The Netherlands Arrhythmogenic Cardiomyopathy Registry: design and status update

Background Clinical research on arrhythmogenic cardiomyopathy (ACM) is typically limited by small patient numbers, retrospective study designs, and inconsistent definitions. Aim To create a large national ACM patient cohort with a vast amount of uniformly collected high-quality data that is readily available for future research. Methods This is a multicentre, longitudinal, observational cohort study that includes (1) patients with a definite ACM diagnosis, (2) at-risk relatives of ACM patients, and (3) ACM-associated mutation carriers. At baseline and every follow-up visit, a medical history as well information regarding (non-)invasive tests is collected (e. g. electrocardiograms, Holter recordings, imaging and electrophysiological studies, pathology reports, etc.). Outcome data include (non-)sustained ventricular and atrial arrhythmias, heart failure, and (cardiac) death. Data are collected on a research electronic data capture (REDCap) platform in which every participating centre has its own restricted data access group, thus empowering local studies while facilitating data sharing. Discussion The Netherlands ACM Registry is a national observational cohort study of ACM patients and relatives. Prospective and retrospective data are obtained at multiple time points, enabling both cross-sectional and longitudinal research in a hypothesis-generating approach that extends beyond one specific research question. In so doing, this registry aims to (1) increase the scientific knowledge base on disease mechanisms, genetics, and novel diagnostic and treatment strategies of ACM; and (2) provide education for physicians and patients concerning ACM, e. g. through our website (www.acmregistry.nl) and patient conferences

Exercise training programs to improve hand rim wheelchair propulsion capacity: a systematic review

Objective: An adequate wheelchair propulsion capacity is required to perform daily life activities. Exercise training may be effective to gain or improve wheelchair propulsion capacity. This review investigates whether different types of exercise training programs are effective in improving wheelchair propulsion capacity. Data sources: PubMed and EMBASE databases were searched from their respective inceptions in October 2013. Review methods: Exercise training studies with at least one outcome measure regarding wheelchair propulsion capacity were included. In this study wheelchair propulsion capacity includes four parameters to reflect functional wheelchair propulsion: cardio-respiratory fitness (aerobic capacity), anaerobic capacity, muscular fitness and mechanical efficiency. Articles were not selected on diagnosis, training type or mode. Studies were divided into four training types: interval, endurance, strength, and mixed training. Methodological quality was rated with the PEDro scale, and the level of evidence was determined. Results: The 21 included studies represented 249 individuals with spinal-cord injury (50%), various diagnoses like spina bifida (4%), cerebral palsy (2%), traumatic injury, (3%) and able-bodied participants (38%). All interval training studies found a significant improvement of 18-64% in wheelchair propulsion capacity. Three out of five endurance training studies reported significant effectiveness. Methodological quality was generally poor and there were only two randomised controlled trials. Conclusion: Exercise training programs seem to be effective in improving wheelchair propulsion capacity. However, there is remarkably little research, particularly for individuals who do not have spinal-cord injury