transthyretin , liver transplantation , cardiac amyloidosis , transplantation , ejection fraction , cardiomyopathy , restrictive cardiomyopathy , heart transplantation , heart failure , surgery , gastroenterology , cardiology
Background The amyloidogenic transthyretin ( ATTR ) mutation L eu111 M et causes a primarily cardiac amyloidosis: Familial amyloidotic cardiomyopathy ( FAC ). Combined heart–liver transplantation ( CHLT x) is the preferred treatment for patients with heart failure due to familial amyloidosis, but information on outcome of patients with L eu111 M et mutation is limited. The aim of this study was to evaluate the long‐term outcome of CHLT x in patients with FAC . Methods and materials Between 1998 and 2009, CHLT x was performed in 7 FAC patients (four men). Six patients underwent simultaneous transplantation. All patients suffered from severe cardiomyopathy. Results Mean recipient age at transplantation was 48.3 ± 4.2 yr. Mean follow‐up was 55 months. No peroperative mortality occured. Two patients died within the first year (infection, multi‐organ failure) of transplantation. Cumulative survival at 4.5 yr was 71%. No significant liver rejections occurred. One patient experienced an episode of cardiac rejection requiring treatment ( H 2 R ). For the surviving five patients, most recent left ventricular ejection fraction was 0.61 ± 0.02, and plasma creatinine was 129 ± 47 μM. None developed significant allograft vasculopathy or neuropathy after transplantation. No recurrence of cardiac amyloid was found. Conclusions CHLT x in selected patients with FAC due to L eu111 M et mutation offers acceptable long‐term survival, almost comparable with isolated cardiac transplantation. Allograft rejection was rare.