Neurological Injury and Cerebral Blood Flow in Single Ventricles Throughout Staged Surgical Reconstruction

Abstract

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.

Keywords: Fontan procedure; cerebral infarction; cerebrovascular circulation; heart ventricles; magnetic resonance imaging.

© 2016 American Heart Association, Inc.

Figures

Figure 1. Breakdown of single ventricle patients studied by stage of surgery

Numbers on the right represent the number of patients with multiple scans and those at the bottom represent those with a single scan only.

Figure 2. Example of acute ischemia on diffusion imaging and cerebral veins on susceptibility weighted images (SWI)

ADC=apparent diffusion coefficient, DWI=diffusion weighted imaging.

Figure 3. Example of generalized atrophy and ventriculomegaly

Figure 4. Example of intraventricular hemorrhage on susceptibility weighted images (SWI), non-acute ischemic changes, punctate microbleeds on SWI and punctate periventricular leukomalacia (PVL)

Figure 5. Example of choroid plexus susceptibility and focal tissue loss and atrophy

Figure 6. Plot of cerebral blood flow / body surface area (CBF/BSA) (A) and pCASL (B) in patients with serial MRIs. There were 24, 57 and 43 scans in patients at the pre-BDG, BDG and Fontan stage respectively for a total of 124 scans in 60 patients. See figure 1 and table 6 for further delineation of patients with serial scans

Figure 6. Plot of cerebral blood flow / body surface area (CBF/BSA) (A) and pCASL (B) in patients with serial MRIs. There were 24, 57 and 43 scans in patients at the pre-BDG, BDG and Fontan stage respectively for a total of 124 scans in 60 patients. See figure 1 and table 6 for further delineation of patients with serial scans