PRecIsion Medicine in CardiomyopathY

Development of a Risk Calculator to Predict Sudden Cardiac Death in Children With Hypertrophic Cardiomyopathy

This is a retrospective cohort study of pediatric hypertrophic cardiomyopathy (HCM) patients using chart and registry review methodology. The studies objective is to develop and validate a sudden cardiac death (SCD) risk calculator that is age-appropriate for children with HCM that includes clinical and genetic factors.

Inclusion Criteria:

• phenotype-positive patients diagnosed with hypertrophic cardiomyopathy
• phenotype-negative, genotype positive patients considered at risk for developing hypertrophic cardiomyopathy

Exclusion Criteria:

• Neuromuscular, metabolic, syndromic (other than Noonan Syndrome and related RAS-opathies) or endocrine (including infants of diabetic mothers) causes of HCM
• (Other treatable causes of left ventricular hypertrophy (systemic hypertension, anatomic defects causing left ventricular outflow tract obstruction e.g. aortic stenosis, subAS, subaortic membrane, coarctation)