Coupling of central venous pressure and intracranial pressure in a 6-year-old patient with fontan circulation and intracranial hemorrhage.

A sharp rise in intracranial pressure (ICP) was noted in a 6-year-old girl with Fontan circulation after surgical removal of a hematoma associated with intracranial hemorrhage. Inhalation of nitric oxide resulted in reduction of central venous pressure (CVP) and precipitous fall in ICP, which was otherwise resistant to conventional therapy. Specifically, the rate of fall of ICP (9 mm Hg) exceeded that of the CVP (3 mm Hg), indicating disproportionate CVP-ICP coupling. The present case provides not only a novel insight into cardio-cerebral interaction in Fontan physiology but also useful information regarding the treatment of Fontan patients in the setting of increased ICP.