Neurodevelopmental Outcomes in CHD¶

Learning Objectives¶

Core Knowledge & Clinical Reasoning¶

[ ] Stratify neurodevelopmental risk by category (CPB in infancy, chronic cyanosis, additional risk factors)
[ ] Identify specific neurodevelopmental domains commonly affected in CHD (executive function, attention, social)
[ ] Analyze multifactorial contributions to neurodevelopmental outcomes (genetic, perioperative, social)
[ ] Evaluate perioperative neuroprotective strategies (NIRS monitoring, avoiding hypoxia/hypotension)

Management Decisions¶

[ ] Formulate referral pathway for developmental evaluation and early intervention
[ ] Determine appropriate timing for CNOC evaluations at key developmental stages
[ ] Select educational support services based on identified deficits

Communication & Counseling¶

[ ] Counsel families on expected neurodevelopmental trajectory with realistic expectations
[ ] Advocate for appropriate educational accommodations and services
[ ] Address parental anxiety about developmental outcomes while maintaining hope

Systems-Based Practice¶

[ ] Coordinate multidisciplinary developmental follow-up (neurology, psychology, early intervention)
[ ] Develop comprehensive transition plans addressing vocational and educational needs

Key Guidelines¶

2024 AHA Scientific Statement: Neurodevelopmental Outcomes for Individuals with CHD Circulation. 2024;149:e997-e1022

Epidemiology¶

~40-50% of children with CHD have neurodevelopmental impairment
Most common deficits: executive function, attention, social skills
Risk persists into adulthood
Multifactorial etiology

Risk Categories¶

Category 1 - HIGH RISK¶

Cardiac surgery with cardiopulmonary bypass during infancy - Most well-established risk factor - Duration of CPB, circulatory arrest, deep hypothermia all contribute

Category 2 - HIGH RISK¶

Chronic cyanosis - Independent of surgery - Cumulative hypoxic injury - Single ventricle physiology particularly affected

Category 3 - VARIABLE RISK¶

Not meeting Categories 1 or 2, BUT: - Intervention/hospitalization for CHD during infancy-adolescence - PLUS ≥1 additional risk factor (see below)

Additional Risk Factors¶

Category	Examples
Genetic/Syndromic	Variants affecting heart AND brain development
Fetal/Perinatal	Abnormal placenta, prematurity, low birth weight
Procedural	Prolonged/repeated anesthesia exposure
Social/Family	Low SES, parental mental health, limited resources
Growth/Development	FTT, early developmental delays

Neurodevelopmental Deficits by Domain¶

Domain	Common Deficits
Cognitive	Lower IQ, learning disabilities
Executive function	Planning, organization, working memory
Attention	ADHD-like symptoms
Language	Expressive and receptive delays
Motor	Fine and gross motor delays
Social	Social cognition deficits, autism spectrum features
Behavioral/Emotional	Anxiety, depression, internalizing behaviors

Management Algorithm¶

High-Risk (Categories 1 & 2)¶

Referral for developmental evaluation
Early intervention services
CNOC (Cardiac Neurodevelopmental Outcome Collaborative) evaluations at key stages
School-based services as needed
Ongoing monitoring through adolescence

Evaluation Timing¶

Prior to school entry
Transition to middle school
Transition to high school
Young adulthood

Neuroprotective Strategies¶

Prenatal¶

Early prenatal CHD detection
Optimize fetal growth
Delivery close to term (avoid elective preterm)
Delivery at specialized center

Perioperative¶

Brain monitoring during CPB (NIRS)
Standardized perioperative protocols
Avoid hypoxia, hypotension, hypoglycemia
Minimize inflammatory response

Postoperative¶

Early developmental screening
Parent education and support
Early intervention referral

Transition Planning¶

Begin discussing in early adolescence
Address educational and vocational needs
Mental health screening
Driving considerations
Reproductive counseling

Board Pearls¶

Pearl: CPB in infancy OR chronic cyanosis = HIGH RISK

Refer for developmental evaluation and early intervention

Pearl: ~40-50% of CHD children have neurodevelopmental impairment

Most common: executive function, attention, social skills

Pearl: CNOC evaluations at key developmental stages

Pre-school, middle school, high school, young adult transitions

Self-Assessment¶

Q1: A 3-year-old with HLHS (Fontan completed at age 3) is noted to have speech delay and difficulty with transitions. What is the most appropriate next step?

Answer

**Answer**: Referral for comprehensive developmental evaluation and early intervention **Rationale**: This child is Category 1 (cardiac surgery with CPB in infancy) + Category 2 (chronic cyanosis from single ventricle). High risk for neurodevelopmental impairment. Comprehensive evaluation and early intervention services are indicated.

References¶

AHA Scientific Statement. Circulation. 2024;149:e997-e1022