Background Hypertrophic cardiomyopathy (HCM) results in myocardial disarray, hypertrophy and fibrosis. Late gadolinium enhanced MRI (LGE) can assess the presence and extent of fibrosis, which is associated with the development of arrhythmias and sudden cardiac death. However, enhancement may not always be present or only sparsely distributed. Thus, one of the challenges is how best to describe ...

D ynamic obstruction to left ventricular (LV) outflow as a result of mitral valve systolic anterior motion is a potentially deleterious facet of hypertrophic cardiomyopathy (HCM).1-4 In many patients, outflow obstruction is largely responsible for disabling symptoms of heart failure such as exertional dyspnea (often with chest pain), fatigue, and orthopnea.ls3 Consequently, treatment interventi...

Background Hypertrophic cardiomyopathy (HCM) is associated with increased myocardial fibrosis and collagen deposition. Collagen reduces cardiovascular magnetic resonance (CMR) derived T2 times, however, the inflammatory milieu required for its deposition is often associated with localised oedema. Focal areas of high T2 signal intensity have been reported in association with areas of late gadoli...

BACKGROUND Mutations in the gene that encode cardiac troponin T (cTnT) account for approximately 15% of cases of familial hypertrophic cardiomyopathy (HCM). These mutations are associated with a particularly severe form of HCM characterized by a high incidence of sudden death and a poor overall prognosis, despite subclinical or mild left ventricular hypertrophy. METHODS AND RESULTS We evaluat...

Some studies have reported that angiotensin converting enzyme (ACE) and angiotensinogen (AGT) genes have been associated with hypertrophic cardiomyopathy (HCM). However, there have been inconsonant results among different studies. To clarify the influence of ACE and AGT on HCM, a systemic review and meta-analysis of case-control studies were performed. The following databases were searched to i...

BACKGROUND Data on the outcomes of hypertrophic cardiomyopathy (HCM) with biventricular obstruction are limited. OBJECTIVE Our aim is to compare mid-term outcomes of biventricular outflow tract obstruction (BVOTO) HCM, left ventricular outflow tract obstruction (LVOTO) HCM and nonobstructive hypertrophic cardiomyopathy (NO-HCM) in children and adolescents who were treated with standard medica...

Hypertrophic cardiomyopathy (HCM) is a common heart disease with a prevalence of 1 in 500 in the general population. Several mutations in genes encoding cardiac proteins have been found in HCM patients, but these changes do not predict occurrence or prognosis and the molecular mechanisms underlying HCM remain largely elusive. Here we show that cardiac expression of vacuolar protein sorting 34 (...

AIMS Mutations in the cardiac myosin-binding protein C gene (MYBPC3) are the most common genetic cause of hypertrophic cardiomyopathy (HCM) worldwide. The molecular mechanisms leading to HCM are poorly understood. We investigated the metabolic profiles of mutation carriers with the HCM-causing MYBPC3-Q1061X mutation with and without left ventricular hypertrophy (LVH) and non-affected relatives,...

BACKGROUND In hypertrophic cardiomyopathy (HCM), myocardial abnormalities are commonly heterogeneous. Two patterns of late gadolinium enhancement (LGE) have been reported: a bright "confluent" and an intermediate intensity abnormality termed "diffuse," each representing different degrees of myocardial scarring. We used MRI to study the relation between intramural cardiac function and the extent...