Familial isolated arrhythmogenic right ventricular dysplasia

Orpha code: 217656OMIM code: 604401

Definition

Familial isolated arrhythmogenic right ventricular dysplasia (ARVC) is the familial autosomal dominant form of ARVC (see this term), a heart muscle disease characterized by life-threatening ventricular arrhythmias with left bundle branch block configuration that may manifest with palpitations, ventricular tachycardia, syncope and sudden fatal attacks, and that is due to dystrophy and fibro-fatty replacement of the right ventricular myocardium that may lead to right ventricular aneurysms.

Disease data
Classification

Disease

Synonyms
Familial isolated ARVC
Rodzinna izolowana ARVC
Rodzinna izolowana ARVD
Rodzinna izolowana arytomogenna dysplazja komorowa
Rodzinna izolowana arytomogenna kardiomiopatia komorowa
Rodzinna izolowana arytomogenna kardiomiopatia prawej komory
Familial isolated ARVD
Familial isolated arrhythmogenic right ventricular cardiomyopathy
Familial isolated arrhythmogenic ventricular cardiomyopathy
Familial isolated arrhythmogenic ventricular dysplasia
ORPHA code
217656
OMIM code
604401
ICD10 code
I42.8
ICD11 code
BC43.6

No additional description.

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