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  • The Natural History Of Amyl...
    Jhawar, Nikita; Schneider, Artur; Hodge, David; El Sabbagh, Abdallah; Grogan, Martha; Nkomo, Vuyisile; Dispenzieri, Angela; Rosenthal, Julie; Nicolau, Jose Nativi; Leoni-Moreno, Juan Carlos; Sher, Taimur; Lyle, Melissa

    Journal of cardiac failure, January 2024, 2024-01-00, Letnik: 30, Številka: 1
    Journal Article

    Cardiac amyloidosis (CA) is characterized by the deposition of insoluble amyloid fibrils in the extracellular space of the heart. Infiltration of amyloid into the heart valves can compromise their integrity and predispose to regurgitation. Although mitral valve (MV) involvement in CA has been described in case reports, the natural history of mitral regurgitation (MR) in CA remains under studied. The study cohort included patients with functional moderate, moderate-to-severe, or severe MR and CA evaluated within the Mayo Clinic enterprise between 2012 to 2022. Demographic information, type of amyloidosis, diagnostic data, imaging results, mortality outcomes, and procedural data were retrospectively extracted from medical charts. Lastly, a Kaplan-Meier approach was taken to generate a product-limit failure curve. 72 patients with cardiac amyloidosis and moderate or more severe MR were identified. The average age of the subjects was 69.9 ±8.04 years. Table 1 depicts the baseline demographics, comorbidities, MR severity, therapies, and mean echocardiographic parameters of the cohort. Of the 72 subjects, 4.17% underwent transcatheter edge-to-edge repair (TEER) of the MV, 2.82% had MV replacement, and 1.41% had MV repair. Stroke volume index (SVI) and TAPSE were found to be predictors of mortality (p<0.005, Table 2). Five-year mortality of our cohort was 48% (Figure 1). As amyloidosis-specific pharmacotherapies continue to advance, ongoing studies to determine mortality risk will be crucial. We demonstrate a five-year mortality of 48% associated with moderate, moderate-to-severe, or severe MR, with SVI and TAPSE being significant prognostic indicators. The minority of patients underwent interventional mitral valve procedures. Further investigation is needed to determine the role of MV interventions in CA patients.