Myocardial Perfusion and Scarring in Congenital Heart Disease

Background: \- People with congenital heart disease may develop heart failure earlier that those who do not have the disease. One theory to explain this is that the heart s own blood supply may be different in people with congenital heart disease. Problems with this blood supply can severely damage the heart. This damage can be studied with a heart imaging test called a cardiac magnetic resonance imaging (MRI) scan. Researchers want to use this type of scan to look at the blood supply to the heart in people with congenital heart disease. Objectives: \- To learn more about the blood supply to the heart in people with congenital heart disease. Eligibility: \- Individuals at least 18 years of age who have heart defects caused by congenital heart disease. Design: * Participants will be screened with a physical exam and medical history. Blood and urine samples will be collected. * Participants will have a cardiac MRI scan to look at the blood flow to the heart. * Participants will also have a heart stress test to measure heart function during exercise. * Other imaging studies of the heart may be performed to collect more information on heart function.

This is a study of the perfusion of the myocardium in adults with specific forms of repaired congenital heart disease using established cardiac MRI techniques and correlating perfusion with clinical outcomes. Our objectives are to examine myocardial perfusion both during stress and at rest in adults with repaired or palliated congenital heart disease as well as quantify ventricular function, regional myocardial strain and evidence of myocardial fibrosis with quantitative measures of myocardial perfusion. The specific aim of this study is to understand whether clinical subendocardial perfusion defects contribute to the late decompensation of adult subjects that have single ventricle physiology and adult subjects that have a systemic right ventricle.