Medicine & Dentistry-Pediatrics
University of Alberta
Summary of research:
Hypertrophic cardiomyopathy (HCM) is a genetic condition which causes severe, progressive thickening of the heart muscle. The muscle thickening can cause obstruction of blood flow to the body, and symptoms of heart failure such as shortness of breath on exercise. It is also the most common cause of sudden death in children and young adults which occurs secondary to a serious irregular heart rhythm problem. Genetic testing is not yet available for family members of an affected person. The diagnosis of is made by echocardiography which works by taking ultrasound pictures of the heart and detecting thickened heart muscle. New techniques to look at the strain on the heart muscle and how the heart twists are emerging as more sensitive methods in the evaluation of HCM. However, echocardiography can miss some parts of the heart which may be affected and has some technical limitations. Cardiac magnetic resonance imaging (CMRI) has been shown to have excellent agreement with measurements made by echocardiography, providing high-resolution images to detect muscle thickening involving any region of the heart. After injection of a contrast agent called gadolinium, areas of scarring in the heart muscle are seen as bright spots on CMRI. It is thought that these regions of scarring may produce a focus for life-threatening irregular heart rhythm disturbance. The purpose of this study is to evaluate the use of these newer echocardiographic and CMRI techniques in children with HCM.