Myocardial Perfusion and Scarring in Adults With Congenital Heart Disease

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

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.

Study Overview

• Status: Completed
• Conditions: Cardiac Disease

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. The investigators 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.

• Study Type: Observational
• Enrollment (Actual): 18

Contacts and Locations

Study Locations

• United States
  • District of Columbia
    • Washington, District of Columbia, United States, 20010
      • Children's National Health System

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Primary care clinic

Description

Inclusion Criteria:

• 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:

• Subjects with a contraindication to magnetic resonance imaging (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

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Myocardial Ischemia measuring coronary perfusion reserve.	Compare quantitative myocardial perfusion at stress and rest with measures of systolic and diastolic function in exams performed in close temporal proximity.	5 years
Myocardial Ischemia measuring ejection fraction.	Compare the extent of myocardial ischemia through ejection fraction measurement of the systemic ventricle.	5 years
Myocardial Ischemia measuring heart inflow Doppler.	Compare heart inflow Doppler of the systemic atrioventricular valve to understand the extent of myocardial ischemia.	5 years

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Scarring and fibrosis by measuring Late Gadolinium Enhancement results.	Directly compare the extent of myocardial scarring/fibrosis with echo-derived measures of systolic and diastolic function.	5 years
Scarring and fibrosis by measuring systolic and diastolic function.	Compare the extent of myocardial scarring/fibrosis with echo-derived measures of systolic and diastolic function.	5 years
Comparison of MRI measurements with blood test.	Number of participants with predictable future heart failure symptom will be assessed by comparing all cardiac MRI exam measurements with blood test.	5 years
Comparison of MRI measurements with walk test.	Number of participants with predictable future heart failure symptom will be assessed by comparing all cardiac MRI exam measurements with walk test.	5 years
Comparison of MRI measurements with NY Heart Association class	Number of participants with predictable future heart failure symptom will be assessed by comparing all cardiac MRI exam measurements with NY Heart Association class.	5 years

Collaborators and Investigators

• Sponsor: Children's National Research Institute
• Collaborators: National Institutes of Health (NIH)
• Investigators: Principal Investigator: Laura Olivieri, MD, Children's National Health Systems

Publications and helpful links

General Publications

• 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.
• 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.
• 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.

Study record dates

Study Major Dates

• Study Start: July 1, 2013
• Primary Completion (Actual): January 18, 2020
• Study Completion (Actual): January 18, 2020

Study Registration Dates

• First Submitted: July 12, 2016
• First Submitted That Met QC Criteria: August 5, 2016
• First Posted (Estimate): August 10, 2016

Study Record Updates

• Last Update Posted (Actual): March 5, 2021
• Last Update Submitted That Met QC Criteria: March 4, 2021
• Last Verified: March 1, 2021

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Cardiovascular Diseases; Congenital Abnormalities; Cardiovascular Abnormalities; Heart Diseases; Heart Defects, Congenital
• Other Study ID Numbers: Pro00003067; contract (Other Identifier: NIH)

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: YES
• IPD Plan Description: Results of MRI scans will be shared with the primary cardiologist.
• IPD Sharing Time Frame: MRI report becomes available once the scan has been assessed, usually within 24 hours. It will be available indefinitely.
• IPD Sharing Access Criteria: Primary care takers will have access to this report.
• IPD Sharing Supporting Information Type: CSR