Arrhythmogenic right ventricular cardiomyopathy: From pathophysiology to diagnosis and advances in management

Our understanding of arrhythmogenic right ventricular cardiomyopathy (ARVC) has advanced considerably over the past 30- 40 years. This is an inherited cardiomyopathy with complicated genetic inheritance and variable penetrance. Desmosomal dysfunction underlies most cases, and appreciating this pathophysiology has contributed to patient management, particularly with respect to exercise restriction to reduce disease progression. The diagnosis is made according to a series of Task Force Criteria, and subsequent management is guided by expert consensus in the absence of comparative data. ARVC is associated with sudden cardiac death (SCD), particularly in young athletic individuals who unknowingly harbour the condition. Risk stratification is important to guide implantable cardioverter- defibrillator use and reduce SCD. Residual gaps in our understanding, particularly surrounding incomplete penetrance, the underlying pathophysiology and risk stratification, are being targeted by collaborative efforts, large registries, prospective studies and translational research

Novel electrocardiographic criteria for the diagnosis of arrhythmogenic right ventricular cardiomyopathy

Aims: In order to improve the electrocardiographic (ECG) diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC), we evaluated novel quantitative parameters of the QRS complex and the value of bipolar chest leads (CF leads) computed from the standard 12 leads. Methods and results: We analysed digital 12-lead ECGs in 44 patients with ARVC, 276 healthy subjects including 44 age and sex-matched with the patients and 36 genotyped members of ARVC families. The length and area of the terminal S wave in V1 to V3 were measured automatically using a common for all 12 leads QRS end. T wave negativity was assessed in V1 to V6 and in the bipolar CF leads computed from the standard 12 leads. The length and area of the terminal S wave were significantly shorter, whereas the S wave duration was significantly longer in ARVC patients compared with matched controls. Among members of ARVC families, those with mutations (n = 15) had shorter QRS length in V2 and V3 and smaller QRS area in lead V2 compared with those without mutations (n = 20). In ARVC patients, the CF leads were diagnostically superior to the standard unipolar precordial leads. Terminal S wave duration in V1 >48 ms or major T wave negativity in CF leads separated ARVC patients from matched controls with 90% sensitivity and 86% specificity. Conclusion: The terminal S wave length and area in the right precordial leads are diagnostically useful and suitable for automatic analysis in ARVC. The CF leads are diagnostically superior to the unipolar precordial leads

Changing Perspectives of Local Therapists Eight Years after the Implementation of an Occupational Therapy Service in a Unique Himalayan Cross-Cultural Setting.

Background. Whether concepts and principles of Occupational Therapy (OT) can successfully be applied to non-Western and cross-cultural settings is being intensively discussed. Aims/Objectives. We explored the changing perspectives of local occupational therapists (OTs) eight years after the implementation of an OT service in a Himalayan cross-cultural setting in terms of (1) treatment applied, (2) professional identity, and (3) cross-cultural interactions. Material and Methods. A qualitative study design was chosen, and semistructured interviews were conducted in all employed practitioners (a) during implementation and (b) eight years later (n = 7). Questions were carefully formulated in order to narrow down the intended issues but respecting crosscultural differences. The framework method was implemented for data analysis. Findings. Long-term empowering local OTs resulted in the successful development of a sustainable OT department in a unique Himalayan cross-cultural setting. Practitioners became aware of their therapeutic potentials, a clear sense of professional identity was developed, and it was recognised that sensitive cross-cultural practice is only achieved by an ongoing and intentional cultural learning process. Conclusions and Significance. Our findings suggest that OT can be applied to non-Western cross-cultural settings

Effectiveness of robot-assisted gait training in children with cerebral palsy: a bicenter, pragmatic, randomized, cross-over trial (PeLoGAIT)

Walking ability is a priority for many children with cerebral palsy (CP) and their parents when considering domains of importance regarding treatment interventions. Partial body-weight supported treadmill training has become an established therapeutic treatment approach to address this demand. Further, new robotic rehabilitation technologies have increasingly been implemented in the clinical setting to allow for longer training sessions with increased step repetitions while maintaining a consistent movement pattern. But the current evidence about its clinical effectiveness in pediatric rehabilitation is weak. The aim of this research project is therefore to investigate the effectiveness of robot-assisted gait training on improvements of functional gait parameters in children with cerebral palsy

The role of the practice order: A systematic review about contextual interference in children

Aim: We aimed to identify and evaluate the quality and evidence of the motor learning literature about intervention studies regarding the contextual interference (CI) effect (blocked vs. random practice order) in children with brain lesions and typically developing (TD) children. Method: Eight databases (Cinahl, Cochrane, Embase, PubMed, Pedro, PsycINFO, Scopus and Web of Knowledge) were searched systematically with predefined search terms. Controlled studies examining the CI effect in children (with brain lesions or TD) were included. Evidence level, conduct quality, and risk of bias were evaluated by two authors independently. A best evidence synthesis was performed. Results: Twenty-five papers evaluating TD children were included. One of these studies also assessed children with cerebral palsy. Evidence levels were I, II, or III. Conduct quality was low and the risk of bias high, due to methodological issues in the study designs or poor description thereof. Best evidence synthesis showed mainly no or conflicting evidence. Single tasks showed limited to moderate evidence supporting the CI effect in TD children. Conclusion: There is a severe limitation of good-quality evidence about the CI effect in children who practice different tasks in one session, especially in children with brain lesions

Effectiveness of physiotherapy and costs in patients with clinical signs of shoulder impingement syndrome: One-year follow-up of a randomized controlled trial

Objectives: To investigate the effect of manual physiotherapy and exercises compared with exercises alone in patients with shoulder impingement syndrome one year after inclusion. Design: Randomized controlled trial. Subjects: Patients with shoulder impingement of more than 4 weeks. Methods: The intervention group received individualized manual physiotherapy plus individualized exercises; the control group received individualized exercises only. Both groups had 10 treatments over 5 weeks; afterwards all patients continued their exercises for another 7 weeks at home. Primary outcomes were the Shoulder Pain and Disability Index and Patients' Global Impression of Change. The Generic Patient-Specific Scale was used as secondary outcome. Costs were recorded in a log-book. Results: Ninety patients were included in the study and 87 could be analyzed at 1-year follow-up. Both groups showed significant improvements in all outcome measures, but no difference was detected between the groups. Only costs differed significantly in favour of the control group (p=0.03) after 5 weeks. Conclusion: Individualized exercises resulted in lower costs than manual physiotherapy and showed a significant effect on pain and functioning within the whole group after one year. Exercises should therefore be considered as a basic treatment. Due to the progressive improvement that occurred during the follow-up period with individualized exercises further treatments should be delayed for 3 to 4 months

Responsiveness of the German version of the Neck Disability Index in chronic neck pain patients: a prospective cohort study with a seven-week follow-up

Background The need for an efficient and feasible strategy to deal with neck pain has a high priority for many countries. Validated assessment tools like the Neck Disability Index (NDI) to evaluate the functional status of a neck pain patient are urgently needed to treat and to follow-up patients purposefully. A German version (NDI-G) was shown to be valid and reliable, but has so far not been tested for responsiveness. The aim of this study was to evaluate the NDI-G`s responsiveness. Methods This was a prospective cohort study with a seven-week follow-up. Fifty chronic neck pain patients filled out NDI-G twice. Additionally, the Patients’ Global Impression of Change score (PGIC) was assessed at follow-up. Wilcoxon and Spearman tests were used to assess direction and strength of the association between the change in NDI-G and PGIC. The receiver operating characteristics method and the area under the curve (AUC) were calculated to assess sensitivity and specificity of the NDI-G change over time. Results The Wilcoxon test showed statistically significant differences for NDI-G at baseline and follow-up in the total sample, the “clinically improved” and “clinically not improved” subgroups as indicated in the PGIC. Spearman test resulted in a moderate correlation between the NDI-G and the PGIC (rS = -0.53, p = 0.01) at follow-up. AUC showed an acceptable discrimination [AUC = 0.78 (95% confidence interval 0.64 – 0.91)] of the NDI-G, with a cutoff score of 1.5, between clinically improved and clinically not improved patients, based on the PGIC. Conclusions The NDI-G is responsive to change in chronic neck pain. Together with the results of a previous study on its validity and reliability, the NDI-G can be recommended for research and clinical settings in patients with neck pain in German speaking countries