Back خلل تنسج البطين الأيمن المحدث لاضطراب النظم Arabic Aritmogena desnokomorska displazija BS Arrhythmogene rechtsventrikuläre Kardiomyopathie German Αρρυθμιογόνος καρδιομυοπάθεια Greek Displasia arritmogénica Spanish Dysplasie ventriculaire droite arythmogène French Displasia ventricolare destra aritmogena Italian Arytmogenna kardiomiopatia prawej komory Polish Аритмогенная дисплазия правого желудочка Russian Aritmogena kardiomiopatija Serbian
 | This article needs additional citations for verification. (February 2020) |
| Arrhythmogenic cardiomyopathy | |
|---|---|
| Other names | Arrhythmogenic right ventricular cardiomyopathy (ARVC), arrhythmogenic right ventricular dysplasia (ARVD) |
 | |
| Typical micro-histologic features of ARVC/D. Ongoing myocyte death (upper) with early fibrosis and adipocyte infiltration (lower). | |
| Specialty | Cardiology |
Arrhythmogenic cardiomyopathy (ACM) is an inherited heart disease.[1]
ACM is caused by genetic defects of parts of the cardiac muscle known as desmosomes, areas on the surface of muscle cells which link them together. The desmosomes are composed of several proteins, and many of those proteins can have harmful mutations.
ARVC can also develop in intense endurance athletes in the absence of desmosomal abnormalities. Exercise-induced ARVC is possibly a result of excessive right ventricular wall stress during high intensity exercise.[2][3]
The disease is a type of non-ischemic cardiomyopathy that primarily involves the right ventricle, though cases of exclusive left ventricular disease have been reported. It is characterized by hypokinetic areas involving the free wall of the ventricle, with fibrofatty replacement of the myocardium, with associated arrhythmias often originating in the right ventricle. The nomenclature ARVD is currently thought to be inappropriate and misleading as ACM does not involve dysplasia of the ventricular wall. Cases of ACM originating from the left ventricle led to the abandonment of the name ARVC.
ACM can be found in association with diffuse palmoplantar keratoderma, and woolly hair, in an autosomal recessive condition called Naxos disease, because this genetic abnormality can also affect the integrity of the superficial layers of the skin most exposed to pressure stress.[4]: 513 [5]
ACM is an important cause of ventricular arrhythmias in children and young adults. It is seen predominantly in males, and 30–50% of cases have a familial distribution.
- ^ Pilichou K, Thiene G, Bauce B, Rigato I, Lazzarini E, Migliore F, et al. (April 2016). "Arrhythmogenic cardiomyopathy". Orphanet Journal of Rare Diseases. 11 (1): 33. doi:10.1186/s13023-016-0407-1. PMC 4818879. PMID 27038780.
- ^ Heidbuchel H, Prior DL, La Gerche A (November 2012). "Ventricular arrhythmias associated with long-term endurance sports: what is the evidence?". British Journal of Sports Medicine. 46 (Suppl 1): i44–i50. doi:10.1136/bjsports-2012-091162. PMID 23097479. S2CID 41256939.
- ^ "Exercise-induced arrhythmogenic right ventricular cardiomyopathy: fact or fallacy?". 2011. Retrieved 2022-02-18.
- ^ Freedberg IM, Fitzpatrick TB (2003). Fitzpatrick's Dermatology in General Medicine (6th ed.). McGraw-Hill. ISBN 978-0-07-138076-8.
- ^ James WD, Berger TG, Elston DM, Odom RB (2006). Andrews' Diseases of the Skin: Clinical Dermatology (10th ed.). Saunders. ISBN 978-0-7216-2921-6.