Myocardial Perfusion and Scarring in Congenital Heart Disease

Study Overview
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Brief Summary
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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
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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.

Official Title
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Quantitative Myocardial Perfusion, Myocardial Scarring and Their Contribution to Late Clinical Decompensation in Adults With Congenital Heart Disease

Conditions
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Congenital Heart Disease

Participation Criteria
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Eligibility Criteria
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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 EXCLUSION CRITERIA: Exclusion Criteria for All Arms of the Protocol: Subjects with a contraindication to MRI scanning will be excluded. These contraindications include subjects with the following devices: Central nervous system aneurysm clips Implanted neural stimulator Implanted cardiac pacemaker or defibrillator Cochlear implant Ocular foreign body (e.g. metal shavings) Implanted Insulin pump Metal shrapnel or bullet Severe heart damage that makes it difficult to breathe while lying flat Pregnant women (Women of childbearing potential who are uncertain as to whether they are pregnant will be required to have a screening urine or blood pregnancy test) Subjects with active symptoms of myocardial ischemia occurring despite maximally tolerated doses of oral antianginal therapy and intravenous nitroglycerin Furthermore, the following subject groups will be excluded from studies involving the administration of MRI contrast agents: lactating women unless they are willing to discard breast milk for 24 hours after receiving gadolinium renal disease (estimated glomerular filtration rate [eGFR] < 30 ml/min/1.73 m2 body surface area) The eGFR will be used to estimate renal function if reported by the laboratory. Otherwise, estimated glomerular filtration rate (eGFR) can be based on the Modification of Diet in Renal Disease (MDRD) study equation (see below) in subjects with stable renal function. This formula is not applicable to subjects with acute renal insufficiency: eGFR (ml/min/1.73 m2) = 175 x (serum creatinine)-1.154 x (age)-0.203 x 0.742 (if the subject is female) x 1.212 (if the subject is black) Additional Exclusion Criteria for Vasodilator Stress MRI: Myocardial infarction within 24 hours Uncontrolled heart failure Uncontrolled asthma or emphysema Ventricular arrhythmia (sustained ventricular arrhythmia at the time of MR scan) Second degree heart block or higher

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 80 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| 1<br>Subjects with palliated congenital heart disease including, but not limited to, d TGA, ccTGA, single ventricles, hypoplastic left heart syndrome and tricuspid atresia will be recruited | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| 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. | 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. | 5 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| 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 &amp; systemic right ventricles or post-surgical... | 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 &amp; systemic right ventricles or post-surgical consequences. | 5 years | 
| myocardial scarring/fibrosis will correlate with symptoms, NYHA functional class and BNP. | myocardial scarring/fibrosis will correlate with symptoms, NYHA functional class and BNP. | 5 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Cardiac MRI, Congestive Heart Failure, Cardiac Fibrosis, Late Gadolinium Enhancement