Arrhythmogenic right ventricular dysplasia classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Variants of arrythmogenic right ventricular dysplasia can be classified based on the the genetic abnormality involved Variants of arrhythmogenic right ventricular dysplasia can be classified based upon the genetic abnormality involved. So far, 12 variants have been identified. Another classification was based on the clinical manifestation which was first developed by Fontaine et al. and lead to the identification of 3 clinical forms (based on a 9-year observation of 4 patients with different clinical course of ARVC). Later on, another variant of the ARVC classification was proposed, in which the RV and left ventricular forms were distinguished and a total of eleven different clinical forms were identified. However, these classifications did not define prognosis and approaches to treatment and hence, none was widely used in clinical practice. The development of clinical classification is considered an important task and an ultimate challenge.
Classification
Variants of arrhythmogenic right ventricular dysplasia can be classified based upon the genetic abnormality involved into 12 variants:
| Variant | Associated mutation |
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| ARVD1 | This variant is due to a heterozygous mutation in the TGFB3 gene on chromosome 14q24 190230 |
| ARVD2 | Associated with a mutation in the RYR2 gene on chromosome 1q42-q43 180902 |
| ARVD3 | Associated with a mutation in the chromosome 14q12-q22 region 602086 |
| ARVD4 | Associated with a mutation in the chromosome 2q32.1-q32.3 region 602087 |
| ARVD5 | Associated with a mutation in the TMEM43 gene on chromosome 3p23 region 604400 |
| ARVD6 | Associated with a mutation in the chromosome 10p14-p12 region 604401 |
| ARVD7 | Associated with a mutation in the chromosome 10q22.3 region 609160 |
| ARVD8 | Associated with a mutation in the DSP gene on chromosome 6p24 607450, 125647 |
| ARVD9 | Associated with a mutation in the PKP2 gene on chromosome 12p11 609040, 125647 |
| ARVD10 | Associated with a mutation in the DSG2 gene on chromosome 18q12.1-q12 610193, 125671 |
| ARVD11 | Associated with a mutation in the DSC2 gene on chromosome 18q12.1 610476, 125645 |
| ARVD12 | Associated with a mutation in the JUP gene on chromosome 17q21 611528, 173325 |
Clinical classification:[1][2][3][4]
Based on the clinical manifistations and course of the disease, four clinical forms of ARVC have been identified
| Type | Characterestics |
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| Sudden arrhythmic death |
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| Latent arrhythmic form |
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| The manifested arrhythmic form |
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| ARVC with a progressive CHF |
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| ARVC in combination with LVNC |
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| Nonarrhythmic form of ARVC |
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References
- ↑ Fontaine G, Brestescher C, Fontaliran F, Himbert C, Tonet J, Frank R (1995). "[Outcome of arrhythmogenic right ventricular dysplasia. Apropos of 4 cases]". Arch Mal Coeur Vaiss. 88 (7): 973–9. PMID 7487328.
- ↑ Fontaine G, Fontaliran F, Frank R (1998). "Arrhythmogenic right ventricular cardiomyopathies: clinical forms and main differential diagnoses". Circulation. 97 (16): 1532–5. doi:10.1161/01.cir.97.16.1532. PMID 9593556.
- ↑ Gilotra NA, Bhonsale A, James CA, Te Riele ASJ, Murray B, Tichnell C; et al. (2017). "Heart Failure Is Common and Under-Recognized in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia". Circ Heart Fail. 10 (9). doi:10.1161/CIRCHEARTFAILURE.116.003819. PMID 28874384.
- ↑ Norman MW, McKenna WJ (1999). "Arrhythmogenic right ventricular cardiomyopathy: perspectives on disease". Z Kardiol. 88 (8): 550–4. doi:10.1007/s003920050324. PMID 10506390.