A study to validate blood flow and brain oxygen delivery measurements in pre-surgical hypoplastic heart syndrome using cardiac MRI
Fetuses with Hypoplastic Left Heart Syndrome (HLHS) have been known for some time to have different brain blood flow to other fetuses. We have recently been studying blood flow in HLHS in the newborn period before any operations, and have found that there is evidence of reduced brain blood flow using echocardiography, which may be because too much blood is flowing to the lungs. These findings are important, as in the longer term, HLHS can be associated with some measures of cognitive and other brain function which are lower than normal - reduced brain blood flow in the newborn period may contribute, and might be avoidable. To confirm these findings, we would like to measure the blood flow in stable newborns in non-invasive ways: a cardiac MRI as well as echocardiography, and also measure oxygen levels using a non-invasive test called NIRS (near infrared spectroscopy). To see how these differ from before birth, we would also like to measure blood flow and oxygen levels using fetal MRI close to the time of birth. In this way, we hope to better understand the blood flow in newborns with HLHS, which may allow better monitoring and treatment strategies to prevent brain injury and encougage better long term brain development. The study protocol involved a fetal echocardiogram and a fetal cardiac MRI with extended flow and oxygen measurements at 37 weeks gestation. After birth, we will measure brain oxygenation continuously using NIRS, and do echocardiograms at 6, 24, 48 and 96 hours of life. A cardiac and brain MRI will be performed at 48 hours of life, only on HLHS babies who are stable and breathing by themselves. Routine neurodevelopmental follow-up at 2 and 4 years will be available to check against the results for any trends.