Implications of a neurosurgical intervention in a patient with a surgically repaired hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) accounts for 4-9% of congenital heart disease in children. The mortality rate among children with HLHS undergoing cardiac repair is well documented, but comparable data for noncardiac surgical procedures are not well known. Medically intractable epilepsy is one of those problems, which arises as a complication of cardiovascular surgery in HLHS and necessitates neurosurgical intervention. There is no published knowledge about neurosurgical procedures performed in children with HLHS in the English literature. Thus, we present a 10-year-old boy who developed medically intractable epilepsy after cardiac surgery for HLHS. The aim of this study is to outline the pre-, intra- and postoperative precautions needed for neurosurgical intervention in HLHS patients to decrease morbidity and mortality.