Imaging brain vascular function in Cystic Fibrosis: an MRI study of cerebral blood flow and brain oxygenation

Background Cystic fibrosis (CF) is a progressive inherited disorder that primarily affects the lungs. With recent breakthroughs in effective treatments for CF that increase life-expectancy, a higher prevalence of age-related comorbidities has been reported including cardiovascular disease, stroke and cognitive decline. Despite the known relationship between cardiovascular health and cerebrovascular function, very little is known about brain blood flow and oxygen metabolism in people with CF (PwCF). Methods In 14 PwCF and 56 healthy age / sex matched controls, we used pseudo-continuous arterial spin labelling (pCASL) to quantify cerebral perfusion in grey-matter and T2-Relaxation-Under-Spin-Tagging (TRUST) to estimate global oxygen extraction fraction (OEF) and cerebral metabolic rate of oxygen consumption (CMRO2). Results Compared to healthy controls, PwCF showed elevated CMRO2 ( p = 0.015). There were no significant between-group differences in grey-matter CBF ( p = 0.342), or whole brain OEF ( p = 0.091). However, regional analysis showed certain areas with higher CBF in PwCF ( p < .05, FDR). Conclusions Our results show increased CMRO2 and regional CBF in PwCF, which could be explained by potential differences in PaO2/PaCO2 and/or endothelial cell function. Our findings highlight the need for further investment in brain research in PwCF to reduce the risk of early cerebrovascular breakdown that leads to premature cognitive decline.