Symbol
| PCCD1
|
Location
| 3p22.2
|
Name
|
progressive cardiac conduction defect 1 |
Other name(s)
|
progressive familial heart block, type I
Lenegre-Lev disease |
Corresponding gene
|
SCN5A
|
Other symbol(s)
| PFHB1
|
Main clinical features
|
progressive alteration of cardiac conduction through the His-Purkinje system with right or left bundle branch block and large QRS complexes, leading to complete atrioventricular block and causing syncope and sudden death |
Genetic determination
| autosomal dominant |
Related entries
| including the sudden unexplained nocturnal death syndrome (SUNDS)
|
Function/system disorder
| cardiovascular |
Type
| disease
|