Heart transplantation in amyloidosis. Clinical and imaging manifestations




Santiago Decotto, Servicio de Cardiología, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina
Eugenia Villanueva, Servicio de Cardiología, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina
Diego Pérez de Arenaza, Servicio de Cardiología, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina
Elsa M. Nucifora, Servicio de Hematología, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina
María A. Aguirre, Servicio de Clínica, Área de Investigación en Medicina Interna, Instituto de Medicina Traslacional e Ingeniería Biomédica, CONICET, Instituto Universitario del Hospital Italiano, Ciudad Autónoma de Buenos Aires, Argentina
María L. Posadas-Martínez, Servicio de Clínica, Área de Investigación en Medicina Interna, Instituto de Medicina Traslacional e Ingeniería Biomédica, CONICET, Instituto Universitario del Hospital Italiano, Ciudad Autónoma de Buenos Aires, Argentina
Ricardo G. Marenchino, Servicio de Cirugía Cardiovascular, Hospital Italiano de Buenos Aires; Equipo de Trasplante Cardíaco de Adultos, Hospital Italiano de Buenos Aires. Ciudad Autónoma de Buenos Aires, Argentina
César A. Belziti, Servicio de Cardiología, Hospital Italiano de Buenos Aires; Equipo de Trasplante Cardíaco de Adultos, Hospital Italiano de Buenos Aires. Ciudad Autónoma de Buenos Aires, Argentina


Objective: To describe the clinical and imaging characteristics of heart transplantation patients due to amyloidosis in a community institution. Method: Descriptive case series of consecutive heart transplantation patients with amyloidosis in a medical center. All patients with diagnosis of amyloidosis with cardiac compromise receiving heart transplantation, performed in the period November 2008 to February 2021, were included in the analysis. Results: A total of 16 patients were included. The mean age was 59.9 years (± 10.2). 81.25% (n = 13) were male. According to the type of amyloidosis, 12 patients were transplanted for AL amyloidosis and 4 for ATTR amyloidosis. The most frequent clinical presentations were left overload (50%) and cardiogenic shock (32%). The mean ejection fraction prior to transplantation was 43% (± 16), 14 of the 16 patients had right ventricular dysfunction. The most common finding on cardiac magnetic resonance was the diffuse subendocardial late gadolinium enhancement pattern, with cancellation of the blood pool. The heart transplantation gave 6 patients the chance to receive a bone marrow transplantation afterwards. Conclusions: Heart transplantation has become an option for patients with heart failure due to AL and ATTR amyloidosis. In patients with AL amyloidosis, it might even allow bone marrow transplantation in a second stage.



Keywords: Amyloidosis. Heart transplantation. Heart failure.