BACKGROUND:

Despite increasing survival, patients with hypoplastic left heart syndrome (HLHS) and other forms of functionally univentricular heart defects (UVHs) remain at increased risk of long-term neurodevelopmental deficits.

METHODS:

A nationwide sample of 23 patients with HLHS, 13 with UVH, and 40 controls were followed prospectively until the age of 5 years, when neurologic, neuropsychological, and motor examinations and brain MRI were performed.

RESULTS:

The median full-scale IQ was significantly lower in patients with HLHS (97, P < .001) and patients with UVH (112, P = .024) compared with controls (121). Major neurodevelopmental impairment was found in 26% of the patients with HLHS and 23% of those with UVH, and minor neurologic dysfunction was found in 43% and 46%, respectively. MRI revealed abnormalities, mostly ischemic changes of different degrees, in 82% of the patients with HLHS and in 56% of those with UVH. Prominent changes were significantly associated with neurodevelopmental findings and parental reports of adaptive behavior. In linear regression, significant risk factors for a worse outcome were a history of clinical seizures in connection with the primary operation, a lower diameter of the neonatal ascending aorta, and several pre-, peri-, and postoperative factors related to the primary and bidirectional Glenn operations.

CONCLUSIONS:

Although median cognitive performance was within the normal range, neurodevelopmental and brain MRI abnormalities were found in the majority of the patients with UVH, and especially in those with HLHS, at preschool age. Both a narrowed ascending aorta and operation-related factors contributed to these findings.

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