Hypertrophic cardiomyopathy (HCM) is the most prevalent genetic cardiomyopathy in children and young adults. Despite advances in understanding the molecular etiology of this disorder, the clinical phenotypes vary widely from asymptomatic septal hypertrophy, to frank congestive heart failure due to left ventricular outflow tract obstruction (LVOTO), to unexpected sudden cardiac death. Thus, isolating a specific genetic defect in this disease does little to predict the clinical consequence. This is best seen in large families with several people affected by HCM who all have the identical mutation but who may have very different clinical phenotypes. Evaluating individuals with HCM consists of establishing the diagnosis through cardiac imaging, noninvasively assessing the risk for sudden death, and medically or surgically treating symptomatic LVOTO when it is present.
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http://dx.doi.org/10.1007/s11936-008-0035-8 | DOI Listing |
Rev Cardiovasc Med
January 2025
Department of Cardiology, Chinese Academy of Sciences Sichuan Translational Medicine Research Hospital, 610072 Chengdu, Sichuan, China.
Background: There is a shortage of patients with hypertrophic cardiomyopathy (HCM) with concurrent coronary artery disease (CAD), and the influence of CAD on the prognosis of patients with HCM is uncertain. This real-world cohort study was conducted to evaluate the prognosis of patients with patients with CAD.
Methods: This cohort study of patients with HCM was conducted from May 2003 to September 2021.
World J Cardiol
January 2025
Institute of Cardiovascular Diseases & Department of Cardiology, Sichuan Provincial People's Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu 610072, Sichuan Province, China.
Hypertrophic cardiomyopathy (HCM) is an autosomal dominant inherited cardiomyopathy characterized by left ventricular hypertrophy. It is one of the chief causes of sudden cardiac death in younger people and athletes. Molecular-genetic studies have confirmed that the vast majority of HCM is caused by mutations in genes encoding sarcomere proteins.
View Article and Find Full Text PDFCirc Genom Precis Med
January 2025
Clalit Research Institute, Clalit Health Services, Ramat Gan, Israel (R.G., O.I., S.B.-S.).
Heart Rhythm
January 2025
Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, USA; Department of Molecular Pharmacology & Experimental Therapeutics (Windland Smith Rice Sudden Death Genomics Laboratory). Electronic address:
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