A novel wide-band dielectric imaging system to guide radiofrequency ablation for pulmonary vein isolation.

INTRODUCTION: Recently, the wide-band dielectric mapping system Kodex-EPD was introduced. This study reports the first clinical experience using a novel system to guide pulmonary vein isolation (PVI) with radiofrequency (RF) ablation. METHODS AND RESULTS: The study included 20 consecutive patients undergoing de-novo PVI for symptomatic paroxysmal or persistent atrial fibrillation guided by Kodex-EPD. The primary efficacy endpoint was successful PVI. Secondary endpoints included procedural parameters and complications. In all 20 patients (mean age 68 ± 8 years, 12 male patients, paroxysmal fibrillation in 14/20 [70%] patients), PVI was successfully completed. One patient underwent additional cavo-tricuspid isthmus ablation for concomitant typical atrial flutter and one patient required additional ablation of a focal atrial tachycardia. A conventional three-dimensional image of the left atrium as well as the innovative endocardial panoramic view were used to guide catheter manipulation and ablation. Median procedure time was 115 [1st; 3rd quartile 93,75; 140] min and median total fluoroscopy time was 9.9 [9.7; 11.2] min, of which a median of 0.8 [0.6; 0.9] min was required to create left atrial maps. Complete left atrial imaging using Kodex-EPD was achieved within a median of 7.1 [5.7; 8.3] min. Median RF ablation time was 45.1 [34.6; 58.7] min. No major complications were observed. CONCLUSION: RF ablation PVI guided by Kodex-EPD seems safe and feasible. The system provides effective three-dimensional guidance for PVI.

Pulsed-field ablation combined with ultrahigh-density mapping in patients undergoing catheter ablation for atrial fibrillation: Practical and electrophysiological considerations.

BACKGROUND: Pulsed-field ablation (PFA) yields a novel ablation technology for atrial fibrillation (AF). PFA lesions promise to be highly durable, however clinical data on lesion characteristics are still limited. OBJECTIVE: This study sought to investigate PFA lesion creation with ultrahigh-density (UHDx) mapping. METHODS: Consecutive AF patients underwent PFA-based pulmonary vein isolation (PVI) using a multispline catheter (Farwave, Farapulse Inc.). Additional ablation, including left atrial posterior wall isolation (LAPWI) and mitral isthmus ablation (MI) were performed in a subset of persistent AF patients. The extent of PFA-lesions and decrease of LA-voltage were assessed with pre- and post PFA UHDx-mapping (Orion™ catheter and Rhythmia™ 3D-mapping system, Boston Scientific). RESULTS: In 20 patients, acute PVI was achieved in 80/80 PVs, LAPW isolation in 9/9 patients, MI ablation in 2/2 (procedure time: 123 ± 21.6 min, fluoroscopy time: 19.2 ± 5.5 min). UHDx-mapping subsequent to PVI revealed early PV-reconnection in five case (5/80, 6.25%). Gaps were located at the anterior-superior PV ostia and were successfully targeted with additional PFA. Repeat UHDx mapping after PFA revealed a significant decrease of voltage along the PV ostia (1.67 ± 1.36 mV vs. 0.053 ± 0.038 mV, p < .0001) with almost no complex electrogram-fractionation at the lesion border zones. PFA-catheter visualization within the mapping system was feasible in 17/19 (84.9%) patients and adequate in 92.9% of ablation sites. CONCLUSION: For the first time illustrated by UHDx mapping, PFA creates wide antral circumferential lesions and homogenous LAPW isolation with depression of tissue voltage to a minimum. Although with a low incidence, early PV reconnection can still occur also in the setting of PFA.

The core FOXG1 syndrome phenotype consists of postnatal microcephaly, severe mental retardation, absent language, dyskinesia, and corpus callosum hypogenesis.

BACKGROUND: Submicroscopic deletions in 14q12 spanning FOXG1 or intragenic mutations have been reported in patients with a developmental disorder described as a congenital variant of Rett syndrome. This study aimed to further characterise and delineate the phenotype of FOXG1 mutation positive patients. METHOD: The study mapped the breakpoints of a 2;14 translocation by fluorescence in situ hybridisation and analysed three chromosome rearrangements in 14q12 by cytogenetic analysis and/or array comparative genomic hybridisation. The FOXG1 gene was sequenced in 210 patients, including 129 patients with unexplained developmental disorders and 81 MECP2 mutation negative individuals. RESULTS: One known mutation, seen in two patients, and nine novel mutations of FOXG1 including two deletions, two chromosome rearrangements disrupting or displacing putative cis-regulatory elements from FOXG1, and seven sequence changes, are reported. Analysis of 11 patients in this study, and a further 15 patients reported in the literature, demonstrates a complex constellation of features including mild postnatal growth deficiency, severe postnatal microcephaly, severe mental retardation with absent language development, deficient social reciprocity resembling autism, combined stereotypies and frank dyskinesias, epilepsy, poor sleep patterns, irritability in infancy, unexplained episodes of crying, recurrent aspiration, and gastro-oesophageal reflux. Brain imaging studies reveal simplified gyral pattern and reduced white matter volume in the frontal lobes, corpus callosum hypogenesis, and variable mild frontal pachgyria. CONCLUSIONS: These findings have significantly expanded the number of FOXG1 mutations and identified two affecting possible cis-regulatory elements. While the phenotype of the patients overlaps both classic and congenital Rett syndrome, extensive clinical evaluation demonstrates a distinctive and clinically recognisable phenotype which the authors suggest designating as the FOXG1 syndrome.