Arrhythmias & electrophysiology
Electrophysiological Phenotype-Genotype Study of Sustained Monomorphic Ventricular Tachycardia in Inherited, High Arrhythmic Risk, Left Ventricular Cardiomyopathy
Authors' conclusion
The anatomic substrate of SMVTs shows a strong correlation with the underlying genotype, electrocardiographic morphology, and recurrence rate. Particularly, patients with nuclear membrane gene variants have a significantly higher recurrence rate compared with those with desmosomal gene variants.
Study context
- Population
- Patients with inherited high-risk genetic cardiomyopathy with LV involvement and sustained monomorphic ventricular tachycardia (SMVT) treated by electrophysiological study76% men
- Participants
- 71 patients
- Exposure
- Genotype groups
- Outcome
- Location and electrophysiological profile of SMVT substrate by genotype
Methodological details18 European/US centres
- 18 European/US centres
Main finding
As reported in the abstract
Clinical-SMVT substrate location by genotype
Substrate location differed significantly by genotype
P=0.005
Desmosomal genes, TMEM43, PLN biventricular disease; LMNA and cytoskeleton predominant LV involvement
P=0.001
Observational data: associations, not effects.
Further findings
| Outcome | HR (95% CI) | |
|---|---|---|
| Recurrence of clinical SMVT after ablation · median 26 Q1-Q3 (10.6-65) monthsNuclear membrane vs Desmosomal | 4.56 (1.5–13.8) | |
| 0.050.21520Hazard ratio (log scale) |
- Recurrence of clinical SMVT after ablation — Recurrence in all patients
- 27%
- Recurrence of clinical SMVT after ablation — Differences between genotypes (log-rank)
- 0.016
Original publication
Electrophysiological Phenotype-Genotype Study of Sustained Monomorphic Ventricular Tachycardia in Inherited, High Arrhythmic Risk, Left Ventricular Cardiomyopathy. Circ Arrhythm Electrophysiol. Published 29 November 2024. doi:10.
Summarised from the published abstract; every number is checked against it