Introduction - Cardiac amyloidosis either caused by immunoglobulin light chains (AL) or deposition of transthyretin (ATTR) is associated with a poor prognosis. At an advanced stage of the disease, heart transplantation (HTX) represents a treatment option for selected patients. We here present the results of post-transplantation outcomes in cardiac amyloidosis within the Eurotransplant area, investigating possible predictors of survival.

Methods - One hundred-fifteen patients undergoing HTX due to cardiac amyloidosis were carried out in the Eurotransplant region between November 1987 and May 2020. For 81 of these patients, detailed assessment prior to transplantation was reported by the transplant centers, including right heart catheterization, echocardiography and biomarkers. The present study was conducted in a retrospective approach. Primary endpoint was overall survival. Baseline variables were entered in a cox proportional hazard model with the primary endpoint as a dependent variable.

Results - Median overall survival following HTX was 6.1 years, without significant difference between AL and ATTR patients. Univariate cox proportional hazard model revealed a significant relationship between year of transplantation (hazard ratio 0.95, p=0.039), the transplantation period (2008 to 2020 vs 1987 to 2008; hazard ratio 0.53, p=0.05), albumin concentration at baseline (hazard ratio 0.97, p=0.001), systolic blood pressure at baseline (hazard ratio 0.96, p=0.006) and overall survival.

Conclusion - HTX is a feasible treatment option for patients at an advanced stage of cardiac amyloidosis and overall survival after HTX has improved in the modern age. Careful patient selection and modern chemotherapy strategies for AL patients are important factors to improve outcome. Still, patients at a very advanced stage of the disease characterized by compromised blood pressure and albumin levels show worse outcome following HTX.