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

Kumar, Saurabh; Androulakis, Alexander F A; Sellal, Jean-Marc; Maury, Philippe; Gandjbakhch, Estelle; Waintraub, Xavier; Rollin, Anne; Richard, Pascale; Charron, Philippe; Baldinger, Samuel Hannes; Macintyre, Ciorsti J; Koplan, Bruce A; John, Roy M; Michaud, Gregory F; Zeppenfeld, Katja; Sacher, Frederic; Lakdawala, Neal K; Stevenson, William G; Tedrow, Usha B (2016). Multicenter Experience With Catheter Ablation for Ventricular Tachycardia in Lamin A/C Cardiomyopathy. Circulation. Arrhythmia and electrophysiology, 9(8) Lippincott Williams & Wilkins 10.1161/CIRCEP.116.004357

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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.

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