Congenital heart disease (CHD) is common, occurring in about 4-50 out of every 1000 live births.1 Left untreated, many forms of CHD are lethal within the first days of life and about 1 in 3 babies born with CHD will need a surgical or catheter intervention prior to their first birthday. In the past 50 years, advances in medical, surgical, and cardiac catheterization-based interventions have led to a substantial decrease in early mortality from congenital heart disease, and today greater than 90 % of neonates with cardiac conditions live past 1 year of age.2 Despite the marked increase in survival, neurological morbidity is substantial and up to 40 % of patients who undergo cardiac repair in infancy have measurable neurological impairments on childhood examinations.3 While certain genetic and disease-specific factors are non-modifiable, the field of pediatric cardiology and cardiac surgery has dedicated considerable energy towards identifying opportunities to decrease preventable brain injury.

The relationship between the heart and brain is complex and the pediatric neurologist plays an important and ever-evolving role in the multidisciplinary teams caring for children with CHD. Neurologists provide recommendations for neuromonitoring, rapid interpretation of neuromonitoring and imaging studies, guiding management of neurological emergencies, medication recommendations and prognosis. A basic understanding of terminology used to describe pediatric cardiac diseases, common surgical pathways, and awareness of mechanical circulatory support (MCS) devices is valuable for the consulting neurologist. Additionally, one should be aware of the nuances of care in the pediatric cardiac intensive care unit (CICU) which need to be considered when managing acute neurologic emergencies, such as tenuous hemodynamic profiles and oxygen saturation targets that might differ from children with a normal circulation.

This article provides a neurologically-oriented review of pediatric heart disease, designed to arm the consulting neurologist with the basic tools for understanding the common anatomy and physiology encountered in the pediatric CICU. It will focus on features most associated with neurologic injury including single ventricle (SV) disease, Tetralogy of Fallot (TOF), cardiopulmonary bypass (CPB) and heart failure (HF) supported with MCS devices.