Comments on the 2023 guidelines of the ESC on management of cardiomyopathies

KARDIOLOGIE(2024)

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摘要
The European Society of Cardiology (ESC) presented new guidelines for the broad clinical field of cardiomyopathies, incorporating parts from previous guidelines while updating and replacing others. The guidelines emphasize a phenotypic approach for the classification of cardiomyopathies, incorporating clinical variables, morphological and functional characteristics and genetic information to enable a phenotype-based, etiologically informed diagnosis. It advocates a multimodal diagnostic approach involving patient history, family history, laboratory tests, electrocardiogram, imaging, and genetic examinations. Emphasis is placed on risk stratification utilizing validated risk calculators and integrating clinical variables and genotype to determine the actual risk. The importance of echocardiography and cardiac magnetic resonance imaging (MRI) for diagnosis and monitoring is underlined, with advancements in cardiac MRI providing detailed tissue characterization crucial for etiological assignment and prognosis determination. The role of genetic testing is emphasized, not only for confirmation of the diagnosis but also for effective family screening. Treatment recommendations include the incorporation of novel treatments, such as mavacamten for obstructive hypertrophic cardiomyopathy (oHCM). Furthermore, the guidelines address the evolving understanding of cardiomyopathy forms, such as nondilated left ventricular cardiomyopathy (NDLVC) and underlines the importance of integrated, multidisciplinary care.
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关键词
Cardiac MRI,Genetic diagnostics,Mavacamten,NDLVC,Risk calculator
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