Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disease. The disease is characterized by marked variability in morphological expression and natural history, ranging from asymptomatic to heart failure or sudden cardiac death. Left ventricular hypertrophy and abnormal ventricular configuration result in dynamic left ventricular outflow obstruction in most patients. The goal of pharmacological therapy in HCM is to alleviate the symptoms, and it includes pharmacotherapies and septal reduction therapies. In this review, we summarize the relevant clinical issues and treatment options of HCM.
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http://dx.doi.org/10.1016/j.ijcha.2020.100503 | DOI Listing |
J Am Soc Echocardiogr
March 2025
Division of Cardiology, The Children's Hospital of Philadelphia, Children's Hospital of Philadelphia, The Perelman School of Medicine at The University of Pennsylvania, Philadelphia, PA.
Eur Heart J Cardiovasc Imaging
March 2025
Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Background: Myocardial work assessment has emerged as a promising tool for left ventricular (LV) performance evaluation. Existing non-invasive methods for assessing it rely on assumptions on LV pressure and geometry. Recently, shear wave elastography allowed to quantify changes in myocardial stiffness throughout the cardiac cycle.
View Article and Find Full Text PDFFront Cardiovasc Med
February 2025
Department of Cardiology, Shandong Provincial Medical and Health Laboratory of Cardiac Electrophysiology and Arrhythmia, The First Affiliated Hospital of Shandong First Medical University & Shandong Provincial Qianfoshan Hospital, Jinan, China.
Background: Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disorder characterized by asymmetric hypertrophy of the ventricles and the ventricular septum, leading to subsequent left ventricular outflow tract (LVOT) obstruction and diastolic dysfunction. Typically, patients with HCM experience sinus tachycardia and sinus arrest relatively infrequently. In addition, the concurrent occurrence of HCM with non-surgically induced (ablation or myectomy) bradyarrhythmia and idiopathic pericardial effusion in adult patients has not been previously reported.
View Article and Find Full Text PDFFront Cardiovasc Med
February 2025
Department of Ultrasound, The People's Hospital of China Medical University, The People's Hospital of Liaoning Province, Shenyang, China.
Introduction: Mutations in the sarcomere protein, particularly in cardiac myosin binding protein C gene (), were the most frequent genetic cause of hypertrophic cardiomyopathy (HCM). The pathogenic MyBPC3 P459fs mutation has been reported in HCM patients. However, there was limited knowledge of the structure-function relationships and potential pathways in clinical HCM with MyBPC3 P459fs mutation.
View Article and Find Full Text PDFEur Heart J Case Rep
March 2025
Xijing Hypertrophic Cardiomyopathy Center, Department of Ultrasound, Xijing Hospital, Fourth Military Medical University, 127 Changle W Rd, Xi'an, Shaanxi 710032, China.
Background: Systolic anterior motion (SAM) of the mitral valve is a hallmark feature of hypertrophic obstructive cardiomyopathy (HOCM) and a primary cause of dynamic left ventricular outflow tract obstruction. This case report highlights an unusual presentation of SAM associated with acute haemodynamic collapse.
Case Summary: A 36-year-old male with HOCM presented with recurrent episodes of syncope.
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