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Late neurodevelopmental outcome after repair of total anomalous pulmonary venous connection
Paul M Kirshbom1, Thomas B Flynn, Robert R Clancy
1Division of Cardiovascular Surgery, Emory University, Emory Cklinic Building A, Atlanta, GA 30322, USA. paul_kirshbom@emoryhealthcare.org
Insights
School-aged survivors of infant total anomalous pulmonary venous connection (TAPVC) repair often face neurodevelopmental challenges. Fine motor skills, visual-motor integration, and attention are commonly affected, requiring early intervention for learning disabilities.
Area of Science:
- Pediatric Cardiology
- Developmental Neuroscience
- Congenital Heart Disease Research
Background:
- Total anomalous pulmonary venous connection (TAPVC) is a critical congenital heart defect requiring surgical repair in infancy.
- Long-term neurodevelopmental outcomes in survivors of TAPVC repair are not fully understood.
- Infant cardiac surgery, particularly with circulatory arrest, may pose risks to neurodevelopment.
Purpose of the Study:
- To assess the neurodevelopmental status of school-aged children who underwent surgical repair for TAPVC during infancy.
- To identify specific neurodevelopmental domains that may be affected in TAPVC survivors.
- To inform clinical practice regarding the need for targeted evaluations and interventions.
Main Methods:
- A cohort of school-aged survivors of TAPVC repair underwent standardized neurologic examinations and neurodevelopmental testing.
- Cognitive abilities (IQ), fine motor skills, visual-motor integration, attention, and memory were assessed.
- Data were analyzed to compare performance against population norms and identify significant impairments.
Main Results:
- Significant neurodevelopmental difficulties were observed in school-aged TAPVC survivors.
- Microcephaly was present in 28% of patients, and 27% had abnormal neuromuscular examinations.
- Performance IQ was significantly lower than population norms, with notable impairments in fine motor skills, visual-motor integration, and attention.
Conclusions:
- School-aged survivors of infant TAPVC repair demonstrate a high incidence of neurodevelopmental deficits.
- Fine motor function, visual-motor integration, and attention are the most frequently impacted areas.
- Routine neurodevelopmental evaluation is crucial for early identification of learning disabilities and to facilitate timely intervention.
Abstract:
Objective We sought to define the neurodevelopmental status of school-aged survivors of total anomalous pulmonary venous connection repaired during infancy. Methods All school-aged survivors of total anomalous pulmonary venous connection repair performed at a single institution were eligible. Thirty children returned for neurologic examination and neurodevelopmental testing. Results The median age at total anomalous pulmonary venous connection repair was 16 days (range, 1-141 days), and age at testing was 11 years (range, 6-19 years). Pulmonary venous return was supracardiac in 14 patients, infracardiac in 12 patients, cardiac in 3 patients, and mixed in 1 patient. Preoperative obstructed total anomalous pulmonary venous connection was present in 6 patients. Circulatory arrest was used in all repairs, with a median duration of 35 minutes (range, 17-55 minutes). At follow-up, microcephaly (head circumference <5%) was present in 28%. Neuromuscular examination was suspect or abnormal in 27%. Mean Full-scale IQ (95.3 +/- 18.5) and Verbal IQ (98.6 +/- 20.2) were not different from population norms, but Performance IQ (92.3 +/- 16.9) was significantly lower than population norms ( P = .02). Fine motor skills and visual-motor coordination were significantly impaired ( P < .01 for Grooved Pegboard and Test of Visual-Motor Integration). Patients with total anomalous pulmonary venous connection also had difficulty with tests of attention (Test of Everyday Attention for Children, P < .01), but results of tests of memory function were not significantly different from population norms. Conclusions School-aged survivors of infant total anomalous pulmonary venous connection repair exhibit a significant incidence of neurodevelopmental difficulties. Fine motor function, visual-motor integration, and attention are the most commonly affected domains. Evaluation of these children is indicated to identify those who are at risk for learning disabilities and who could benefit from early intervention.
