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  • Updated 03.29.2024
  • Released 07.12.1994
  • Expires For CME 03.29.2027

Congenital heart disease: neurologic complications



Congenital heart defects are the most common birth defects with an estimated prevalence of more than 8 to10 per 1000 live births in the United States, and they account for about 10% of all birth defects worldwide. Acute and chronic neurologic complications are not uncommon in children born with congenital heart disease: stroke, seizures, epilepsy, hemorrhage, infections and cognitive deficits. Data on developmental outcomes, mortality, and morbidity have been rapidly updated as surgical techniques improve. This article reviews the current literature and describes the different types of congenital heart defects, including genetic and environmental factors, and their associated neurologic disorders/complications. Methods for management, treatment, and prevention of the most common types of congenital heart disease are also provided.

Key points

• Children born with congenital, or acquired, heart disease experience adverse neurologic complications in up to 25% of cases.

• Changes in brain development can occur as early as the fetal period in severe heart defects, eg, TGA and HLHS, due to deficiencies in oxygen and nutrients delivered to the brain resulting in worse neurologic outcomes in patients with congenital heart diseases compared to their unaffected peers.

• With the growing success of complex surgical procedures that correct congenital heart defects, infants who might have previously died are now surviving into adulthood.

• Alongside improved survival rates, advancements in critical care and neuroprotective strategies and technology have altered the neurodevelopmental outcomes seen in congenital heart disease survivors. Application of noninvasive diagnostic tools, such as MRI, MRA, CT, microarray analysis, and cranial ultrasound studies in neonates, have allowed us to diagnose vascular lesions that in previous years may have been silent.

Historical note and terminology

Aggressive surgical correction of congenital heart disease had been proposed as the future treatment of choice years before the first open heart surgery in children in 1951. Cardiopulmonary bypass with hypothermia techniques soon followed in 1953. As more difficult cases were taken to surgery and as survival rates improved, more neurologic complications were noted. It has been shown that therapeutic hypothermia, now a standard procedure in neonatal hypoxic-ischemic encephalopathy, can be applied to patients with severe congenital heart disease and anoxic injury (05). Advances in technology have altered the types of neurologic complications that are seen in survivors of congenital heart disease who would likely have died decades ago.

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