Catastrophic intracranial hemorrhage associated with eosinophilic granulomatous polyangiitis in a patient after Fontan operation.

A 29-year-old man after the Fontan operation had a catastrophic intracranial hemorrhage associated with eosinophilic granulomatous polyangiitis. Despite combination therapy with cyclophosphamide and methylprednisolone, he was dead at 6 months after the onset. The clinical course was worse owing to underlying coagulopathy and endothelial dysfunction associated with congenital heart disease. Key Clnical Massage The majority of patients with complex congenital heart disease survive into adulthood. Potential endothelial dysfunction and coagulopathy can attribute to unexpected clinical manifestations of other extracardiac disease among them.