Background Single ventricle patients experience a high rate of brain injury and adverse neurodevelopmental outcome, however, the incidence of brain abnormalities throughout surgical reconstruction and its relationship with cerebral blood flow, oxygen delivery and carbon dioxide reactivity remains unknown. Methods Single ventricle patients were studied with MRI scans immediately prior to bidirectional Glenn (pre-BDG), prior to Fontan and then 3–9 months after Fontan reconstruction. Results One hundred and 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). Non-acute ischemic white matter changes on T2 weighted imaging, focal tissue loss, and ventriculomegaly were all more commonly detected in BDG and Fontans compared to pre-BDG (P<0.05). BDG patients has significantly higher CBF than Fontan patients. The odds of discovering brain injury adjusting for surgical stage as well as 2 or more co-existing lesions within a patient all 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 (with the exception of ventriculomegaly in the BDG group) or carbon dioxide reactivity with neurological injury. Conclusion Significant brain abnormalities are commonly present in single ventricle patients and detection of these lesions increase as children progress through staged surgical reconstruction with multiple co-existing lesions more common earlier than later. In addition, this study demonstrated that BDG patients had greater CBF than Fontan patients and that there exists an inverse association of various indices of CBF with these brain lesions, however, CO2 reactivity, oxygen delivery (with one exception) were not associated with brain lesion development.
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