Long-Term Outcomes of Orthotopic Heart Transplantation in Patients with Cardiac Amyloidosis: Differential Survival by Amyloid Type

Abstract

PurposeOrthotopic heart transplantation (OHT) due to cardiac amyloidosis (CAM) is controversial because of concerns about long-term survival and recurrence in the allograft. Since AL amyloid is a systemic disease we hypothesized that long term outcomes of OHT in CAM would differ between primary (AL) and transthyretin (ATTR) CAM.Methods and MaterialsWe retrospectively reviewed 26 patients with CAM undergoing OHT surgery at our institution, from July 2001 to July 2012. Patients were grouped based on CAM type as having AL or TTR amyloidosis. The primary endpoint was all-cause mortality after OHT. Kaplan-Meier estimates were used to determine actuarial survival.ResultsWhen stratified into AL (n=15) and TTR (n=11) groups, patients were 51.2±7 and 55.8±8 years old (p=0.077), had preoperative serum creatinine of 1.33±0.45 and 1.75±0.89 (p=0.13), respectively, and included 69% males. Mean follow-up was 28.9±31.3 months (range 2-110). Corresponding in-hospital mortality was not noted. Of 15 AL patients undergoing successful OHT, stem cell transplantation was performed in 13 (3 prior to OHT) and 3 had recurrence of CAM whereas recurrence was not noted in TTR patients. Twelve patients (46%) died during follow-up; 9 AL patients and 3 with TTR. The mean survival time was 8± 29.9 months for the patients who died. Survival for CAM patients undergoing OHT was similar in both groups at 1 month. However, the AL group compared to TTR had significantly lower 5-year survival (44.4±13.2% vs. 71.4±17.1%; p = 0.029). [figure 1]ConclusionsSurvival differs between AL and ATTR cardiac amyloidosis after OHT with a significantly better survival for TTR CAM patients. Orthotopic heart transplantation (OHT) due to cardiac amyloidosis (CAM) is controversial because of concerns about long-term survival and recurrence in the allograft. Since AL amyloid is a systemic disease we hypothesized that long term outcomes of OHT in CAM would differ between primary (AL) and transthyretin (ATTR) CAM. We retrospectively reviewed 26 patients with CAM undergoing OHT surgery at our institution, from July 2001 to July 2012. Patients were grouped based on CAM type as having AL or TTR amyloidosis. The primary endpoint was all-cause mortality after OHT. Kaplan-Meier estimates were used to determine actuarial survival. When stratified into AL (n=15) and TTR (n=11) groups, patients were 51.2±7 and 55.8±8 years old (p=0.077), had preoperative serum creatinine of 1.33±0.45 and 1.75±0.89 (p=0.13), respectively, and included 69% males. Mean follow-up was 28.9±31.3 months (range 2-110). Corresponding in-hospital mortality was not noted. Of 15 AL patients undergoing successful OHT, stem cell transplantation was performed in 13 (3 prior to OHT) and 3 had recurrence of CAM whereas recurrence was not noted in TTR patients. Twelve patients (46%) died during follow-up; 9 AL patients and 3 with TTR. The mean survival time was 8± 29.9 months for the patients who died. Survival for CAM patients undergoing OHT was similar in both groups at 1 month. However, the AL group compared to TTR had significantly lower 5-year survival (44.4±13.2% vs. 71.4±17.1%; p = 0.029). [figure 1] Survival differs between AL and ATTR cardiac amyloidosis after OHT with a significantly better survival for TTR CAM patients.