PMID: 107884Jan 1, 1979

Hereditary conduction defect in the His system: a dominant autosome disease

Archives des maladies du coeur et des vaisseaux
M Stéphan


An oriental family with over 265 living descendants of a polygamous ancestor known to have had a slow pulse and syncopes was studied. Many examples of conduction defects, mainly right bundle branch block, left anterior hemiblock, bifascicular block and artioventricular block were detected. The transmission of the genetic defect was autosomal dominant, the incidence 43%, the penetration very variable, apparently affecting the male descendants predominantly, the expression probably congenital, but with complete heart block presenting at 75 years of age in one case. The localisation of the conduction defect is discussed: the distal extremity of the His bundle after the origin of part or all of the left posterior fascicle. This study, in its clinical and epidemiological context, demonstrates an inherited disease with both typical and "forme fruste" expressions. Compared with other series, it suggests the diverse nature of hereditary and familial conduction defects.

Related Concepts

Electrocardiographic Recorders
Genetic Conditions, Dominant
Heart Block

Related Feeds


Bradyarrhythmias are slow heart rates. Symptoms may include syncope, dizziness, fatigure, shortness of breath, and chest pains. Find the latest research on bradyarrhythmias here.

Cardiac Conduction System

The cardiac conduction system is a specialized tract of myocardial cells responsible for maintaining normal cardiac rhythm. Discover the latest research on the cardiac conduction system here.

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