Congenital Heart Disease
Conditions
Keywords
Cardiac MRI, Congestive Heart Failure, Cardiac Fibrosis, Late Gadolinium Enhancement
Brief summary
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.
Detailed description
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.
Interventions
None listed
Sponsors
Children's National Research Institute
Suburban Hospital
National Heart, Lung, and Blood Institute (NHLBI)
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to 80 Years
Healthy volunteers
No
Inclusion criteria
* INCLUSION CRITERIA: Inclusion Criteria for All Arms of the Protocol: * Subjects 18 years of age or older * All defects that have a right ventricle that supports the systemic circulation * All defects with a functional single ventricle * Written informed consent
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Hypothesize that myocardial ischemia, as detectable by quantitative stress perfusion MRI, will predict systolic and diastolic dysfunction in subjects with single ventricle physiology and systemic right ventricles. | 5 years | 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. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| A portion of ventricular systolic or diastolic dysfunction will be predictable based on myocardial fibrosis or scarring related to the underlying pathophysiology of single ventricle physiology & systemic right ventricles or post-surgical... | 5 years | A portion of ventricular systolic or diastolic dysfunction will be predictable based on myocardial fibrosis or scarring related to the underlying pathophysiology of single ventricle physiology & systemic right ventricles or post-surgical consequences. |
| myocardial scarring/fibrosis will correlate with symptoms, NYHA functional class and BNP. | 5 years | myocardial scarring/fibrosis will correlate with symptoms, NYHA functional class and BNP. |
Countries
United States
Outcome results
None listed