Author: Anila B. Elliott, MD - University of Michigan, C.S. Mott Children’s Hospital
A 5-day-old male with d-Transposition of the Great Arteries (TGA) and ventricular septal defect presents for arterial switch operation. Which one of the following perioperative factors would INCREASE the risk of poor neurodevelopmental outcomes in this patient?
EXPLANATION
Over the last few decades, survival for those with congenital heart disease (CHD) has improved as advancements in management have led to innovative treatment for even those with more complex anatomy. However, despite a survival improvement, neurodevelopmental outcomes have not improved in the same manner. Children with CHD are at increased risk for a variety of neurodevelopmental issues including motor delays, impaired language development, and reduced cognitive performance. Some of these deficits are more pronounced in those with more complex congenital heart disease, such as a lower intelligence quotient (IQ) in those with hypoplastic left heart syndrome (HLHS) compared to those with atrial septal defects (ASD)1.
In the latest American Heart Association (AHA) guidelines, new research has led to improved understanding regarding perioperative risk factors that impact neurodevelopment1. Genetic predisposition, perinatal, socioeconomic, and perioperative factors all play a role in long-term neurodevelopmental outcomes 1,2.
A summary of major risk factors for neurodevelopmental impairment can be found below:1,2:
1. Genetic predisposition
a. Syndromes (Down syndrome, 22q11.2 deletions, William syndrome, Turner syndrome)
b. Male gender
c. Extracardiac anomalies
2. Fetal and perinatal factors
a. Cyanosis with hypoxia and reduced nutrient delivery
b. Prematurity, especially in those with transposition of great arteries (TGA) or single-ventricle physiology
c. Post-natal diagnosis of CHD requiring early surgical intervention
3. Surgical and perioperative factors
a. Perioperative seizures in infancy
b. Brain injury (white matter injury, stroke)
c. Increased post-operative length of stay (>14 days)
d. Cardiopulmonary resuscitation or cardiac arrest
e. Mechanical circulatory support
f. Heart transplantation in childhood
g. Need for multiple interventions or complications
4. Psychosocial factors
a. Low socioeconomic status
b. Low maternal education
c. Significant parental mental health challenges (anxiety, depression, post-traumatic stress disorder [PTSD])
5. Growth and developmental factors
a. Feeding difficulties
b. Growth failure (including low birth weight and failure to thrive)
c. History of developmental delay
6. Other factors
a. Abnormal fetal cerebral blood flow
b. Abnormal placental development
c. Exposure to environmental neurotoxins
Perioperative seizures (Answer A) are strongly associated with poor neurodevelopmental outcomes. Seizures are linked to underlying brain injury and predict long-term deficits in cognitive, academic, and functional abilities1,2.
Delays in surgical correction are also associated with poorer neurodevelopmental outcomes. For example, in neonates with TGA, prolonged time to arterial switch surgery has been shown to increase the risk of white matter ischemia, which is tied to subsequent neurodevelopmental impairments3. This concern is consistent with earlier findings that delayed surgical intervention is linked to impaired brain growth and worse language development2.
While lower hematocrit levels and hypotension during CPB may impair oxygen delivery, and theoretically contribute to cerebral hypoperfusion and injury, the direct long-term impact of this on neurodevelopment has not been well-established. There is no current data to support specific thresholds for mean arterial pressure (MAP) or hematocrit during bypass that is predictive of preventing poor neurodevelopmental outcomes across large populations1,2,4.
The correct answer is A, perioperative seizures increase the likelihood of poor neurodevelopmental outcomes based on the most recent AHA guidelines1. Although lower hematocrit and hypotension may play a role, there is no definitive data to recommend specific surgical, hemodynamic, or transfusion practices in the congenital cardiac patient population to prevent neurodevelopmental impairment.
REFERNCES
1. Sood E, Newburger JW, Anixt JS, et al. Neurodevelopmental Outcomes for Individuals With Congenital Heart Disease: Updates in Neuroprotection, Risk-Stratification, Evaluation, and Management: A Scientific Statement From the American Heart Association. Circulation. 2024;149(13):e997-e1022. doi:10.1161/CIR.0000000000001211
2. Wernovsky G, Licht DJ. Neurodevelopmental Outcomes in Children With Congenital Heart Disease-What Can We Impact?. Pediatr Crit Care Med. 2016;17(8 Suppl 1):S232-S242. doi:10.1097/PCC.0000000000000800
3. Lim JM, Porayette P, Marini D, et al. Associations Between Age at Arterial Switch Operation, Brain Growth, and Development in Infants With Transposition of the Great Arteries. Circulation. 2019;139(24):2728-2738. doi:10.1161/CIRCULATIONAHA.118.037495
4. Gaynor JW, Stopp C, Wypij D, et al. Neurodevelopmental outcomes after cardiac surgery in infancy. Pediatrics. 2015;135(5):816-825. doi:10.1542/peds.2014-3825
