دانلود رایگان مقاله انگلیسی رویکرد مدیریت ریسک پویا برای کاهش آسیب در کاردیومیوپاتی هیپرتروفی - الزویر 2018

ABSTRACT

Hypertrophic cardiomyopathy (HCM) is thought to be a leading cause of sudden cardiac death (SCD) in athletes, and while SCD is the most dramatic and feared of all HCM presentations, its exact incidence remains unclear. Current expert opinion and consensus panels that formulated exercise recommendations in HCM to reduce the risk of sudden death by avoiding competitive sport are based on scant, observational, often circumstantial, and sometimes conflicting evidence. These recommendations rely on multiple cross-referencing of few original papers from a limited number of research groups. At the same time, there is accumulating data that recommendations to avoid competitive exercise in HCM come at the price of avoidance of all physical activity which carries its own risks and complications. Consequently, physicians are challenged when asked by concerned parents and children to justify overly restrictive clinical judgements and guidance about permitted exercise levels in HCM. In this manuscript, we review the strength of the evidence underlying current sport recommendations in HCM. We propose that developing a working risk management approach to assist anxious parents and children is imperative and must be customized to the needs of the child and their parents. Rather than a blanket recommendation to avoid competitive sport, we believe that HCM patients deserve to have a robust and real-world risk assessment strategy that is tailored to the individual needs, discussed with the child and their parents, and updated as the child grows and matures.

3. Concluding Thoughts and Recommendations

HCM is thought to be a leading cause of SCD in athletes, but the exact incidence of SCD in HCM remains unclear. Any single measure remains unlikely to provide enough information to holistically evaluate predictive model performance for rare SCD events. Though perfect prospective discrimination remains an unattainable goal, incremental improvements in how we identify children at risk may still advance us towards a more personalized medicine approach for children with HCM. The search for causes of SCD is pervasive but when applied to understanding biological behavior in response to changes in the daily life of children with HCM, it tends to ignore the complexity and produces a reductionist approach of universal explanations independent of the context in which the risk emerges [8]. Developing a working risk management approach to assist anxious parents and children is imperative and must be customized to the needs of the child and their parents. The incidence of SCD is probably low, and is estimated at less than 1% per year. The overall risk of SCD, considering the incidence of SCD among athletes and prevalence of HCM in the general population, in actively competing HCM athletes, is likely less than 0.1% per year [1]. It is hard to support firm recommendations given that the evidence for avoidance of competitive sport is based on a very limited number of observational studies/registries (with variable quality data sources) on causes of death and a change in prevalence of SCD after implementation of screening. There are conflicting data and methods, supportive data are deductive, open to interpretation and largely based on the comparison of SCD prevalence between widely different eras or between different geographic regions with different cultures, genetic profiles and clinical practices. Professionally and in the lay culture, HCM is thought, although rare, to be a leading cause of SCD in athletes with recommendations to avoid competitive exercise. Physicians are challenged when asked by concerned parents and children to justify seemingly overly restrictive clinical recommendations and to provide guidance about what exercise should be permitted in children with HCM [41]. On the one hand, despite the weak evidence and retrospective data, strenuous exercise may be deleterious to children with HCM, and is thought to increase the risk of life-threatening arrhythmias and syncope.