Unequal allelic expression of wild-type and mutated β-myosin in familial hypertrophic cardiomyopathy.

Familial hypertrophic cardiomyopathy (FHC) is an autosomal dominant disease, which in about 30% of the patients is caused by missense mutations in one allele of the β-myosin heavy chain (β-MHC) gene (MYH7). To address potential molecular mechanisms underlying the family-specific prognosis, we determined the relative expression of mutant versus wild-type MYH7-mRNA. We found a hitherto unknown mutation-dependent unequal expression of mutant to wild-type MYH7-mRNA, which is paralleled by similar unequal expression of β-MHC at the protein level.

Endocardial radiofrequency ablation for hypertrophic obstructive cardiomyopathy: acute results and 6 months' follow-up in 19 patients.

Objectives: The purpose of this study was to examine the efficacy and safety of endocardial radiofrequency ablation of septal hypertrophy (ERASH) for left ventricular outflow tract (LVOT) gradient reduction in hypertrophic obstructive cardiomyopathy (HOCM).

Background: Anatomic variability of the vessels supplying the obstructing septal bulge can limit the efficacy of transcoronary ablation of septal hypertrophy in HOCM. Previous studies showed that inducing a local contraction disorder without reducing septal mass results in effective gradient reduction.

The left and right ventricle of a patient with a R723G mutation of the beta-myosin heavy chain and severe hypertrophic cardiomyopathy show no differences in the expression of myosin mRNA.

Background: In familial hypertrophic cardiomyopathy (FHC), asymmetric left ventricular (LV) hypertrophy has been considered to be the predominant phenotypic expression, whereas right ventricular (RV) involvement is still ambiguous. In most cases, the right ventricle remains unaffected until secondary pulmonary hypertension develops. Several FHC-causing mutations of genes encoding sarcomere-related proteins have been identified which are transmitted in an autosomal-dominant manner.

Predictors of complete heart block after transcoronary ablation of septal hypertrophy: results of a prospective electrophysiological investigation in 172 patients with hypertrophic obstructive cardiomyopathy.

Objectives: This study analyzed changes in intracardiac conduction during transcoronary ablation of septal hypertrophy (TASH) to identify predictors for pacemaker dependency after TASH.

Background: Transcoronary ablation of septal hypertrophy is an accepted therapeutic option in hypertrophic obstructive cardiomyopathy (HOCM). However, atrioventricular conduction disorders, requiring permanent pacemaker implantation, remain a major adverse effect.