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Neurodevelopmental outcomes after staged palliation for hypoplastic left heart syndrome
Sarah Tabbutt1, Alex S Nord, Gail P Jarvik
1Department of Pediatrics, Division of Cardiology, Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA. tabbutt@email.chop.edu
Insights
Genetic factors, not surgical care, significantly impact neurodevelopmental outcomes in infants with hypoplastic left heart syndrome (HLHS). Many HLHS survivors show concerning motor delays and cognitive deficits by age one.
Area of Science:
- Pediatric Cardiology
- Neurodevelopmental Pediatrics
- Congenital Heart Disease Research
Background:
- Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect requiring complex surgical palliation.
- Survivors of HLHS are at high risk for neurodevelopmental disabilities.
- Understanding factors influencing neurodevelopment is crucial for improving long-term outcomes.
Purpose of the Study:
- To investigate the relative contributions of genetic factors and surgical management to neurodevelopmental outcomes in one-year-old HLHS survivors.
- To identify specific variables associated with neurodevelopmental deficits in this population.
Main Methods:
- A cohort of infants undergoing staged reconstruction for HLHS were assessed at one year of age.
- Neurodevelopmental assessment included neuromuscular examination and the Bayley Scales of Infant Development II (Mental and Psychomotor Development Indices).
- Multivariate analyses evaluated the impact of genetic, perioperative, and operative variables on developmental scores.
Main Results:
- A significant proportion of patients (28%) had confirmed or suspected genetic syndromes.
- At one year, 65% had abnormal or suspect neuromuscular exams, and 48% had Psychomotor Development Index scores below 70.
- Younger gestational age, presence of a genetic syndrome, and preoperative intubation were associated with poorer neurodevelopmental outcomes.
Conclusions:
- One-year-old HLHS survivors exhibit a high incidence of neurodevelopmental disabilities, particularly motor deficits.
- Genetic syndromes significantly impair neurodevelopmental outcomes in HLHS survivors.
- Surgical factors, including cardiopulmonary bypass and circulatory arrest duration, were not associated with neurologic outcomes.
Objective:
The goal was to determine the relative effects of underlying genetic factors and current management strategies on neurodevelopmental disabilities among one-year old survivors of palliation for hypoplastic left heart syndrome.
Methods:
Children who underwent staged reconstruction for hypoplastic left heart syndrome and variants were assessed at 1 year of age by using a neuromuscular examination and the Bayley Scales of Infant Development II, which provide the Mental Development Index and the Psychomotor Development Index. The effects of perioperative, operative, and genetic variables on developmental scores were evaluated.
Results:
The median birth weight was 3.3 kg (range: 2.1-4.5 kg). Eight-three patients (94%) underwent multiple operations with cardiopulmonary bypass during the first year of life (median: 2 operations). Seven patients (8%) required extracorporeal membrane oxygenation. Twenty-five patients (28%) had a confirmed or suspected genetic syndrome. At 1 year of age, the neuromuscular examination results were abnormal or suspect for 57 patients (65%). The median Mental Development Index score was 90, and 10 patients (11%) had scores of <70 (2 SDs below the general population mean). The median Psychomotor Development Index score was 73, and 42 patients (48%) had scores of <70. In multivariate analyses, younger gestational age, the presence of a genetic syndrome, and the need for preoperative intubation had significant negative effects on neurodevelopmental outcomes. No association was found with operative factors, including duration of deep hypothermic circulatory arrest.
Conclusions:
At 1 year of age, there was a significant incidence of neurodevelopmental disabilities in children with hypoplastic left heart syndrome and variants; motor scores were particularly concerning. Many children had suspected or confirmed genetic syndromes, which negatively affected neurodevelopmental outcomes. Surgical variables did not affect neurologic outcomes.
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