Hypertrophic cardiomyopathy (HCM) is a dominant genetic disorder of the myocardium associated with dysfunctional contractile proteins. The major risk of HCM is sudden cardiac death, which may occur even in asymptomatic carriers. Causes are highly heterogeneous. Over 140 different mutations in nine sarcomeric genes have been described to date. The majority of cases (80% or more) may eventually b...

Background The presence of myocardial fibrosis is associated with worse clinical outcome in hypertrophic cardiomyopathy (HCM) patients. Due to the substantial variations in left ventricular (LV) wall thickness and fibrosis in HCM, volumetric coverage of entire LV myocardium is essential for the accurate assessment of myocardial fibrosis. Slice-interleaved T1 (STONE) mapping sequence allows for ...

Sarcomeric hypertrophic cardiomyopathy (HCM) is the most common genetic cause of unexplained left ventricular hypertrophy and has no specific treatment. Anderson-Fabry disease (AFD) is rare and usually multisystemic, but occasionally expresses clinically as a predominantly cardiac phenotype mimicking HCM. We describe an illustrative case of a patient followed regularly for 25 years with a diagn...

Background Abnormal myocardial T2 signal has been described in a minority of patients with hypertrophic cardiomyopathy (HCM). This phenomenon has been associated with arrhythmic outcomes and, given its more restrictive prevalence versus late gadolinium enhancement (LGE), offers a potentially useful biomarker for risk stratification. To date, postulated mechanisms include active tissue disease o...

A seven-year-old castrated male domestic shorthair cat was diagnosed with hypertrophic cardiomyopathy (HCM) and suspected mitral stenosis (MS) based on electrocardiography, thoracic radiographs and echocardiographic findings. Post-mortem examination of the heart revealed morphological features consistent with HCM. In addition, there was marked fibrous deposition on the surfaces of the chordae t...

Hypertrophic cardiomyopathy (HCM) is an inherited disease of heart muscle that can be caused by mutations in sarcomere proteins. Clinical diagnosis depends on an abnormal thickening of the heart, but the earliest signs of disease are hyperdynamic contraction and impaired relaxation. Whereas some in vitro studies of power generation by mutant and wild-type sarcomere proteins are consistent with ...

Abstract Background Hypertrophic cardiomyopathy (HCM), being characterized by enhanced contractility, positively responses to treatment with myosin inhibitors which could promote reverse remodelling. Additionally, speckle tracking analysis highlights left ventricular (LV) contractile alterations in HCM, although the pathophysiological meaning and effect of target-therapy are still unclear. Aim ...

Right ventricular (RV) hypertrophy is common in patients with hypertrophic cardiomyopathy (HCM), and is associated with more severe disease. Conventional surgical strategies such as the traditional Morrow procedure pose a particularly high risk to patients with severe hypertrophy and RV obstruction, for whom the most appropriate therapeutic approach has not yet been established. We have propose...

Objective: Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disorder caused by mutations in sarcomeric proteins and characterized hypertrophy of the heart muscle. 
 Materials Methods: In present study, 21 patients with HCM some their parents were evaluated via next-generation sequencing (NGS) using a targeted panel 17 genes. Results: Pathogenic or likely pathogenic variants detec...

Atrial fibrillation (AF) may appear during the natural clinical course of hypertrophic cardiomyopathy (HCM). It is regarded as a complication of HCM and is a marker of advanced disease. AF is more likely to occur in untreated, obstructive HCM. Unfortunately, this represents a turning point that puts patients at increased risk of further disability, stroke and death. The presence of obstruction ...