Abnormal 3-D MRI Flow Patterns in Adolescents Patients With Bicuspid Aortic Valve

NCT00412386 | Completed

• 1 other identifier: IRB00000134
• Study Type: observational
• Target: 45
• Locations: 1 country
• Sites: 1

Brief Summary

Bicuspid aortic valve (BAV) is a form of congenital heart disease (the person is born with it). With BAV, the heart valves in the aorta (the blood vessel that takes blood away from the heart to the body) are not formed right. A person with BAV has only 2 leaflets instead of three and the valve leaflets are often thickened. This can result in the block of blood flow across the valve (aortic stenosis) and/or valve leakage (aortic valve regurgitation). From our experience at least 1/3 of patients with BAV will eventually develop complications. Many patients with BAV do not develop significant problems until well into adulthood. The most common problem in BAV patients is aortic dilatation and/or dissection. At this point, we do not know on who or why aortic dilatation or dissection occurs.It is unclear whether the enlargement is because of abnormal blood flow patterns, as a result of the shape of the bicuspid valve, or whether it is because the way the aortic valve and/or vessel is formed. In other words, the abnormal shape of the aortic valve may cause blood to flow in a different way than it normally would, causing damage to the aorta as blood leaves the heart. There may be a problem with the way the aortic valve connects to the aorta, which causes the aorta to get larger or break down over time. It is also possible that the wall of the aorta in patients with BAV is weaker than it would be in patients without BAV. At this point, we do not know. It is believed by the investigators that if we can determine why the aorta gets larger or tears, we can minimize the effects or prevent them altogether. This study will collect blood and cardiac MRI images from forty-five (45) patients at Children's Healthcare of Atlanta Egleston. There will be a study group (patients with BAV) and a control group of patients (patients scheduled for a cardiac MRI but without BAV). All enrolled patients will have blood drawn by nursing staff from a peripheral vein and collected in tubes for testing the day of their MRI scan. This test is called a plasma matrix metalloproteinase level. It is believed that patients who have bicuspid aortic valves and dilated aortas have high plasma levels of this protein. This study will compare the MRI images and plasma matrix protein levels of all the patients participating in the study.

Conditions

Congenital Heart Disease; Bicuspid Aortic Valve

Keywords

Pediatric; Congenital Heart Disease; Imaging

Study Arms (2)

BAV

patients with BAV

Normal control

normal patients

Eligibility Criteria

• Age: 10 Years - 18 Years
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Child (0-17), Adult (18-64)
• Sampling Method: Non-Probability Sample

Study Population

patients with bicuspid Aortic valve, normal healthy volunteers, and congential heart disease without bicuspid aortic valve

You may qualify if:

• diagnosed with Bicuspid Aortic Valve 15 with aortic root dilatation 15 without aortic root dilatation
• patients with congenital heart disease, but not bicuspid aortic valve
• meet eligibility criteria for MRI
• years of age

You may not qualify if:

• under 10 years and over 19 years of age
• systemic hypertension for age and height
• Marfan syndrome
• on cardiac or vasoactive medications
• contra-indications to MRI such as metallic implants
• acquired heart disease
• require sedation

Sponsors & Collaborators

• Emory University: lead
• Children's Healthcare of Atlanta: collaborator

Study Sites (1)

Childrens Healthcare of Atlanta

Atlanta, Georgia, 30322, United States

Location

Related Publications (4)

• Robicsek F, Thubrikar MJ, Cook JW, Fowler B. The congenitally bicuspid aortic valve: how does it function? Why does it fail? Ann Thorac Surg. 2004 Jan;77(1):177-85. doi: 10.1016/s0003-4975(03)01249-9.

  PMID: 14726058 BACKGROUND

• Fernandes SM, Sanders SP, Khairy P, Jenkins KJ, Gauvreau K, Lang P, Simonds H, Colan SD. Morphology of bicuspid aortic valve in children and adolescents. J Am Coll Cardiol. 2004 Oct 19;44(8):1648-51. doi: 10.1016/j.jacc.2004.05.063.

  PMID: 15489098 BACKGROUND

• Novaro GM, Tiong IY, Pearce GL, Grimm RA, Smedira N, Griffin BP. Features and predictors of ascending aortic dilatation in association with a congenital bicuspid aortic valve. Am J Cardiol. 2003 Jul 1;92(1):99-101. doi: 10.1016/s0002-9149(03)00480-6. No abstract available.

  PMID: 12842261 BACKGROUND

• Karakaya O, Barutcu I, Esen AM, Dogan S, Saglam M, Karapinar H, Akgun T, Karavelioglu Y, Esen O, Ozdemir N, Turkmen S, Kaymaz C. Relationship between circulating plasma matrix metalloproteinase-9 (gelatinase-B) concentration and aortic root dilatation. Am J Hypertens. 2006 Apr;19(4):361-5. doi: 10.1016/j.amjhyper.2005.08.013.

  PMID: 16580570 BACKGROUND

MeSH Terms

Conditions

Heart Defects, Congenital; Bicuspid Aortic Valve Disease

Condition Hierarchy (Ancestors)

Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Aortic Valve Disease; Heart Valve Diseases

Study Officials

• Denver Sallee, MD

  Emory University

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Assistant Professor

Study Record Dates

• First Submitted: December 14, 2006
• First Posted: December 18, 2006
• Study Start: December 1, 2006
• Study Completion: March 1, 2012
• Last Updated: December 3, 2013

Record last verified: 2013-12

Locations

US (1)