Arrhythmias & electrophysiology

Distribution of myocardial fibrosis in patients with nonischemic cardiomyopathy and ventricular tachycardia based on genetic variant

Heart RhythmPublished Retrospective case series

Authors' conclusion

Understanding spatial relationships between genetic variants causing NICM and VT substrate locations can help lead to generalizable regions in patients with genetically related NICM presenting in VT, which can be investigated during ablation procedures.

Study context

Population
Genetic NICM patients (aged 55 ± 16 years; 94% male; LVEF 34% ± 13%) referred for VT ablation, Oct 2018–Nov 2022
Participants
32 patients
Exposure
TTN vs LMNA vs PKP2 vs MYBPC3 vs DSP vs TTR vs FLNC vs AGL vs DES vs DSG2
  • TTNn = 11
  • LMNAn = 6
  • PKP2n = 5
  • MYBPC3n = 3
  • DSPn = 2
  • TTRn = 1
  • FLNCn = 1
  • AGLn = 1
  • DESn = 1
  • DSG2n = 1
Outcome
VT substrate (scar) locations by genetic variant, defined as areas of low unipolar or bipolar voltage
Methodological detailssingle-centre
  • Single-centre

Main finding

As reported in the abstract

VT substrate location by variant

TTN: substrate only in basal subregions, predominantly anterior and septal

anterior 100%, septal 50%

LMNA: fibrosis in mid inferolateral and apical inferolateral regions

mid inferolateral 60%, apical inferolateral 60%

Observational data: associations, not effects.

Original publication

Distribution of myocardial fibrosis in patients with nonischemic cardiomyopathy and ventricular tachycardia based on genetic variant. Heart Rhythm. Published 28 June 2024. doi:10.1016/j.hrthm.2024.06.047

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

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