Progressive Familial Heart Block, Type Ia (PFHB1A)

Progressive Familial Heart Block, Type Ia(来自ICD-11)

别称:

Bundle Branch Block

Hereditary Bundle Branch System Defect

Heart Block, Nonprogressive

Progressive Familial Heart Block Type Ia

Lenegre-Lev Disease

Pfhb1a

Pfhbia

Heart Block, Progressive Familial, Type I

Progressive Familial Heart Block, Type 1a

Cardiac Conduction Defect, Progressive

Pccd

Hbbd

Heart Block, Progressive, Familial, Type 1a

Progressive Familial Heart Block Type I

Progressive Cardiac Conduction Defect

Progressive Familial Heart Block 1a

Heart Block, Progressive, Type Ia

Conduction Disorder of the Heart

Cardiac Conduction Defect

Bundle-Branch Block

Pfhbi

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Basic Information

Medical Symptom

Gene & Mutation

Related Drugs

Disease Model

References Literature

Progressive Familial Heart Block, Type Ia, also known as bundle branch block, is related to brugada syndrome 1 and ventricular fibrillation, paroxysmal familial, 1, and has symptoms including chest pain, dyspnea and syncopal episode. An important gene associated with Progressive Familial Heart Block, Type Ia is SCN5A (Sodium Voltage-Gated Channel Alpha Subunit 5), and among its related pathways/superpathways are Nervous system development and Activation of cAMP-Dependent PKA. The drugs Prednisolone and Prednisolone acetate have been mentioned in the context of this disorder. Affiliated tissues include heart and atrioventricular node, and related phenotypes are prolonged pr interval and right bundle branch block

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