क्लिनिकल एवं प्रायोगिक कार्डियोलॉजी
खुला एक्सेस

अमूर्त

Where do we Stand in the Treatment of HCM in Children? The Role of Disopyramide and New Medications on the Horizon

Karin Del Vecchio*, Alice Pozza, Giovanni Di Salvo

Pediatric Hypertrophic Cardiomyopathy (HCM) has a wide range of clinical manifestations. Left Ventricular Outflow Tract Obstruction (LVOTO) at rest is present in up to one-third of children with HCM, with a further 50%-60% of symptomatic children developing a gradient under exertion. Treatment options are limited and there is a relative lack of data on the pediatric population. Disopyramide is a sodium channel blocker with negative inotropic properties. This therapy effectively reduces LVOTO in adults with HCM and delays surgical interventions, but it is not licensed for use in children. This study aimed to review and analyze the influence of disopyramide over the pathophysiological, clinical, electrocardiographic and echocardiographic characteristics of patients with HCM in infancy, childhood, adolescence and adult age. While disopyramide remains a basis in the management of pediatric HCM, the advent of mavacamten and aficamten heralds a new era of potential advancements. These emerging therapies could significantly improve the quality of life and prognosis for young patients with HCM.