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Published on: December 31, 2017
Neurological Injury and Cerebral Blood Flow in Single Ventricles Throughout Staged Surgical Reconstruction
Mark A Fogel1, Christine Li2, Okan U Elci2
1From Division of Cardiology, Department of Pediatrics (M.A.F., C.L., T.P., F.W., M.A.H., K.K.W.), Department of Radiology (M.A.F., M.S.K., M.A.H., K.K.W., A.V.), Department of Anesthesiology and Critical Care Medicine (S.C.N., L.M.M., L.D.), Division of Cardiothoracic Surgery, Department of Surgery (T.L.S., J.W.G., S.F., C.M.), and Department of Neurology (P.J.S., D.J.L.), The Children's Hospital of Philadelphia/Perelman School of Medicine, University of Pennsylvania, Philadelphia; and Westat, Rockville, MD (O.U.E., J.B.). fogel@email.chop.edu.
Insights
Brain abnormalities are common in single ventricle patients, increasing with surgical stages. Higher cerebral blood flow is linked to reduced brain injury risk, but oxygen delivery and CO2 reactivity are not associated.
Area of Science:
- Pediatric Cardiology
- Neurodevelopmental Outcomes
- Congenital Heart Disease
Background:
- Patients with single ventricle physiology face high risks of brain injury and neurodevelopmental issues.
- The incidence and progression of brain abnormalities during surgical reconstruction remain unclear.
- The relationship between brain abnormalities and physiological factors like cerebral blood flow is not well understood.
Purpose of the Study:
- To investigate the incidence of brain abnormalities in single ventricle patients throughout surgical reconstruction.
- To determine the association between brain abnormalities and cerebral blood flow, oxygen delivery, and carbon dioxide reactivity.
- To track changes in brain abnormalities across different surgical stages.
Main Methods:
- Magnetic resonance imaging (MRI) scans were performed on single ventricle patients at three time points: pre-bidirectional Glenn (pre-BDG), before Fontan completion (BDG), and 3-9 months post-Fontan.
- A total of 235 scans were analyzed from 168 subjects.
- Brain abnormalities, including ischemic white matter changes, focal tissue loss, and ventriculomegaly, were assessed.
Main Results:
- Brain abnormalities were more common in the BDG and post-Fontan stages compared to the pre-BDG stage (P<0.05).
- Cerebral blood flow was significantly higher in BDG patients than in Fontan patients.
- Increased cerebral blood flow was associated with a reduced likelihood of brain injury (63%-75% decrease).
Conclusions:
- Significant brain abnormalities are prevalent in single ventricle patients and increase with surgical reconstruction.
- Higher cerebral blood flow is inversely associated with brain lesions in these patients.
- Oxygen delivery and carbon dioxide reactivity were generally not associated with neurological injury, with limited exceptions.
Background:
Patients with a single ventricle experience a high rate of brain injury and adverse neurodevelopmental outcome; however, the incidence of brain abnormalities throughout surgical reconstruction and their relationship with cerebral blood flow, oxygen delivery, and carbon dioxide reactivity remain unknown.
Methods:
Patients with a single ventricle were studied with magnetic resonance imaging scans immediately prior to bidirectional Glenn (pre-BDG), before Fontan (BDG), and then 3 to 9 months after Fontan reconstruction.
Results:
One hundred sixty-eight consecutive subjects recruited into the project underwent 235 scans: 63 pre-BDG (mean age, 4.8±1.7 months), 118 BDG (2.9±1.4 years), and 54 after Fontan (2.4±1.0 years). Nonacute ischemic white matter changes on T2-weighted imaging, focal tissue loss, and ventriculomegaly were all more commonly detected in BDG and Fontan compared with pre-BDG patients (P<0.05). BDG patients had significantly higher cerebral blood flow than did Fontan patients. The odds of discovering brain injury with adjustment for surgical stage as well as ≥2 coexisting lesions within a patient decreased (63%-75% and 44%, respectively) with increasing amount of cerebral blood flow (P<0.05). In general, there was no association of oxygen delivery (except for ventriculomegaly in the BDG group) or carbon dioxide reactivity with neurological injury.
Conclusions:
Significant brain abnormalities are commonly present in patients with a single ventricle, and detection of these lesions increases as children progress through staged surgical reconstruction, with multiple coexisting lesions more common earlier than later. In addition, this study demonstrated that BDG patients had greater cerebral blood flow than did Fontan patients and that an inverse association exists of various indexes of cerebral blood flow with these brain lesions. However, CO2 reactivity and oxygen delivery (with 1 exception) were not associated with brain lesion development.
Clinical Trial Registration:
URL: http://www.clinicaltrials.gov. Unique identifier: NCT02135081.

