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This international multidisciplinary document is intended to guide electrophysiologists, cardiologists, other clinicians, and health care professionals in caring for patients with arrhythmic complications of neuromuscular disorders (NMDs). The document presents an overview of arrhythmias in NMDs followed by detailed sections on specific disorders: Duchenne muscular dystrophy, Becker muscular dystrophy, and limb-girdle muscular dystrophy type 2; myotonic dystrophy type 1 and type 2; Emery-Dreifuss muscular dystrophy and limb-girdle muscular dystrophy type 1B; facioscapulohumeral muscular dystrophy; and mitochondrial myopathies, including Friedreich ataxia and Kearns-Sayre syndrome, with an emphasis on managing arrhythmic cardiac manifestations. End-of-life management of arrhythmias in patients with NMDs is also covered. The document sections were drafted by the writing committee members according to their area of expertise. The recommendations represent the consensus opinion of the expert writing group, graded by class of recommendation and level of evidence utilizing defined criteria. The recommendations were made available for public comment; the document underwent review by the Heart Rhythm Society Scientific and Clinical Documents Committee and external review and endorsement by the partner and collaborating societies. Changes were incorporated based on these reviews. By using a breadth of accumulated available evidence, the document is designed to provide practical and actionable clinical information and recommendations for the diagnosis and management of arrhythmias and thus improve the care of patients with NMDs., (Copyright © 2022 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

A case of a preoperative airway examination performed using telemedicine is presented. The use of this technology has the ability to provide crucial information regarding the feasibility of office-based anesthesia in advance and limit schedule interruptions on the day of surgery., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: Increased left ventricular mass (LVM) is a well-recognized predictor of cardiovascular morbidity and mortality in epidemiological studies, but its impact on mortality after cardiac surgery is poorly defined. We hypothesized that patients with increased LVM index (LVMI) were more likely to have greater 30 day and 1 yr mortality., Methods: With IRB approval, intraoperative transoesophageal echocardiography images of 844 cardiac surgical patients were reviewed. LVMI was calculated using the American Society of Echocardiography recommended formula. Outcome variables studied were 30 day and 1 yr mortality., Results: Mortality within 30 days occurred in 28 patients (3.3%) and within 1 yr in 91 patients (10.8%). An almost linear relationship was found between increasing LVMI and the risk of mortality within 30 days of cardiac surgery. The odds ratio (OR) of dying within 30 days of surgery was 1.15 (95% confidence interval 1.01-1.31) per 20 g m(-2) increase in LVMI. This finding remained statistically significant in multivariate analysis controlling for the effects of age, weight, gender, surgery type, LV function, and functional status [OR=1.36 (1.11-1.66) per 20 g m(-2) increase]. Increased LVMI was not found to be a statistically significant predictor of 1 yr mortality., Conclusions: Increased LVMI, but not LV systolic function as measured by the fractional area of contraction (FAC) was identified as a strong independent predictor of perioperative mortality after adult cardiac surgery. The relationship between LVMI and risk of 30 day mortality was nearly linear. Furthermore, decreased FAC, and not LVMI, was a strong independent predictor of 1 yr mortality.

Purpose: The purpose of this study was to evaluate the biomechanical effect of core decompression of the distal radius for the treatment of Kienböck's disease., Methods: In 7 fresh cadaver upper extremities, axial loads were applied and the pressure in the radiocarpal joint measured using pressure-sensitive film before and after core decompression of the distal radius., Results: Biomechanically, the stiffness of the distal forearm statistically decreased significantly from 229.4 N/mm to 198.6 N/mm after core decompression. No or minimal changes in the distribution of the force in each radiocarpal fossa and ulnocarpal fossa, the area of contact in each fossa, and the location of the centroid of force were observed., Conclusions: Core decompression of the distal radius in the setting of Kienböck's disease has been documented good clinical outcomes, yet the biomechanical analysis of this surgical technique does not demonstrate obvious unloading of the lunate.

The role of the dorsal radiocarpal wrist ligament has been the subject of several investigations. Several biomechanical studies have used sensors inserted dorsally into the wrist joint to evaluate its pressure distribution. The purpose of this study was to evaluate whether a dorsal capsulotomy that sections the dorsal radiocarpal ligament or insertion of a flexible pressure sensor alters scaphoid or lunate kinematics. Eight cadaver upper extremities were instrumented with motion sensors and placed in a wrist joint simulator. Each arm was moved through continual cycles of wrist flexion/extension and radial/ulnar deviation. Motion data were obtained in the intact state, after a capsulotomy, and after insertion of the sensor. We found that either a dorsal capsulotomy sectioning the dorsal radiocarpal ligament or insertion of the pressure sensor alters scaphoid and lunate kinematics during dynamic wrist motion. This study supports the clinical belief that this dorsal wrist ligament should be spared during surgical approaches to the carpus.

The ulnar styloid (US) architecture was examined radiologically and directly by dissection in 13 cadaver wrists to examine a potential relationship between US shape and US abutment syndrome and to determine an optimal radiographic view that could be used to detect US fractures. A higher incidence of US abutment syndrome in the radially deviated US type was observed by dissection. Radiographs taken at varying shoulder abduction angles suggested that the base of the US and ulnar head are not round. To best evaluate the fovea and the US for possible US fractures, radiographs should be taken with the glenohumeral joint abducted 45 degrees, the elbow flexed 90 degrees, the forearm in neutral rotation, and the x-ray beam directed through the wrist perpendicular to the floor.