Scar architecture affects the electrophysiological characteristics of induced ventricular arrhythmias in hypertrophic cardiomyopathy

Europace. 2024 Mar 1;26(3):euae050. doi: 10.1093/europace/euae050.

Abstract

Aims: Late gadolinium enhancement cardiac magnetic resonance (LGE-CMR) detects myocardial scarring, a risk factor for ventricular arrhythmias (VAs) in hypertrophic cardiomyopathy (HCM). The LGE-CMR distinguishes core, borderzone (BZ) fibrosis, and BZ channels, crucial components of re-entry circuits. We studied how scar architecture affects inducibility and electrophysiological traits of VA in HCM.

Methods and results: We correlated scar composition with programmed ventricular stimulation-inducible VA features using LGE intensity maps. Thirty consecutive patients were enrolled. Thirteen (43%) were non-inducible, 6 (20%) had inducible non-sustained, and 11 (37%) had inducible sustained mono (MMVT)- or polymorphic VT/VF (PVT/VF). Of 17 induced VA, 13 (76%) were MMVT that either ended spontaneously, persisted as sustained monomorphic, or degenerated into PVT/VF. Twenty-seven patients (90%) had LGE. Of these, 17 (57%) had non-sustained or sustained inducible VA. Scar mass significantly increased (P = 0.002) from non-inducible to inducible non-sustained and sustained VA patients in both the BZ and core components. Borderzone channels were found in 23%, 67%, and 91% of non-inducible, inducible non-sustained, and inducible sustained VA patients (P = 0.003). All 13 patients induced with MMVT or monomorphic-initiated PVT/VF had LGE. The origin of 10/13 of these VTs matched scar location, with 8/10 of these LGE regions showing BZ channels. During follow-up (20 months, interquartile range: 7-37), one patient with BZ channels and inducible PVT had an ICD shock for VF.

Conclusion: Scar architecture determines inducibility and electrophysiological traits of VA in HCM. Larger studies should explore the role of complex LGE patterns in refining risk assessment in HCM patients.

Keywords: Hypertrophic cardiomyopathy; Magnetic resonance; Programmed ventricular stimulation; Scar; Sudden death; Ventricular tachycardia.

MeSH terms

  • Arrhythmias, Cardiac / complications
  • Arrhythmias, Cardiac / etiology
  • Cardiomyopathy, Hypertrophic* / complications
  • Cardiomyopathy, Hypertrophic* / diagnostic imaging
  • Cicatrix / complications
  • Cicatrix / pathology
  • Contrast Media
  • Gadolinium / pharmacology
  • Humans
  • NAV1.5 Voltage-Gated Sodium Channel / deficiency*
  • Tachycardia, Ventricular* / diagnosis
  • Tachycardia, Ventricular* / etiology
  • Ventricular Fibrillation*

Substances

  • Contrast Media
  • Gadolinium
  • NAV1.5 Voltage-Gated Sodium Channel

Supplementary concepts

  • Ventricular Fibrillation, Paroxysmal Familial, 1