Arrhythmias & electrophysiology
The Relevance of the Type of Ventricular Arrhythmia in Titin-Related Dilated Cardiomyopathy: A Multicenter Study
Authors' conclusion
In TTNtv carriers, SMVTs but not frequent PVCs are associated with high mortality due to heart failure. Occurrence of SMVT may identify a subgroup at risk for rapid, progressive adverse remodeling. The prognostic significance of different VA subtypes needs to be confirmed in a larger cohort.
Study context
- Population
- Truncating titin variant (TTNtv) carriers referred for ablation of sustained monomorphic VT (SMVT) or frequent premature ventricular complexes (PVCs)TTNtv carriers referred for ablation · SMVT 14 · frequent PVCs 8
- Participants
- Twenty-two participants
- Comparison
- SMVT vs frequent PVCs among TTNtv carriers
- SMVTn = 14
- PVCn = 8
- Outcome
- NSVT cycle length
Methodological details5 centers
- 5 centers
Main finding
As reported in the abstract
Further findings
Substrate location
- SMVT substrates extended in a basal ring-like fashion with septal predominance; PVC sites of origin limited to the basal anterior left ventricular segment
Procedural success and follow-up outcomes, SMVT group
- Acute complete procedural success
- 36%
- Recurrent VT
- 86%
- Died of progressive heart failure
- 50%
Procedural success and follow-up outcomes, PVC group
- Complete abolition of PVCs
- 13%
- Median PVC burden at 3 months
- 1%
- No deaths or sustained VT during follow-up
Association with mortality and VT-free survival
- Ventricular arrhythmia (VA) subtype and nonsustained ventricular tachycardia (NSVT) cycle length were associated with mortality and poor VT-free survival
Follow-up
- Median follow-up
- 44 months
Original publication
The Relevance of the Type of Ventricular Arrhythmia in Titin-Related Dilated Cardiomyopathy: A Multicenter Study. JACC Clin Electrophysiol. Published 12 March 2025. doi:10.
Summarised from the published abstract; every number is checked against it