The challenge of predicting cardiac arrhythmias Who is at risk of sudden cardiac death?

Part I – Risk stratification for primary prevention ICD therapy
The declining rates of appropriate ICD therapy over the years, along with lower sudden death incidence in recent studies such as the DANISH study, prompt a reevaluation of primary prevention strategies. Chapter 2 introduced a meta-analysis on the benefits of primary prevention ICD therapy in non-ischemic cardiomyopathy. Chapter 3 introduces prediction models for mortality and ICD therapy, revealing persistent notable event rates despite medical advancements. While the mortality model shows better predictive ability, both models face challenges in accurately stratifying risk. In Chapter 4 complications associated with ICD implantation are discussed.
Part II of the thesis shifts focus to risk stratification in phospholamban (PLN) p.Arg14del variant carriers.
In chapter 5 the age-related penetrance of PLN p.Arg14del cardiomyopathy is discussed. PLN p.Arg14del carriers exhibit high penetrance of major cardiac events, reaching up to 70% affected at age 70. Chapter 6 presents a novel prediction model for sustained ventricular arrhythmia, outperforming existing models in discerning individual risk. An online tool aids clinicians in decision-making regarding ICD implantation.
Overall, the thesis underscores the challenges and importance of accurate risk stratification in primary prevention strategies, especially in diverse patient populations like PLN p.Arg14del carriers, and offers insights into improving prediction models for better clinical outcomes.