رویکرد مدیریت ریسک پویا برای کاهش آسیب در کاردیومیوپاتی هیپرتروفی A dynamic risk management approach to reduce harm in hypertrophic cardiomyopathy

1. Risk Management Approach Towards HCM When parents of children with HCM seek medical care, they entrust their health and wellness to us. While life expectancy is generally good in hypertrophic cardiomyopathy (HCM), premature mortality can occur unexpectedly via three modes: heart failure, stroke and sudden cardiac death (SCD). Of these, SCD is the most dramatic and feared of all presentations, especially as it occurs seemingly without warning in young, healthy appearing, and athletic individuals. The response to these rare events is often further fueled by sensationalist media attention. The hypothesized mechanisms underlying SCD in HCM are diverse and powerful, including arrhythmia, ischemia and hemodynamic mechanisms, but none are specific and all are unproven [1]. So, have we really made significant progress in better understanding the ‘interlocking factors’ that lead to SCD in HCM? Traditionally, risk has been seen as exposure to potentially injurious events that may threaten or damage the individual or an organization [2]. The variability in risk tolerance by patients and clinicians in medicine is complicated and not well understood. There is a pervasive and troubling belief among advocates of the patient safety movement that all adverse events in a health system are discoverable and preventable. The belief is primarily that having more information at hand will be sufficient to improve health systems and prevent all risk from leading to harmful outcomes [3]. Fortunately, more sensitive analyses based on expertise in accident investigation acknowledges that ‘adverse events should be characterized as emergent properties of complex systems, and they cannot always be predicted’ [4]. Perhaps our ‘explanatory hypotheses’ need revision in HCM in order to take into consideration our evolving knowledge about the role of risk mitigation and help to make better observations of SCD and how best to prevent these extraordinary outcomes? Our central hypothesis proposed by the paper is that the affordances of the environment of children with HCM and the thinking it entails resists reduction to stable and standardized risk identification and management methods. The historical context of the everyday experiences of clinicians treating HCM with children and their parents is not adequately captured by statistical measures employed in evidence-based medicine. The clinical experience with HCM is more nuanced and dynamic than the simple cause and effect sequences constructed in observational studies and investigations. This situation warrants a co-production care model [5] which entails open and frank discussions with parents and other clinicians in order to identify more effective models of inquiry and a more robust risk management and mitigation approach to help prevent SCD in these patients [6]. Developing an understanding of the unexpected events in the lives, of children with HCM and their parents, requires a different approach, sets of tools and mental models [7]. We explicitly define the “rare events problem” as a situation where only a small proportion of patients are at comparatively “high risk” of experiencing an event. How can we identify these children at risk? We hasten to reinforce a conceptual distinction: the goal of risk classification is not predicting precisely who will live or die. Rather, the goal is identification of a small subset of high-risk patients.