Hereditary bundle branch system defect. A new genetic entity? 1979

E Stéphan

A familial survey demonstrated mendelian inheritance in three large kindreds with conduction abnormalities and heart block. The trait was autosomal dominant, with varying expressivity and penetrance, apparent male preponderance, and congenital onset. Manifestations included right bundle branch block, left axis deviation, and right bundle branch block associated with left axis deviation. Complete heart block proved almost always to be a late event, and developed in all documented cases from bilateral bundle branch block. An r' pattern, most likely representing a right ventricular conduction delay, is discussed.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D007861 Lebanon A country located in the Middle East, bordering the Mediterranean Sea, between Israel and Syria. The capital is Beirut. Lebanese Republic
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010375 Pedigree The record of descent or ancestry, particularly of a particular condition or trait, indicating individual family members, their relationships, and their status with respect to the trait or condition. Family Tree,Genealogical Tree,Genealogic Tree,Genetic Identity,Identity, Genetic,Family Trees,Genealogic Trees,Genealogical Trees,Genetic Identities,Identities, Genetic,Tree, Family,Tree, Genealogic,Tree, Genealogical,Trees, Family,Trees, Genealogic,Trees, Genealogical
D010641 Phenotype The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment. Phenotypes
D002036 Bundle of His Small band of specialized CARDIAC MUSCLE fibers that originates in the ATRIOVENTRICULAR NODE and extends into the membranous part of the interventricular septum. The bundle of His, consisting of the left and the right bundle branches, conducts the electrical impulses to the HEART VENTRICLES in generation of MYOCARDIAL CONTRACTION. Atrioventricular Bundle,Anterior Fascicle,Kent-His Bundle,Left Bundle Branch of His,Posterior Fascicle,Right Bundle Branch of His,Atrioventricular Bundles,Bundle, Atrioventricular,Bundle, Kent-His,Bundles, Atrioventricular,Fascicle, Anterior,Fascicle, Posterior,His Bundle,Kent His Bundle
D002037 Bundle-Branch Block A form of heart block in which the electrical stimulation of HEART VENTRICLES is interrupted at either one of the branches of BUNDLE OF HIS thus preventing the simultaneous depolarization of the two ventricles. Fascicular Block,Anterior Fascicular Block,Bundle Branch Block,Left Bundle-Branch Block,Posterior Fascicular Block,Right Bundle-Branch Block,Anterior Fascicular Blocks,Block, Anterior Fascicular,Block, Bundle Branch,Block, Bundle-Branch,Block, Fascicular,Block, Left Bundle-Branch,Block, Posterior Fascicular,Block, Right Bundle-Branch,Blocks, Anterior Fascicular,Blocks, Bundle Branch,Blocks, Bundle-Branch,Blocks, Fascicular,Blocks, Left Bundle-Branch,Blocks, Posterior Fascicular,Blocks, Right Bundle-Branch,Branch Block, Bundle,Branch Blocks, Bundle,Bundle Branch Blocks,Bundle-Branch Block, Left,Bundle-Branch Block, Right,Bundle-Branch Blocks,Bundle-Branch Blocks, Left,Bundle-Branch Blocks, Right,Fascicular Block, Anterior,Fascicular Block, Posterior,Fascicular Blocks,Fascicular Blocks, Anterior,Fascicular Blocks, Posterior,Left Bundle Branch Block,Left Bundle-Branch Blocks,Posterior Fascicular Blocks,Right Bundle Branch Block,Right Bundle-Branch Blocks
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children

Related Publications

E Stéphan
April 2012, Journal of cardiovascular translational research,
E Stéphan
December 1979, Journal of medical genetics,
E Stéphan
September 1973, The American journal of cardiology,
E Stéphan
July 1972, The American journal of cardiology,
E Stéphan
January 2017, Annals of cardiac anaesthesia,
E Stéphan
June 1973, British heart journal,
Copied contents to your clipboard!