Arrhythmias & electrophysiologyCohort study
Incidence of Atrial and Ventricular Arrhythmias in Patients With Titin Truncating Variants Across the Ventricular Morphofunctional Phenotypic Spectrum
- retrospective review
- registry-based
Authors' conclusion
When stratified by morphofunctional phenotype, there was no difference in VAs across morphofunctional phenotypes in patients with TTNtvs. This highlights that patients with TTNtvs may be at risk of SCA/SCD even without overt ventricular cardiomyopathy. The risk of new-onset AF was highest in patients with DCM but was seen at all phenotypic stages during follow-up.
Study context
- Population
- TTNtv-positive patients with pathogenic/likely pathogenic TTNtvs in an Arrhythmogenic/Dilated Cardiomyopathy RegistryTTNtv-positive patients identified from 1,229 registry patients
- Participants
- 253 patients
- Exposure
- Nondilated left ventricular cardiomyopathy (NDLVC) and dilated cardiomyopathy (DCM) phenotypes compared with G+/P- patients
- G+/P-n = 54reference
- NDLVCn = 49
- DCMn = 150
Main finding
As reported in the abstract
New-onset AF · median follow-up of 3.8 years
Each group vs G+/P-Hazard ratio (95% CI)P value
NDLVC
3.29(0.71–15.3)
P = 0.13DCMworse AF-free survival
4.86(1.17–20.1)
P = 0.03- new-onset atrial fibrillation (AF) (26% of those at risk)
- 67 patients
Observational data: associations, not effects.
Further findings
| Outcome | HR (95% CI) | P value | |
|---|---|---|---|
| New-onset VAs — NDLVC vs G+/P- | 0.71 (0.19–2.69) | P = 0.62 | |
| New-onset VAs — DCM vs G+/P- | 0.97 (0.37–2.54) | P = 0.95 | |
| 0.20.5125Hazard ratio (log scale) |
- New-onset ventricular arrhythmias (VAs) — New-onset VAs
- 44 (17%)
- New-onset VAs — SCA
- 12 (5%)
Cardiac transplantation and cardiac death
- Cardiac transplantation
- 14 (6%)
- Died of cardiac causes
- 6 (2%)
- SCD
- 2 cases
Original publication
Incidence of Atrial and Ventricular Arrhythmias in Patients With Titin Truncating Variants Across the Ventricular Morphofunctional Phenotypic Spectrum. JACC Clin Electrophysiol. Published 28 August 2026. doi:10.1016/j.jacep.2026.07.022
Summarised from the published abstract; every number is checked against it