Lars Gross-Wortmann, MD

Prevalence and Evolution of Late Gadolinum Enhancement and Myocardial Hypertrophy in Childhood Cardiomyopathy
The Hospital for Sick Children, Toronto, Canada
Lars Gross-Wortmann, MD – $46,918

Hypertrophic cardiomyopathy, or HCM, is a disease in which the heart muscle is abnormally thickened. HCM is among the most common reasons for dying suddenly in children and young adults. Sudden cardiac death in these patients is often the result of an abnormal heart rhythm. Unfortunately, even if a patient is known to have HCM it is very difficult to predict whether he or she is at risk of sudden death. Many patients with HCM have no warning signs before collapsing and, in some cases, dying. From experience in adults with HCM, patients with severe thickening of the heart muscle and/or those with scars in the heart muscle tend to be at higher risk for complications. Scarring and thickening can be measured with magnetic resonance imaging, or MRI. In contrast to adults, it is not known how much muscle scarring has already occurred in children. It also is not known how the scarring and thickening progress during childhood and adolescence or whether they are linked to poor outcomes. The numbers of patients who are followed at any one center are too small to answer these questions. Therefore, this study will combine MRI data from several hospitals and look at whether pediatric patients had rhythm problems, fainting or heart failure. The goal is to determine how many children with HCM have scarring of their heart muscle and how thickened their heart is. The findings from this study will inform physicians on when to start ordering MRIs in children with HCM. Ultimately, this study’s aim is to determine whether MRI is able to identify those pediatric patients who are at risk for sudden cardiac death.