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BC43.6 Arrhythmogenic ventricular cardiomyopathy

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

Arrhythmogenic ventricular cardiomyopathy is a cardiomyopathy characterised by myocardial cell loss with partial or total replacement of right ventricular muscle by adipose and fibrous tissue, beginning subepicardially to become transmural in time, sparing the papillary muscles and trabeculae, and often associated with aneurysms particularly of the right ventricular outflow tract. There is progressive systolic impairment with ventricular dilation and marked propensity for ventricular arrhythmias of right, as well as left, ventricular origin. Classically a disease of the right ventricle, more recent evidence suggests left ventricular involvement to a varying extent in up to 75% of cases, as well as isolated left ventricular disease.

postcoordination

synonyms

• Arrhythmogenic ventricular cardiomyopathy
• arrhythmogenic ventricular dysplasia
• Arrhythmogenic ventricular cardiomyopathy due to desmosomal mutation
• Arrhythmogenic ventricular cardiomyopathy with right ventricular dominance
• arrhythmogenic right ventricular dysplasia
• arrhythmogenic right ventricular cardiomyopathy
• ARVC - [Arrhythmogenic right ventricular cardiomyopathy]
• ARVD - [Arrhythmogenic right ventricular dysplasia]
• Familial isolated arrhythmogenic right ventricular dysplasia
• Naxos disease
• Keratosis palmoplantaris with arrhythmogenic cardiomyopathy
• Arrhythmogenic ventricular cardiomyopathy with left ventricular dominance
• arrhythmogenic left ventricular dysplasia
• arrhythmogenic left ventricular cardiomyopathy
• Woolly hair – palmoplantar keratoderma – dilated cardiomyopathy
• Carvajal-Huerta syndrome
• Carvajal syndrome

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