The mechanical and perfusion basis of exercise limitation in apical hypertrophic cardiomyopathy

Patients with apical hypertrophic cardiomyopathy (ApHCM) commonly suffer symptoms of chest pain and dyspnea. Apical hypertrophy causes cavity obliteration early in systole, persisting into diastole; generating high pressures and creating basal to apical heterogeny in myocardial deformation across the cardiac cycle.1 Conventional measures of systolic function (eg, ejection fraction [EF]) may be supranormal, but other parameters may be abnormal either globally (global longitudinal strain [GLS]),2 or apically (longitudinal, radial and circumferential strain, including twist).3 Cardiac magnetic resonance (CMR) demonstrated that apical perfusion defects are a universal feature across the phenotypic spectrum.4 Reduced exercise capacity in HCM is widely reported; however, in ApHCM, functional limitation has been little explored, and the underpinning roles for abnormal myocardial mechanics and perfusion abnormalities are unknown. We hypothesized that patients with ApHCM would have functional limitation associated with abnormalities of global/regional myocardial mechanics (strain, twist) and myocardial blood flow (MBF).

Palavras-chave

Apical hypertrophic cardiomyopathy Cardiac magnetic resonance imaging Cardiopulmonary exercise testing Exercise echocardiography Multimodality imaging Transthoracic echocardiography

URI

http://hdl.handle.net/10400.1/27389

Editora

Elsevier

DOI

10.1016/j.jacadv.2024.101555

Coleções

FCB2-Artigos (em revistas ou actas indexadas)

Licença CC

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