Heart failure & cardiomyopathies
Diagnostic and Prognostic Significance of Exercise Stress Testing in Desmosomal Arrhythmogenic Cardiomyopathy
Authors' conclusion
PKP2-ACM patients display a distinct recovery phase arrhythmic profile predictive of MVA events, emphasizing the importance of EST in risk stratification and management.
Study context
- Population
- Patients with desmosomal arrhythmogenic cardiomyopathy (pathogenic/likely pathogenic variant in DSC2, DSG2, DSP, JUP or PKP2) with exercise stress test data
- Participants
- 147 patients · 326 exercise stress tests (ESTs)
- Exposure
- Recovery-phase ventricular arrhythmias on EST, PKP2 vs other desmosomal genotypes
- PKP2n = 89
- DSPn = 45
- DSG2n = 10
- DSC2n = 3
- Outcome
- Major ventricular arrhythmia events:
- Sudden cardiac arrest
- Sustained VT
- Appropriate ICD therapies
Main finding
As reported in the abstract
Major ventricular arrhythmia (MVA) events with recovery-phase arrhythmias
- PKP2: recovery-phase arrhythmias vs no recovery-phase arrhythmias
- HR 10.580; P = 0.003
- Other genotypes: recovery-phase arrhythmias vs no recovery-phase arrhythmias
- HR 1.037; P = 0.973
- PKP2: recovery-phase arrhythmias vs no recovery-phase arrhythmias · multivariable analysis
- adjusted HR 3.851; P = 0.040
Observational data: associations, not effects.
Further findings
Recovery-phase arrhythmic burden, PKP2-ACM vs other genotypes
- Increased PVCs per minute in PKP2-ACM
- P < 0.001
- Increased bigeminy in PKP2-ACM
- P < 0.001
- Increased couplets in PKP2-ACM
- P < 0.001
- Increased nonsustained VT in PKP2-ACM
- P < 0.001
Original publication
Diagnostic and Prognostic Significance of Exercise Stress Testing in Desmosomal Arrhythmogenic Cardiomyopathy. JACC Clin Electrophysiol. Published 4 September 2025. doi:10.
Summarised from the published abstract; every number is checked against it