Case Reports: Emery-Dreifuss Muscular Dystrophy Presenting as a Heart Rhythm Disorders in Children.

Emery-Dreifuss muscular dystrophy (EDMD) is inherited muscle dystrophy often accompanied by cardiac abnormalities in the form of supraventricular arrhythmias, conduction defects and sinus node dysfunction. Cardiac phenotype typically arises years after skeletal muscle presentation, though, could be severe and life-threatening. The defined clinical manifestation with joint contractures, progressive muscle weakness and atrophy, as well as cardiac symptoms are observed by the third decade of life. Still, clinical course and sequence of muscle and cardiac signs may be variable and depends on the genotype. Cardiac abnormalities in patients with EDMD in pediatric age are not commonly seen. Here we describe five patients with different forms of EDMD (X-linked and autosomal-dominant) caused by the mutations in EMD and LMNA genes, presented with early onset of cardiac abnormalities and no prominent skeletal muscle phenotype. The predominant forms of cardiac pathology were atrial arrhythmias and conduction disturbances that progress over time. The presented cases discussed in the light of therapeutic strategy, including radiofrequency ablation and antiarrhythmic devices implantation, and the importance of thorough neurological and genetic screening in pediatric patients presenting with complex heart rhythm disorders.

Comparative Safety and Efficacy of Left Atrial Appendage Occlusion with the Watchman Device and Amplatzer Cardiac Plug: Results of the Russian National Registry.

PURPOSE: This multicenter, prospective registry evaluated the comparative safety and efficacy of left atrial appendage occlusion (LAAO) using the Watchman device (WD) and the Amplatzer Cardiac Plug (ACP) in patients with nonvalvular atrial fibrillation (NVAF) in real-world clinical practice in Russia. METHODS: The study included data from 200 consecutive NVAF patients (66.8 ± 7.8 years, 44.5% female, median CHA2DS2VASc 4, median HAS-BLED 3) who had undergone LAAO implantation using WD (n = 108) or ACP (n = 92) from September 2015 to December 2017 in 5 medical centers in Russia. The primary safety endpoint was the procedure-related major adverse events, and the primary efficacy endpoint was the composite of thromboembolic events, device thrombosis, hemorrhagic events, and unexplained death during the 12-month follow-up. RESULTS: Successful LAAO was performed in all 92 (100%) patients with ACP and 105 (97.2%) with WD (p = 0.053). At 12 months, primary safety endpoint occurred in 6.5% of patients in the ACP group with no events in the WD group (6.5% vs. 0%, p = 0.008). During the 12-month follow-up, the primary efficacy endpoint has occurred in 8.3% of patients in the WD group (n = 9) and 1.1% of patients in the ACP group (n = 1) (p = 0.016). CONCLUSIONS: In this multicenter prospective registry, LAA closure with the WD was associated with significantly higher thromboembolic events rate in NVAF patients. Patients, receiving the ACP, had more procedure-related major adverse events. However, further multicenter studies are necessary to evaluate these findings.

Catheter Ablation of Incisional Atrial Tachycardia.

Tachycardias after atrial incisions represent frequent and serious problem. The majority of them are based on a re-entry electrical activation around a combination of anatomic and surgically created obstacles. Considering significant progress of cardiovascular surgery during the last decade along with potential large amount of open-heart procedures in the near future the number of incisional tachycardias has a tendency to increase. The aim of this work was to quantify the magnitude of the problem, characterize the tachycardias after different surgical operations and to analyze possible interventional treatment strategies. Nowadays evolution of mapping and ablation technologies may contribute to radically treatment of this type of arrhythmias while there are still a lot of issues that should be solved to improve the results of interventional treatment of incisional tachycardias.