ABSTRACT
A 42-year-old woman was referred to our Cardiac Intensive Care Unit for possible acute coronary syndrome (acute heart failure and elevated serum cardiac troponin levels). Urgent coronary angiogram was unremarkable. Transthoracic echocardiography revealed severe concentric biventricular hypertrophy, systolic dysfunction (LVEF 26%, FAC 20%), and restrictive physiology (E/E' 27). LV strain analysis showed an apical sparing pattern with severely reduced GLS (-6%) and raised the suspicion of cardiac amyloidosis (CA). The endomyocardial biopsy established the diagnosis of lightchains CA. The patient's prognosis was very poor at the diagnosis, with a median survival of 4 months based on Mayo Clinic's revised staging system. Combination chemotherapy with CyBorD scheme (Cyclofosfamide/Bortezomib/Dexamethasone) was promptly started, but prematurely stopped because of the development of rapidly progressive biventricular failure. Therefore, the patient received a total artificial heart (TAH) as a bridge-to-candidacy to orthotopic heart transplantation (OHT). The CyBorD therapy was then restarted, and complete haematological remission was achieved six months later. Therefore, the patient underwent effective monoclonal antibody therapy for nosocomial SARS-CoV-2 infection. Subsequently, the patient was placed on the urgent transplant list because of the bacterial device's driveline infection. Two months later, she underwent OHT. The patients died three days for multiple reasons: difficult TAH explant with prolonge extracorporeal circulation time, the necessity of central V-A ECMO, graft failure.