Multicenter Experience With Catheter Ablation for Ventricular Tachycardia in Lamin A/C Cardiomyopathy

Abstract

Background— Lamin A/C ( LMNA ) cardiomyopathy is a genetic disease with a proclivity for ventricular arrhythmias. We describe the multicenter experience with percutaneous catheter ablation of sustained monomorphic ventricular tachycardia (VT) in LMNA cardiomyopathy. Methods and Results— Twenty-five consecutive LMNA mutation patients from 4 centers were included (mean age, 55±9 years; ejection fraction, 34±12%; VT storm in 36%). Complete atrioventricular block was present in 11 patients; 3 patients were on mechanical circulatory support for severe heart failure. A median of 3 VTs were inducible per patient; in 82%, mapping was consistent with origin from scar in the basal left ventricle, particularly the septum, but also basal inferior wall and subaortic mitral continuity. After multiple procedures (median 2/patient; transcoronary alcohol in 6 and surgical cryoablation in 2 patients), acute success (noninducibility of any VT) was achieved in only 25% of patients. Partial success (inducibility of a nonclinical VT only: 50%) and failure (persistent inducibility of clinical VT: 12.5%) was attributed to intramural septal substrate in 13 of 18 patients (72%). Complications occurred in 25% of patients. After a median follow-up of 7 months after the last procedure, 91% experienced ≥1 VT recurrence, 44% received or were awaiting mechanical circulatory support or transplant for end-stage heart failure, and 26% died. Conclusions— Catheter ablation of VT associated with LMNA cardiomyopathy is associated with poor outcomes including high rate of arrhythmia recurrence, progression to end-stage heart failure, and high mortality. Basal septal scar and intramural VT origin makes VT ablation challenging in this population.

Lamin and the heart

“…There are also doubts about the effectiveness of catheter ablation for VT in LHD where the combination of a predominantly intramural VT substrate and basal perivalvular scar make VT ablation particularly challenging40 and may not prevent arrhythmia recurrence or progression to advanced heart failure 40. Catheter ablation of clinical VT in non-ischaemic DCM is therefore reserved for patients presenting with a clear VT mechanism (eg, bundle branch re-entry) and only in experienced centres.…”

Multipolar mapping with the high‐density grid catheter compared with conventional point‐by‐point mapping to guide catheter ablation for focal arrhythmias

“…Prior clinical studies have demonstrated the role of MPM catheters for the treatment of atrial and ventricular arrhythmias in the presence of structural heart disease 19–22 . However, few studies have compared multipolar with conventional PbyP mapping.…”

European Heart Rhythm Association (EHRA)/Heart Rhythm Society (HRS)/Asia Pacific Heart Rhythm Society (APHRS)/Latin American Heart Rhythm Society (LAHRS) expert consensus on risk assessment in cardiac arrhythmias: use the right tool for the right outcome, in the right population

“…With regard to VT recurrence, in addition to patient related comorbidities, large single centre and multicentre studies have shown that the risk of VT recurrence is driven by the underlying aetiology, particularly in patients with non‐ischaemic heart disease, even after adjusting for other patient comorbidities 492–494 . In particular, patients with Lamin A/C cardiomyopathy, hypertrophic cardiomyopathy, cardiac sarcoidosis, and valvular cardiomyopathy appear to be at higher risk for VT recurrence after catheter ablation as compared to idiopathic dilated cardiomyopathy 485,492 . In addition, location of scar seems to determine risk of VT recurrence post‐catheter ablation 495 .…”