HEART TRANSPLANTATION IN AMYLOIDOSIS IN A DEVELOPING COUNTRY. CLINICAL AND IMAGING MANIFESTATIONS.

DECOTTO, SANTIAGO; ET AL; MARIA LOURDES POSADAS MARTINEZ; DIEGO PEREZ DE ARENAZA; ELSA M NUCIFORA; RODOLFO PIZARRO

Congreso; 2024 ISA; 2024

Heart transplantation in amyloidosis in a developing country. Clinical and imaging manifestations.Santiago Decotto1, Eugenia Villanueva1, Diego Pérez de Arenaza1, Elsa M. Nucifora2, Ma Adela. Aguirre3-4 , Marcelina Carretero3, Ma Lourdes Posadas-Martínez3-4, César A. Belziti1, Rodolfo Pizarro1.1 Servicio de Cardiología, Hospital Italiano de Buenos Aires. Ciudad Autónoma de Buenos Aires, Argentina.2 Servicio de Hematología, Hospital Italiano de Buenos Aires. Ciudad Autónoma de Buenos Aires, Argentina.3 Servicio de Clínica Médica, Hospital Italiano de Buenos Aires. Ciudad Autónoma de Buenos Aires, Argentina.4 Instituto de medicina traslacional e ingeniería biomédica del Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina. CONICET.Background: Cardiac transplantation was not considered an option in amyloidosis patients in the past because it was considered a systemic disease with poor outcomes. Nowadays it is no longer a contraindication but still represents a medical challenge.Objective: To describe the clinical and imaging characteristics of patients who underwent heart transplantation due to amyloidosis, in a healthcare center in Argentina. Method: Descriptive case series of consecutive heart transplantation patients with amyloidosis. We included all patients with a diagnosis of amyloidosis cardiomyopathy who received a heart transplant in the period from November 2008 to February 2021.Results: A total of 16 patients were included. The mean age was 60 (± 10) years, 81% (n = 13) were male. According to the type of amyloidosis, 12 patients were transplanted for AL amyloidosis and 4 for ATTR amyloidosis (features in TABLE). The most frequent clinical presentations were left overload (50%) and cardiogenic shock (32%). The mean left ventricle 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. The heart transplantation gave 6 patients the chance to receive a bone marrow transplantation afterward. 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.