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. 2022 Mar 17;24(10):1665–1674. doi: 10.1093/europace/euac014

Table 1.

Summary prevalence rates of prospectively reported SCD in arrhythmogenic right ventricular cardiomyopathy patients

Subgroup Studies (k) Participants Incidence rate (95% CI) Heterogeneity Egger’s test (P-value)
No Events I 2 P-value
SCD in ICD positive cohorts
– Overall 8 678 8 0.65 (0.00–6.43) 0.0% 0.998 0.162
SCD in ICD negative cohorts
– Overall 15 1340 68 7.21 (2.38–13.79) 0.0% 0.962 0.011

  • – Probands only

 4 354 19 7.14 (0.07–20.99) 0.0% 0.697 0.293

  • – Probands plus familial

 9 901 45 7.24 (1.65–15.41) 0.0% 0.843 0.070

  • – 2010 TFC

 9 890 55 8.44 (2.38–16.99) 0.0% 0.743 0.025

  • – 1994 and 2010 TFC

 2 113 3 3.27 (0.00–28.99) 0.0% 0.962 ND

  • – European cohorts

 12 1080 53 6.65 (1.61–13.93) 0.0% 0.949 0.040

  • – Trans-Atlantic cohorts

 2 197 13 10.02 (0.00–36.79) 0.0% 0.244 ND
Aborted SCD in ICD negative cohorts
– Overall 8 626 21 4.87 (0.09–13.99) 0.0% 0.667 0.215

  • – 2010 TFC

 4 335 15 7.80 (0.03–23.55) 9.9% 0.344 0.491

  • – Definite ARVC

 2 83 2 3.13 (0.00–35.93) 0.0% 0.998 ND
Aborted SCD and SCD in ICD negative cohorts
– Overall 7 572 50 12.66 (3.71–25.26) 0.0% 0.622 0.421

  • – 2010 TFC

 3 281 29 15.24 (1.25–39.54) 30.4% 0.238 0.213

  • – Definite ARVC

 2 83 14 20.72 (0.00–68.59) 0.0% 0.361 ND

ARVC, arrhythmogenic right ventricular cardiomyopathy; CI, confidence interval; ICD, implantable cardioverter-defibrillator; ND, not determined; SCD, sudden cardiac death; TFC, Task Force Criteria.