Introduction: Despite advances in surgical, anesthetic, perfusion, and postoperative care, adverse neurological consequences may occur following cardiac surgery and cardiopulmonary bypass (CPB). Consequences of the physiologic effects of CPB may alter the blood–brain barrier, autoregulation, and intracranial pressure (ICP) in the immediate postoperative period.

Methods: We evaluated the effects of cardiac surgery and CPB on the central nervous system by measuring the optic nerve sheath diameter (ONSD) by using ultrasound as a surrogate marker of ICP. ONSD was measured after anesthetic induction and endotracheal intubation (time 1), after separation from CPB (time 2), and at the completion of the surgical procedure prior to leaving the OR (time 3).

Results: The study cohort included 14 patients, ranging in age from newborn to 6 years. When comparing the Fontan group (n = 5) to the non-Fontan group (n = 9), four elevated ONSD observations were recorded for the Fontan patients during the study period, including one at time 1, one at time 2, and two at time 3. In Fontan versus non-Fontan patients, ONSD was greater at all three time points compared to non-Fontan. The change in the ONSD from time 1 to time 2 was greater (+0.2 mm vs. −0.1 mm), and the mean value at time 2 was significantly higher (4.2 vs. 3.5 mm, P = 0.048).

Conclusions: Patients with Fontan physiology may be more prone to higher levels of baseline intracranial pressure due to elevated systemic venous pressure and decreased cardiac output. Alternatively, the chronically high central venous pressures may artificially elevate ONSD without clinical changes in ICP, necessitating the development of separate normative values based on the type of congenital heart disease.