Arrhythmias & electrophysiologyCohort study

Sudden Death in Young Competitive Athletes Due to Arrhythmogenic Cardiomyopathy: A 4-Decade National Referral Center Experience

Circ Arrhythm ElectrophysiolPublished

  • registry
  • national referral center
  • 1985 to 2024

Authors' conclusion

ACM-related SCD incidence appeared lower in the post-2010 period. A phenotypic shift toward the left ventricular variant is observed, with ECG changes in a minority of cases and usually normal 2-dimensional echocardiography. If the index of suspicion is high, contrast-enhanced cardiac magnetic resonance is crucial for early identification and SCD prevention.

Study context

Population
Competitive athletes (≤40 years old) dying suddenly due to arrhythmogenic cardiomyopathy, North-East Italy registry of juvenile sudden cardiac death (SCD), including cases referred from other regionsathletes with arrhythmogenic cardiomyopathy (ACM) enrolled (50 men, 25±6.4 years)
Participants
Fifty-one athletes
Exposure
before and after 2010

Main finding

As reported in the abstract

ACM as cause of SCD in athletes

ACM was the cause of SCD in athletes

29%

Observational data: associations, not effects.

Further findings

Twelve-lead ECG abnormalities

Twelve-lead ECG abnormalities — overall
50.9%
Twelve-lead ECG abnormalities — negative T waves
39.2%
Twelve-lead ECG abnormalities — low QRS voltage
25.5%
  • Incidence rate of SCD in athletes: before 2010 0.43 (interval 0.27–0.65) per 100 000/y
  • Incidence rate of SCD in athletes: after 2010 0.14 (interval 0.05–0.33) per 100 000/y

Pattern of ACM

right ventricular/biventricular
74.5%
left ventricular
25.5%
left ventricular after 2010
41%

Fibrofatty replacement

transmural in right ventricular/biventricular ACM
63%
exclusively subepicardial-midmural in left ventricular ACM

Abnormalities at first-line preparticipation screening

overall
62.7%
before 1994
83.3%
after 2010
40.9%

Premature ventricular complexes/nonsustained ventricular tachycardia with left bundle-branch block or multiple morphologies on basal or limited exercise ECG

present
35.3%

Positive tests

Maximal stress test
55%
Holter
45.4%
2-dimensional echocardiography
5.8%

Late gadolinium enhancement on contrast-enhanced cardiac magnetic resonance

detected in the only case who underwent contrast-enhanced cardiac magnetic resonance

Original publication

Sudden Death in Young Competitive Athletes Due to Arrhythmogenic Cardiomyopathy: A 4-Decade National Referral Center Experience. Circ Arrhythm Electrophysiol. Published 17 June 2026. doi:10.1161/circep.125.014877

Summarised from the published abstract; every number is checked against it