Myocardial Perfusion and Scarring in Congenital Heart Disease

NCT01639937 | Terminated

• 2 other identifiers: 12015812-H-0158
• Study Type: observational
• Target: 18
• Locations: 1 country
• Sites: 3

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.

Conditions

Congenital Heart Disease

Keywords

Cardiac MRI; Congestive Heart Failure; Cardiac Fibrosis; Late Gadolinium Enhancement

Outcome Measures

Primary Outcomes (1)

• 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 Outcomes (2)

• 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

• myocardial scarring/fibrosis will correlate with symptoms, NYHA functional class and BNP.

  5 years

Study Arms (1)

1

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

Eligibility Criteria

• Age: 18 Years - 80 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Participants will be enrolled at the NIH Clinical Center, Suburban Hospital, or Children s National Medical Center (CNMC). We will recruit subjects of the Washington Adult Congenital Heart program at CNMC to participate in this study.@@@@@@

You may qualify if:

• 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

You may not qualify if:

• 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:

Sponsors & Collaborators

• National Heart, Lung, and Blood Institute (NHLBI): lead
• Children's National Research Institute: collaborator
• Suburban Hospital: collaborator

Study Sites (3)

Childrens National Medical Center

Washington D.C., District of Columbia, 20010, United States

Location

Suburban Hospital

Bethesda, Maryland, 20814, United States

Location

National Institutes of Health Clinical Center, 9000 Rockville Pike

Bethesda, Maryland, 20892, United States

Location

Related Publications (3)

• Verheugt CL, Uiterwaal CS, van der Velde ET, Meijboom FJ, Pieper PG, van Dijk AP, Vliegen HW, Grobbee DE, Mulder BJ. Mortality in adult congenital heart disease. Eur Heart J. 2010 May;31(10):1220-9. doi: 10.1093/eurheartj/ehq032. Epub 2010 Mar 5.

  PMID: 20207625 BACKGROUND

• Rutledge JM, Nihill MR, Fraser CD, Smith OE, McMahon CJ, Bezold LI. Outcome of 121 patients with congenitally corrected transposition of the great arteries. Pediatr Cardiol. 2002 Mar-Apr;23(2):137-45. doi: 10.1007/s00246-001-0037-8. Epub 2002 Feb 19.

  PMID: 11889523 BACKGROUND

• Meijboom F, Szatmari A, Deckers JW, Utens EM, Roelandt JR, Bos E, Hess J. Long-term follow-up (10 to 17 years) after Mustard repair for transposition of the great arteries. J Thorac Cardiovasc Surg. 1996 Jun;111(6):1158-68. doi: 10.1016/s0022-5223(96)70217-9.

  PMID: 8642816 BACKGROUND

Related Links

• NIH Clinical Center Detailed Web Page

MeSH Terms

Conditions

Heart Defects, Congenital; Heart Failure

Condition Hierarchy (Ancestors)

Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

• Arlene Sirajuddin, M.D.

  National Heart, Lung, and Blood Institute (NHLBI)

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: NIH
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: July 11, 2012
• First Posted: July 13, 2012
• Study Start: October 24, 2012
• Primary Completion: April 1, 2017
• Study Completion: December 12, 2019
• Last Updated: December 2, 2021

Record last verified: 2021-11

Locations

US (3)