Abstract 14023: Myocardial Perfusion Abnormalities: A Potential Biomarker for Ventricular Arrhythmias in Hypertrophic Cardiomyopathy Young Patients?

• DOI: 10.1161/circ.146.suppl_1.14023
• Publication Date: 2023-06-15T16:25:16Z
• Authors: Philippe Chevalier; Antoine Deliniere; Gilles MILLAT; Babken Asatryan; Alexandre Janin; Francis BESSIERE; Clement Boiteux; Christelle Haddad
• Keywords: Sudden Death; Sotalol; Sudden cardiac arrest

Introduction: Despite progress in the field, risk stratification for sudden death patients with hypertrophic cardiomyopathy (HCM) remains challenging. Although ventricular arrhythmias (VA) account most of these events, mechanisms responsible cardiac arrest HCM are not understood. Hypothesis: Abnormalities myocardial perfusion may create an arrhythmogenic substrate patients. Methods/ Results: A 12-year-old boy was admitted to our department after out-of-hospital arrest, documented fibrillation while running. non-obstructive asymmetric diagnosed echocardiography (max LV wall thickness 32 mm). He had one conventional factor SCD. Genetic testing revealed a heterozygous state MYH7 variation: p.Glu500Ala (NM_000257.3:c.1499A>C). This variation classified as “probably pathogenic” (class 4 according ACMG). His father and his 15-year-old brother were same variant asymptomatic. The displayed similar phenotype. patient received implantable defibrillator suffered from appropriate exercise-related shocks within 6-months despite sotalol therapy (160 mg bid). Because VA occurred always when engaged vigorous physical activity beta blocker since he could refrain activity, thallium-201 scintigraphy performed. To further understand phenotype, underwent investigation. Significant uptake defects both initial delayed images evident proband (15%) but asymptomatic brother. Treatment antianginal drug (amlodipine 5 mg/day) initiated. With 10 mg/day amlodipine associated significant improvement (6% versus 15% defect). At follow-up 12 months, there no recurrence VA. Conclusions: Regarding patients, present report 1) strengthens hypothesis that identify at VA, 2) suggests tailored anti-ischemic prescription be useful, 3) underscores need prospective studies quantifying ischemia exercise-induced VF.