Optimal imaging strategy for surveillance in children with hypertrophic cardiomyopathy

Background: Hypertrophic cardiomyopathy (HCM) is a diverse disease with a wide spectrum of presentations and clinical course. Sudden cardiac death remains the most visible and high-profile complication of HCM, and in many studies, it is the most common cause of SCD in high school and young adult athletes. Cardiovascular imaging plays a key role in initial diagnosis, phenotypic characterization, and baseline risk stratification of patients. Aim of review: To review the various considerations in developing the optimal non-invasive imaging strategy in children with HCM, including the pros and cons of transthoracic echocardiography and cardiac magnetic resonance imaging. To describe the newer techniques with advanced imaging that will improve our ability to discriminate higher risk children with HCM. Key scientific concepts of review: Transthoracic echocardiography remains widely available and does not require sedation in the pediatric population. A comprehensive echocardiography examination can establish the diagnosis, determine the hypertrophy pattern, evaluate left ventricular systolic and diastolic function, mitral valve function, and evaluate for the presence and severity of left ventricular outflow tract obstruction and concomitant mitral regurgitation. While CMR has traditionally played a complementary or supportive role to echocardiography, CMR has the ability to characterize the composition of the myocardium. Myocardial scarring can be visualized and quantified by CMR using a technique known as late gadolinium enhancement. Late gadolinium enhancement has emerged as an important risk factor in predicting outcomes. Accessibility and need for sedation remain barriers to expanded use in the pediatric population.