Periventricular leukomalacia following neonatal and infant cardiac surgery.

The dramatic reduction in surgical mortality following repair of congenital heart defects has been accompanied by increasing recognition of adverse neurodevelopmental sequelae in some children. Neurodevelopmental abnormalities following neonatal and infant cardiac surgery include mild cognitive impairment, expressive speech and language abnormalities, impaired attention and executive function, impaired visual and spatial motor skills, as well as learning disabilities. These defects result in a significant need for early intervention, as well as rehabilitative and special education services. Central nervous system injury following repair of congenital heart defects results from a complex interaction of patient specific and environmental factors. Recent studies suggest that cerebral white matter injury characterized by periventricular leukomalacia (PVL) is common in infants with congenital heart disease particularly following cardiac surgery. Studies at our institution show that this occurs in greater than 50% of neonates following cardiac surgery, but is rare in older infants. Prolonged exposure to cardiopulmonary bypass (with or without deep hypothermic circulatory arrest) is a risk factor for PVL, possibly secondary to the systemic inflammatory response to cardiopulmonary bypass. Hypotension and hypoxemia in the early postoperative period, especially diastolic hypotension, significantly increase the risk of PVL. Future studies are needed to determine significance of PVL as a marker for long-term developmental dysfunction.