Although most cases of HCM have a benign prognosis, the annual rate of SCD is 0.5-1%, particularly among young and asymptomatic individuals.
This case highlights that in young patients with hypertrophic cardiomyopathy and a strong family history of sudden cardiac death, expert clinical judgment may override a low calculated HCM Risk-SCD score to recommend ICD implantation.
Hypertrophic cardiomyopathy is defined by the presence of increased thickness of the left ventricular wall, which cannot be explained only by abnormal loading conditions. This definition applies to children and adults and makes no "a priori "assumptions about etiology or myocardial pathology. Cellular architecture disorders, interstitial fibrosis, microvascular infarctions, play a role in the emergence of electrical instability, malignant ventricular arrhythmias that cause Sudden Cardiac Death in patients with HCM. Although most cases have a benign prognosis, identifying patients at a risk for Sudden Cardiac Death, requiring prophylactic therapy with ICD, is crucial and prioritized. With increasing awareness of the disease, lower-risk patients are now more frequently diagnosed, and more recent studies show that the annual Sudden Cardiac Death rate 0.5-1%, was not negligible, unfortunately among young and asymptomatic individuals. This paper presents a case of HCM, accidentally detected in a young adult, completed with ICD implantation in primary Sudden Cardiac Death prevention.
Delić et al. (2020) studied Hypertrophic Cardiomyopathy (n=1). ICD (Implantable Cardioverter Defibrillator) was evaluated on Annual rate of Sudden Cardiac Death (SCD). Although most cases of HCM have a benign prognosis, the annual rate of SCD is 0.5-1%, particularly among young and asymptomatic individuals.
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