Background
Orthotopic heart transplant (OHT) followed by myeloablative chemotherapy and autologous stem cell transplant (ASCT) has been successful in the treatment of light chain (AL) cardiac amyloidosis. The purpose of this study is to identify predictors of survival to OHT in patients with end stage heart failure due to AL amyloidosis, and compare post-OHT survival of cardiac amyloid patients to that of other cardiomyopathy patients undergoing OHT.
Methods
From January 2000 to June 2011, 31 patients with end stage heart failure secondary to AL amyloidosis were listed for OHT at Massachusetts General Hospital (MGH). Univariate and multivariate regression analyses identified predictors of survival to OHT. Kaplan-Meier analysis compared survival between MGH amyloidosis patients and the Scientific Registry of Transplant Recipients (SRTR) non-amyloid cardiomyopathy patients.
Results
Low body mass index (BMI) was the only predictor of survival to OHT in patients with end stage heart failure due to cardiac amyloidosis. Survival of cardiac amyloid patients who died prior to receiving a donor heart was only 63 ± 45 days after listing. Patients who survived to OHT received a donor organ at 53 ± 48 days after listing. Survival of AL amyloidosis patients on the waitlist was less than patients waitlisted for all other non-amyloid diagnoses. The long-term survival of transplanted amyloid patients was no different than the survival of non-amyloid, restrictive (p=0.34), non-amyloid dilated (p=0.34) or all non-amyloid cardiomyopathy patients (p=0.22) in the SRTR database.
Conclusions
Those that survive to OHT followed by ASCT have a survival rate similar to other cardiomyopathy patients undergoing OHT. However, more than one third of the patients died awaiting OHT. The only predictor of survival to OHT in AL amyloidosis patients was low BMI, which correlated with shorter waitlist time. To optimize the survival of these patients, access to donor organs must be improved.
In light chain (AL) amyloidosis, amyloid fibrils derived from clonal lambda or kappa immunoglobulin light chains deposit abnormally in organs. Cardiac involvement is apparent echocardiographically in 60% of AL amyloidosis patients at the time of diagnosis, with clinical evidence of heart failure in 69% of patients.1 The median survival of AL amyloidosis patients presenting with any heart failure symptom is 8.5 months2 and even less for end-stage heart failure pateints.
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