LAUSR

BACKGROUND Little is known about persistent atrial fibrillation (AF) ablation in patients with cardiac laminopathy (CLMNA). OBJECTIVE We aimed to characterize atrial electrophysiological properties and to assess the long-term outcomes of persistent AF ablation in CLMNA patients. METHODS All CLMNA patients referred in our center for persistent AF ablation were retrospectively included. Left atrium (LA) volume, left atrial appendage (LAA) cycle length (CL), inter-atrial conduction delay and LA voltage amplitude were analyzed during the ablation procedure. Sinus rhythm maintenance and LA contractile function were assessed during long-term follow-up. RESULTS From 2011 to 2020, 8 patients were included (47 ± 14 years; 3 women). LA volume was 205.8 ± 43.7mL, LAA AF CL was 250.7 ± 85.6ms and inter-atrial conduction delay was 296.5 ± 110.1ms. Large low voltage areas (>50% of LA surface; < 0,5mV EGM) were recorded in all 8 patients. 2 patients had inadvertent LAA disconnection during ablation. All A wave recorded by pulsed doppler in sinus rhythm were < 30cm/s, before, and after AF ablation. Early arrhythmia recurrence was recorded in 7 (87%) patients (time to recurrence 4 ± 4 months; 1.5 procedures per patient). After a mean follow-up of 4.4 ± 3.2 years, 4 patients underwent ICD therapy for life threatening ventricular arrhythmia and 3 patients finally underwent heart transplantation (HT). CONCLUSION Persistent AF patients afflicted by CLMNA exhibit severe LA impairment due to large low-voltage areas, prolonged conduction velocity and reduced contractile function. Ablation procedures have a limited impact with a high recurrence rate.