Prenatal head growth and white matter injury in hypoplastic left heart syndrome

Area of Science:

• Pediatric Cardiology
• Neurodevelopmental Pediatrics
• Fetal Medicine

Background:

• Hypoplastic left heart syndrome (HLHS) is associated with a higher incidence of central nervous system (CNS) abnormalities.
• The precise causes of CNS issues in HLHS, including maldevelopment, prenatal ischemia, or postnatal factors, remain unclear.

Purpose of the Study:

• To investigate the etiology of CNS abnormalities in fetuses and neonates diagnosed with HLHS.
• To analyze prenatal head growth and brain pathology in HLHS cases.

Main Methods:

• Evaluation of 68 neonates with HLHS, including fetal ultrasound and echocardiogram data in 28 cases.
• Assessment of head size, head growth, and aortic valve anatomy.
• Neuropathological examination of brains from 11 electively aborted HLHS fetuses.

Main Results:

• HLHS neonates exhibited significantly smaller mean head circumference percentiles compared to HLHS fetuses.
• Half of the evaluated HLHS fetuses showed a significant decrease in head growth, with nearly a quarter exhibiting growth restriction.
• Brains from HLHS fetuses displayed chronic diffuse white matter injury of varying severity.

Conclusions:

• Prenatal head growth patterns and brain histopathology in HLHS fetuses indicate a spectrum of abnormal CNS development and/or injury.
• These findings highlight the importance of early CNS assessment in HLHS management.