Related Experiment Video

Updated: Aug 6, 2026

Ablation of Ischemic Ventricular Tachycardia Using a Multipolar Catheter and 3-dimensional Mapping System for High-density Electro-anatomical Reconstruction
06:57

Ablation of Ischemic Ventricular Tachycardia Using a Multipolar Catheter and 3-dimensional Mapping System for High-density Electro-anatomical Reconstruction

Published on: January 31, 2019

Ventricular Arrhythmia Ablation in Inherited Cardiomyopathy: Phenotype Variability and Outcomes Based on Functional

Arian Afzalian1, Alireza Oraii1, Timothy M Markman1

  • 1Section of Cardiac Electrophysiology, Cardiovascular Division, Perelman School of Medicine University of Pennsylvania Philadelphia PA USA.

Journal of the American Heart Association
|July 17, 2026
PubMed

Insights

Genetics influence ventricular tachycardia (VT) substrate in inherited cardiomyopathies. Ablation reduces VT burden, but recurrence is common, especially in Lamin A/C (LMNA) variants, while non-desmoplakin (DSP) desmosomal variants show better outcomes.

Area of Science:

  • Cardiology
  • Genetics
  • Electrophysiology

Background:

  • Inherited cardiomyopathies are a significant cause of ventricular tachycardia (VT) and heart failure.
  • Current understanding of VT substrate based on genetic mutations is limited.

Purpose of the Study:

  • To investigate the correlation between specific genetic variants and VT substrate characteristics.
  • To evaluate the efficacy of ablation in managing VT and premature ventricular complexes (PVCs) in patients with inherited cardiomyopathies.
  • To assess long-term outcomes, including VT recurrence and heart failure progression, stratified by genotype.

Main Methods:

  • Retrospective analysis of 105 patients with inherited cardiomyopathy undergoing ablation for VT or PVCs.
  • Characterization of arrhythmia substrate, ablation procedures, and clinical outcomes.
  • Genotyping included desmosomal (DSP, non-DSP), titin, Lamin A/C (LMNA), sarcomeric, ion-channel, and cytoskeletal/Z-disk variants.

Main Results:

  • VT substrate location varied significantly by genotype, with LMNA, titin, and sarcomeric groups showing predominantly septal involvement.
  • Ablation eliminated clinical VT in 90% of cases, with 65% achieving noninducibility.
  • Three-year VT-free survival was 51%, with highest rates in non-DSP desmosomal and lowest in LMNA groups (66% vs. 20%, P=0.03).
  • LMNA variants were associated with a higher risk of VT recurrence and end-stage heart failure (52% vs. 29% overall).

Conclusions:

  • Genetic genotype significantly correlates with VT substrate patterns in inherited cardiomyopathies.
  • Catheter ablation effectively reduces VT and PVC burden, but long-term VT recurrence remains a concern, particularly for LMNA variants.
  • Non-DSP desmosomal variants demonstrate superior long-term VT-free survival compared to other genotypes.
Abstract

Keywords:
FLNCLMNAVT ablationarrhythmogenic cardiomyopathyinherited cardiomyopathyventricular tachycardia

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