Clinical Trial: Cerebral Anatomy, Hemodynamics and Metabolism
Study Status: Recruiting
Recruit Status: Recruiting
Study Type: Observational
Official Title: Cerebral Anatomy, Hemodynamics and Metabolism In Single Ventricles: Relationship to Neurodevelopment
Brief Summary: Single ventricle lesions are the leading cause of illness and death from congenital heart disease. The modified Fontan Operation is the corrective surgery for these lesions. The operation is done in stages over a few years and children who complete the operation are known to have greater neurodevelopmental (ND) deficits than the general population. The purpose of this study is to understand how blood flow to the brain (CBF) and brain lesions relate to ND outcome, as well as how CMRO2 relates to anatomic brain lesions. These relationships will be studied through Magnetic Resonance Imaging (MRI) and ND Testing.
Detailed Summary:
Single ventricle (SV) lesions are the leading cause of morbidity and mortality from congenital heart disease (CHD) in the United States. The definitive palliative surgery is the modified Fontan operation where systemic venous return is routed directly to the pulmonary arteries. The surgical reconstruction is performed in stages over a few years which includes the "Stage I" and hemiFontan or bidirectional Glenn operations. These children are known to have greater neurodevelopmental (ND) deficits than the general population and other forms of CHD. For example, a study at Children's Hospital of Philadelphia revealed that at 9 years old, 1/3 were receiving some form of special education; the median intelligence quotient (IQ) was 86 with mental retardation in 18%. One component to ultimate ND outcome is cerebral blood flow (CBF). Preliminary data in SV in the literature across all age ranges and multiple disease states, suggests that CBF is related to ND; a recent review of 25 studies bears this out. Another component to ND outcome is anatomic brain lesions. Preliminary data from a current NIH study of CBF study suggests a link between CBF and brain lesions (decreased CBF is associated with more brain lesions), weaving a complex interaction leading to ultimate ND outcome. There is a pressing need to understand CBF and brain lesions as it relates to childhood ND; this rapid growth stage may be especially important to ultimate cognitive function having not only a humanistic/social impact but a large economic one as well.
Data from a previous NIH grant which ended November 2014 indicates that CBF in SV patients changes throughout the staged surgeries and in the first 2 stages, under stressed conditions such as hypercarbia; in addition, initial look at the data suggests a difference in brain abnormalities as well. These children are especially at risk for altered CBF and
Sponsor: Children's Hospital of Philadelphia
Current Primary Outcome: Relationship of CBF and Brain Abnormalities to ND Outcomes [ Time Frame: Up to 10 years ]
Original Primary Outcome: Same as current
Current Secondary Outcome: Relationship of CMRO2 to ND outcome [ Time Frame: Up to 10 years ]
Original Secondary Outcome: Same as current
Information By: Children's Hospital of Philadelphia
Dates:
Date Received: August 2, 2016
Date Started: April 2016
Date Completion: June 2019
Last Updated: September 28, 2016
Last Verified: September 2016