https://doi.org/10.4081/monaldi.2024.3084
How effective is disopyramide in treating pediatric hypertrophic cardiomyopathy? State of the art and future directions
Submitted: June 16, 2024
Accepted: August 3, 2024
Published: September 19, 2024
Accepted: August 3, 2024
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All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Authors
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. We 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 cornerstone 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.
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How to Cite
Del Vecchio, Karin, Caterina Rizzardi, Alice Pozza, Francesco Prati, Luisa Ye, Alessia Fattoretto, Elena Reffo, and Giovanni Di Salvo. 2024. “How Effective Is Disopyramide in Treating Pediatric Hypertrophic Cardiomyopathy? State of the Art and Future Directions”. Monaldi Archives for Chest Disease, September. https://doi.org/10.4081/monaldi.2024.3084.
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