Lenegr's disease

Description:

The syndrome (disease) of Lenegr is primary (idiopathic) not ischemic degenerative (fibrosis or a degeneration) bilateral defeat of branches of a ventriculonector without involvement in process of a myocardium and a fibrous skeleton of heart (there are no authentic data on prevalence of a syndrome of Lenegr).

Lenegr etiologies:

The damaged fibers of branches of a ventriculonector long time can keep delayed conduction. Over time, however, extent of blockade accrues and full AV-blockade develops. An electrophysiologic basis of a disease is the disturbances in cells of the carrying-out system of heart caused by a mutation of a gene of SCN5A coding alpha subunit of potentsialzavisimy natrium channels (Nav1.5) and their modulators. As a rule, the specified pathological processes it is shown by a combination of a total block of the right leg and an anterosuperior branching of the left leg of a ventriculonector.

Symptoms of a disease of Lenegr:

Classification of a syndrome of Lenegr:

1. first type: it is shown by a total block of the right leg of a ventriculonector, a front branch of the left leg; clinically - at development of full AV-blockade there are preunconscious states and faints or a sudden cardiac death;
2. second type: it is shown by a sinus bradycardia and blockade of a back branch of the left leg of a ventriculonector.  

Clinical symptoms of a disease of Lenegr (in a disease debut) can be scanty and not specific. At emergence of AV-blockade of high degree in patients feelings of interruptions in cardiac performance, weakness, fatigue, dizzinesses, preunconscious conditions and faints, symptoms of heart failure are observed. They often listen so-called gun tone and bifurcation of cardiac sounds. Can lead the progressing disturbance of conductivity at Lenegr's syndrome to emergence of such life-threatening states as ventricular that hi-and bradyarrhythmias, fibrillation of ventricles and an asystolia. These arrhythmias and blockade are the main reasons of a sudden cardiac death of such patients.

Diagnosis:

The main diagnostic method is the standard ECG which reveals proximal or distal AB-blockade of various degree. The only laboratory diagnostic method of a disease is the molecular and genetic research for identification of a mutation in SCN5A gene.

Treatment of a disease of Lenegr:

The unique method of treatment - implantation EX-. As in a pathogeny of a syndrome of Lenegr the leading role is played by mutations in the gene coding natrium channels perspective methods of gene therapy which are developed now are considered.