Exercise Restriction in ARVC
Definition
The evidence-based guideline recommendation limiting competitive and high-intensity endurance exercise in patients with ARVC and in genotype-positive, phenotype-negative relatives. Exercise is the most important modifiable environmental determinant of ARVC penetrance, arrhythmic risk, and structural progression.
Key Concepts
Dose-Dependent Relationship
- There is a dose-dependent relationship between exercise exposure (intensity × duration) and: (1) likelihood of ARVC disease development; (2) severity of RV and LV structural dysfunction; (3) risk of sustained ventricular arrhythmias. (sources/acm-hrs-2019)
- Endurance athletes had significantly higher exercise doses (MET-min/week) across their lifetime than non-athletic relatives who did not develop ARVC; earlier onset of athletic training correlated with earlier ICD implantation (Saberniak et al.).
- In 10 PKP2 families, family members who developed ARVC exercised at a higher dose across their lifespan than those who did not (Groeneweg et al.).
Exercise Definitions Used in Guidelines
- Competitive exercise: Organized team or individual sport requiring regular competition, systematic intense training, and premium on achievement.
- Endurance exercise: Class B or C activities per AHA/ACC (moderate or high dynamic demand): e.g., running, cross-country skiing, rowing, basketball, soccer, competitive cycling (≥6 METs = vigorous intensity).
- Intensity independently predicts arrhythmic outcomes after adjusting for duration (Lie et al.).
Recommendations
- COR III: Harm, LOE B-NR — Individuals with ARVC should NOT participate in competitive or frequent high-intensity endurance exercise. This is associated with increased risk of ventricular arrhythmias and promotion of structural disease progression. (sources/acm-hrs-2019)
- COR I, LOE B-NR — Clinicians should counsel adolescent and adult individuals who are genotype-positive/phenotype-negative for ARVC that competitive or frequent high-intensity endurance exercise is associated with increased likelihood of developing ARVC and ventricular arrhythmias. (sources/acm-hrs-2019)
- Reducing exercise after presentation is associated with better arrhythmic outcomes — patients who continued competitive exercise had significantly worse arrhythmic course (North American ARVC Registry, n=108 probands).
- Among 129 ARVC patients with ICDs, the largest reduction in exercise dose (MET-hours/year) predicted best survival from ICD therapy (Wang et al.).
Gene-Specific Exercise Effects (Muller 2025)
- The evidence that exercise is a modifiable penetrance/VA risk factor is gene-specific — not uniform across all ACM genotypes. (sources/ACM-Genotype-Mx-JCE-2024, rating: high)
- PKP2-ARVC: Strongest evidence base — exercise is clearly associated with penetrance and VA. Exercise restriction warranted. (sources/ACM-Genotype-Mx-JCE-2024)
- TMEM43-ARVC: Exercise associated with worse outcomes — restriction warranted. (sources/ACM-Genotype-Mx-JCE-2024)
- Gene-elusive ARVC: Exercise associated with VAs — restriction warranted. (sources/ACM-Genotype-Mx-JCE-2024)
- PLN-p.Arg14del: Exercise does NOT influence development of penetrant disease, VA, or HF events — restriction may not be necessary in carriers without known VA risk factors. This is a gene-specific departure from standard ACM exercise guidance. (sources/ACM-Genotype-Mx-JCE-2024)
- DSP: Impact of exercise on penetrance is uncertain — insufficient data. (sources/ACM-Genotype-Mx-JCE-2024)
- Other genotypes: For genotypes with no available exercise data, err on the side of caution and advise restriction. (sources/ACM-Genotype-Mx-JCE-2024)
- 2024 HRS athlete consensus recommends gene-specific shared decision-making for exercise recommendations. (sources/ACM-Genotype-Mx-JCE-2024)
- Gene-elusive, athletic ARVC: A subset of patients with very high athletic exposure, no identifiable pathogenic desmosomal variant, and no family history may have a largely exercise-induced form. Unaffected relatives have low risk; exercise restriction is not yet strongly evidence-based for these patients.
- Incidental genetic findings: Penetrance of ARVC-associated variants identified through population sequencing (not familial cascade testing) may be lower; the benefit of exercise restriction is uncertain and requires further study.
- Other ACMs (PLN, LMNA, FLNC): Exercise-related arrhythmias occur in PLN R14del carriers; however, athletic history does not appear to predict penetrance in this group. Exercise restriction data for non-ARVC ACMs are insufficient.
Quantitative Exercise Reference
| METs | Activity |
|---|---|
| 16 | Competitive cycling |
| 15 | Cross-country ski racing (>8 mph) |
| 12 | Competitive rowing/crew |
| 10 | Competitive soccer |
| 9.8 | Running 6 mph |
| 8 | Basketball game |
| 5 | Brisk walking 4 mph |
| 3.5 | Walking for pleasure |
| 2.5 | Yoga |
Activities ≥6 METs are vigorous-intensity and should be avoided or performed very rarely by ARVC patients.
AHA/ACC 2025 Sports Statement — Key Update: PKP2 vs. Non-PKP2 ACM
- The 2025 AHA/ACC statement codifies the PKP2 vs. non-PKP2 distinction for the first time in a major US guideline: (sources/competitive-sports-aha-2025, rating: very high)
- Clinical PKP2 ACM: Risks of VA, structural disease progression, and SCD with endurance/high-intensity competitive sport likely outweigh benefits. Align with prior exercise restriction data.
- Clinical non-PKP2 ACM: Evidence for increased SCA risk or disease acceleration is not established. Competitive sports can be considered with SDM — individualize carefully given uncertainty.
- Genotype+/phenotype- (any genotype): Competitive sports reasonable to consider with SDM; 6–12 month imaging surveillance to detect phenotypic conversion.
- This marks a shift from the HRS 2019/ESC 2022 approach of treating all definite ACM uniformly, toward genotype-guided nuance. The PKP2 restriction aligns with established exercise-penetrance data; the SDM approach for non-PKP2 reflects absence of equivalent evidence for those genotypes.
- See concepts/Sports-Cardiology-SDM for the full SDM framework.
Contradictions / Open Questions
- PKP2-centric evidence — other genotypes extrapolated: The dose-dependent exercise-penetrance data are predominantly from PKP2 carriers. Exercise restriction in PLN, LMNA, FLNC, DSP, and non-desmosomal ACMs is based on expert extrapolation rather than direct evidence. PLN R14del carriers have exercise-related arrhythmias but athletic history does not appear to predict penetrance, suggesting exercise restriction may not carry the same benefit across all genotypes. (sources/acm-hrs-2019)
- ESC 2022 vs. HRS 2019 on genotype-positive/phenotype-negative individuals: HRS 2019 (COR I, LOE B-NR) requires clinicians to counsel phenotype-negative individuals that competitive/high-intensity exercise increases likelihood of developing ARVC. ESC 2022 (Class IIb) takes a softer stance — exercise avoidance in phenotype-negative mutation carriers "may be considered." The same underlying evidence supports different recommendation strengths in two major guidelines published within 3 years of each other. (sources/acm-hrs-2019, sources/VA-SCD-ESC-2022)
- Gene-elusive athletic ARVC: A subset of patients with very high athletic exposure and an ARVC phenotype carry no identifiable pathogenic variant. If exercise is the primary driver, their unaffected relatives have low risk and may not need restriction counseling — yet current guidelines do not formally differentiate management based on gene-elusive vs. gene-positive status. (sources/acm-hrs-2019)
Connections
- Related to concepts/Arrhythmogenic-Cardiomyopathy
- Related to entities/ARVC
- Related to entities/PKP2
- Related to concepts/Sudden-Cardiac-Death
- Related to concepts/Sports-Cardiology-SDM
- Related to sources/competitive-sports-aha-2025
- Related to sources/ACM-Genotype-Mx-JCE-2024