Left ventricular noncompaction/hypertrabeculation is a condition which is characterized by a highly trabeculated, "spongy" myocardium.It can present at any age with heart failure, arrhythmia and/or thromboembolic events.A wide variety of mutations have been found to be a cause of hypertrabeculation and it is possible that there is a continuum of hypertrophic cardiomyopathy, dilated cardiomyopat...

Genotype-phenotype correlations provide another perspective in studies seeking to identify the factors that underlie the clinical variability that is a feature of several inherited diseases. This approach has been particularly revealing in investigations into the molecular causes and phenotypic heterogeneity associated with hypertrophic cardiomyopathy (HCM), a common inherited primary cardiac d...

Recent meta-analyses have shown good long-term survival of patients with hypertrophic obstructive cardiomyopathy (HOCM) after alcohol septal ablation (ASA) comparable with surgical myectomy. However, gradient reduction after ASA was slightly less favourable [1, 2] and in a singlecentre study a higher rate of aborted sudden cardiac death was found [3]. The following case report illustrates the i...

A 6-year-old, 3.0 kg, neutered female, Yorkshire terrier was referred for orthopedic surgery. Cardiac arrest followed unsuccessful treatment of bradycardia and systemic arterial hypotension under general anesthesia. Postmortem examination revealed hypertrophic cardiomyopathy. A possible relationship between treatment of bradycardia, systemic arterial hypotension, and sudden cardiac death is des...

BACKGROUND Disopyramide is effective in ameliorating symptoms in patients with hypertrophic cardiomyopathy; however, its potential for proarrhythmic effect has raised concerns about its use in the ambulatory setting. The risk of initiating disopyramide in this manner has never been evaluated. METHODS AND RESULTS All charts of patients seen in the outpatient hypertrophic cardiomyopathy clinic ...

Familial hypertrophic cardiomyopathies (FHC) are the most common genetic heart diseases in the United States, affecting nearly 1 in 500 people. Manifesting as increased cardiac wall thickness, this autosomal dominant disease goes mainly unnoticed as most affected individuals are asymptomatic. Up to 1-2% of children and adolescents and 0.5-1% adults with FHC die of sudden cardiac death, making i...

ith great interest we read the review by Corrado et al. (1) on re-participation screening (PPS) of young competitive athletes to revent sudden cardiac death (SCD). It is important that Corrado et al. (1) briefly mentioned the ossibility of cascade screening of relatives of a competitive athlete ith an inherited heart disease. Unfortunately, this topic is requently neglected in articles on PPS. ...

mon inherited cardiomyopathy, occurring in about 1 in 500 individuals. The first gene mutation for this condition was identified in a large French Canadian family cohort in 1989. Clinical presentation typically includes left ventricular hypertrophy in the absence of abnormal loading conditions, such as hypertension or aortic stenosis. Hypertrophic cardiomyopathy has come to public recognition i...

BACKGROUND The aim of the study was to clarify the relationship between genotype status and major cardiovascular outcomes in a large cohort of patients with hypertrophic cardiomyopathy. METHODS AND RESULTS Genetic testing was performed in 558 consecutive proband patients with hypertrophic cardiomyopathy. Baseline and follow-up (mean follow-up 6.3 years) clinical and echocardiographic data wer...