• Rev Med Interne · Sep 2022

    Review

    [Cardiac amyloidosis: State of art in 2022].

    • S Oghina, M A Delbarre, E Poullot, K Belhadj, P Fanen, and T Damy.
    • Service de cardiologie, Centre Hospitalier Universitaire Henri-Mondor, AP-HP (Assistance Publique-Hôpitaux de Paris), 1, rue Gustave-Eiffel, 94010 Créteil, France; Centre de référence national des amyloses cardiaques et réseau amylose Mondor, Filière Cardiogen, Centre Hospitalier Universitaire Henri-Mondor, AP-HP (Assistance Publique-Hôpitaux de Paris), 1, rue Gustave-Eiffel, 94010 Créteil, France. Electronic address: silvia.oghina@aphp.fr.
    • Rev Med Interne. 2022 Sep 1; 43 (9): 537-544.

    AbstractThe 3 main types of cardiac amyloidosis are linked to two protein precursors: AL amyloidosis secondary to free light chain deposits in the context of monoclonal gammopathy (mainly of undetermined significance or myeloma) and transthyretin amyloidosis (ATTR), comprising wild-type transthyretin amyloidosis (ATTRwt for wild type) and hereditary transthyretin amyloidosis (ATTRv for variant). These diseases are underdiagnosed and highly prevalent in common cardiac phenotypes in recent studies (heart failure with preserved ejection fraction, severe aortic stenosis, hypertrophic cardiomyopathy). Myocardial amyloid infiltration affects all cardiac structures and clinically promotes predominantly heart failure, conductive disorders and cardioembolic events. The search for extracardiac signs makes it possible to arouse diagnostic suspicion. Electrocardiogram, echocardiography and cardiac MRI can suspect cardiac amyloidosis. The diagnostic confirmation follows a simple algorithm including a systematic search for monoclonal gammapathy and a disphosphonate scintigraphy. Histological proof is necessary in case of AL or ATTR amyloidosis with concomitant monoclonal gammopathy in order to initiate specific treatment. Due to the late disease onset in ATTRv, genetic testing must be routine in all cases of ATTR. These diseases are no longer perceived as incurable since recent therapeutic innovations. A better knowledge of the disease is more than ever necessary.Copyright © 2022 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

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