Prevalence of Systolic Impairment in an Unselected Regional Population With Hypertrophic Cardiomyopathy

Chronotropic incompetence and its relation to exercise intolerance in hypertrophic cardiomyopathy

“…In fact, HCM represents a model of diastolic dysfunction with a clear component of impairment of myocardial systolic function [18]. Less than 10% of patients with HCM may transit to a phase characterized by systolic dysfunction and left ventricular dilation, resembling dilated cardiomyopathy [19]. Furthermore, a high prevalence of abnormal sinus-node function (66%) and His-Purkinje conduction (30%) was noted in a cohort of 155 HCM patients who underwent electrophysiologic studies, which may be explained by myocardial disarray and distinct fibrotic changes in the atrial myocardium [20].…”

Long-Term Survival Following Cardiac Arrest Without Implantable Defibrillator Protection in a Hypertrophic Cardiomyopathy Patient

“…Progression to DCM has been reported up to 18% in patients with ‘cardiac myosin binding protein C’ mutation [10]. A study with 248 HCM patients reported that half of the patients who have progressed to DCM had family history of HCM and 25% of them had family history of SCD [11]. No difference was detected in aspect of the cardiac mortality among the patients with and without left ventricular dilation in this study.…”

Evolving Anatomic, Functional, and Molecular Imaging in the Early Detection and Prognosis of Hypertrophic Cardiomyopathy

“…Although left ventricular global systolic dysfunction is a relatively uncommon occurrence (3-8%) in HCM, a majority of affected patients do develop diastolic dysfunction [6]. Myocyte disarray, interstitial fibrosis, left ventricular hypertrophy, alterations in Ca 2+ homeostasis, and myocardial ischemia all contribute to the development of HCM-related diastolic dysfunction [11], which may be detected before the development of LVH.…”

“…Echocardiography has served as the cornerstone of diagnosis and risk assessment HCM; newer techniques such as tissue Doppler imaging (TDI) which measures the low-frequency, high-amplitude signals of myocardial tissue motion, and speckle tracking imaging (STI), which quantifies myocardial torsion, have been evaluated in the assessment of ventricular function and the pre-clinical diagnosis of HCM. These techniques have also been explored as a means of differentiating HCM from other causes of hypertrophy [4][5][6][7][8]. Myocardial perfusion imaging and cardiac magnetic resonance imaging (CMR) have provided insight into the pathogenesis of HCM-related myocardial dysfunction.…”