MRI Assessment of RV Function: Patients With TOF or Aortic Coarctation

MRI Assessment of Two Congenital Heart Disease States: Assessment of Right Ventricular Function: Predictor of Optimal Timing of Pulmonary Valve Replacement in Patients With TOF; Assessment of Flow Dynamics in Patients With Aortic Coarctation

At Children's Healthcare of Atlanta at Egleston, it is standard of care to do a cardiac MRI on patients with the diagnosis of Tetralogy of Fallot and Aortic Coarctation to evaluate heart function. We propose to do a retrospective chart review of patient data along with their MRI data to analyze ventricular function and cavity volumes pre-Tetralogy of Fallot repair, post-Tetralogy of Fallot repair (pre-valve repair/replacement) and post-valve repair/replacement in order to assess the efficacy and optimal timing of valve replacement.

Study Overview

• Status: Completed
• Conditions: Congenital Disorders

Detailed Description

Patients with Tetralogy of Fallot have various degrees and levels of right ventricular outflow tract obstruction. They undergo surgical removal of the obstructing structures, which includes the pulmonary valve. The repair of the outflow tract is done utilizing a transannular patch. This approach, which is considered the standard of care, results in free pulmonary insufficiency, which may become severe and lead to right heart overloading and subsequent dilatation and dysfunction. This chronic overloading results in progressive tricuspid insufficiency and a suspected decline in effective pulmonary blood flow, cardiac output and right ventricle functional capabilities. Many patients develop progressive exercise intolerance, arrhythmias and severe cardiomegaly leading to dilated cardiomyopathy. The progressive decline in patient clinical status results in recommendations of pulmonary valve replacement to interrupt further dysfunction of the ventricles. Clinical improvement is generally seen among the patients and cardiac function is measurably improved in many cases, however the timing of surgery remains undefined and its impact on function improvement and recovery post -valve replacement is under serious debate and continuous discussion.

Preliminary work suggests that Magnetic Resonance Imaging (MRI) determined right to left ventricular volume ratio's of greater than 2:1 with associated tricuspid regurgitation, right ventricular regurgitant volumes of greater than 50%, and right ventricular ejection fractions less than 45% are common among patients with severe right ventricular failure and dysfunction. These patients are in need of pulmonary valve replacement and have routinely been referred for surgical intervention.

Patients with Aortic Coarctation have a localized malformation characterized by deformity of the aortic media, causing narrowing, usually severe, of the lumen of the vessel. Surgical repair or removal of this malformation is the treatment of choice however; the use of MRI to better determine the extent of damage or malformation would allow the surgeon to plan an operative strategy prior to opening the chest.

• Study Type: Observational
• Enrollment (Actual): 390

Contacts and Locations

Study Locations

• United States
  • Georgia
    • Atlanta, Georgia, United States, 30322
      • Children's Healthcare of Atlanta

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: No older than 21 years (Child, Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

The study population is Patients with the diagnosis of Tetralogy of Fallot or Aortic Coarctation being Treated at Children's Healthcare of Atlanta at Egleston

Description

Inclusion Criteria:

• Patients with the diagnosis of Tetralogy of Fallot or Aortic Coarctation being Treated at Children's Healthcare of Atlanta at Egleston.

Exclusion Criteria:

• Those patients who do not meet the inclusion criteria

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
(1) Determine Right and left ventricular volumes during all phases of the cardiac cycle.	(1) Determine Right and left ventricular volumes during all phases of the cardiac cycle.	7 years

Collaborators and Investigators

• Sponsor: Children's Healthcare of Atlanta
• Investigators: Principal Investigator: James Parks, MD, Children's Healthcare of Atlanta, Sibley Heart Center Cardiology

Study record dates

Study Major Dates

• Study Start: March 1, 2002
• Primary Completion (Actual): July 1, 2009
• Study Completion (Actual): July 1, 2009

Study Registration Dates

• First Submitted: January 13, 2006
• First Submitted That Met QC Criteria: January 13, 2006
• First Posted (Estimate): January 18, 2006

Study Record Updates

• Last Update Posted (Estimate): December 2, 2014
• Last Update Submitted That Met QC Criteria: November 26, 2014
• Last Verified: November 1, 2014

More Information

Terms related to this study

• Keywords: MRI; pediatric; cardiac; Aortic Coarctation; Tetralogy of Fallot TOF
• Additional Relevant MeSH Terms: Heart Diseases; Cardiovascular Diseases; Congenital Abnormalities; Heart Defects, Congenital; Cardiovascular Abnormalities; Aortic Coarctation
• Other Study ID Numbers: 02-122