Arrhythmogenic right ventricular dysplasia epidemiology and demographics

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

ARVD is a rare disease. It is observed more commonly in males, and accounts for 17% of all sudden cardiac deaths in the young.

Epidemiology and demographics

Incidence

The incidence of ARVC/D is difficult to be determined due the different clinical manifestations of the disease which vary greatly, especially in different ethnic groups. This could be secondary to the genetic heterogeneity and variable phenotype expression, diverse disease progression. All this make the diagnosis difficult and decreases the real evaluation of the incidence.

The incidence of ARVD is about 1/10,000 in the general population in the United States, although some studies have suggested that it may be as common as 1/1,000.
In Italy, the incidence is 40/10,000, making it the most common cause of sudden cardiac death in the young population. It is more common in Northern Italy ^[1]^[2]

Prevalence

The estimated prevalence of ARVC/D in the general population is approximately 1:5000
ARVC/D accounts for 11%–22% of cases of SCD in the young athlete patient population^[3]^[4]
It accounts for approximately 22% of cases in athletes in northern Italy and about 17% of SCD in young people in the United States

Gender

Men are more frequently affected than women, with a male to female ratio of 3:1

References

↑ Lahtinen AM, Lehtonen E, Marjamaa A, Kaartinen M, Heliö T, Porthan K; et al. (2011). “Population-prevalent desmosomal mutations predisposing to arrhythmogenic right ventricular cardiomyopathy”. Heart Rhythm. 8 (8): 1214–21. doi:10.1016/j.hrthm.2011.03.015. PMID 21397041.
↑ Peters S, Trümmel M, Meyners W (2004). “Prevalence of right ventricular dysplasia-cardiomyopathy in a non-referral hospital”. Int J Cardiol. 97 (3): 499–501. doi:10.1016/j.ijcard.2003.10.037. PMID 15561339.
↑ Thiene G, Nava A, Corrado D, Rossi L, Pennelli N (1988). “Right ventricular cardiomyopathy and sudden death in young people”. N Engl J Med. 318 (3): 129–33. doi:10.1056/NEJM198801213180301. PMID 3336399.
↑ Corrado D, Basso C, Schiavon M, Thiene G (1998). “Screening for hypertrophic cardiomyopathy in young athletes”. N Engl J Med. 339 (6): 364–9. doi:10.1056/NEJM199808063390602. PMID 9691102.

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[pmid21397041-1] Lahtinen AM, Lehtonen E, Marjamaa A, Kaartinen M, Heliö T, Porthan K; et al. (2011). “Population-prevalent desmosomal mutations predisposing to arrhythmogenic right ventricular cardiomyopathy”. Heart Rhythm. 8 (8): 1214–21. doi:10.1016/j.hrthm.2011.03.015. PMID 21397041.

[pmid15561339-2] Peters S, Trümmel M, Meyners W (2004). “Prevalence of right ventricular dysplasia-cardiomyopathy in a non-referral hospital”. Int J Cardiol. 97 (3): 499–501. doi:10.1016/j.ijcard.2003.10.037. PMID 15561339.

[pmid3336399-3] Thiene G, Nava A, Corrado D, Rossi L, Pennelli N (1988). “Right ventricular cardiomyopathy and sudden death in young people”. N Engl J Med. 318 (3): 129–33. doi:10.1056/NEJM198801213180301. PMID 3336399.

[pmid9691102-4] Corrado D, Basso C, Schiavon M, Thiene G (1998). “Screening for hypertrophic cardiomyopathy in young athletes”. N Engl J Med. 339 (6): 364–9. doi:10.1056/NEJM199808063390602. PMID 9691102.

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