[Clinical polymorphisms and approaches of arrhythmias treatment in a family with δKPQ1505-1507 deletion in SCN5A gene].

Background: The aim of the study was to analyze spectrum of manifestation and treatment response in large family with rhythm disturbances caused by p.delKPQ1505-1507 mutation in SCN5A gene.

Patients And Methods: We had under our observation 18 members of large Iranian family with various combination of inherited arrhythmic syndromes.

[Dilated cardiomyopathy caused by p.E446K mutation in SCN5A gene].

Dilated cardiomyopathy (DCM) is myocardial disorder characterized by progressive heart chambers enlargement and impairment of myocardial contractility. This disorder is the most common cause of advanced heart failure requiring the heart transplantation. The prevalence of the disease is 36.

[Clinic and genetic polymorphism of Brugada syndrome in Russian patients, caused by mutation in SCN5A gene].

Brugada syndrome (BrS) is an inherited cardiac arrhythmic disorder, characterized by ST-segment elevation in right precordial leads V1-V2>2 mm, pseudo right bundle branch block (RBBB), T-wave inversion and an increased risk of cardiac sudden death (SCD) due to molymorphic VT. It is estimated to be responsible for 12% of SCD cases and about 20% of deaths in patients with structurally normal hearts in autopsy. Mutations in the SCN5A gene account 15-30% of all cases.

[Possibilities myocardial biopsy in the diagnosis of myocarditis verification in patients with idiopathic arrhythmias].

Unlabelled: Aim of the study was to elucidate nosological nature of "idiopathic" arrhythmias by means of right ventricular endomyocardial biopsy (EMB) and to assess effect of etiotropic and pathogenetic treatment.

Material And Methods: We included into this study 19 patients (mean age 42.6 +/-11.