Critical congenital heart disease beyond HLHS and TGA: neonatal brain injury and early neurodevelopment

Rachel Vassar; Shabnam Peyvandi; Dawn Gano; Stephany Cox; Yensy Zetino; Steven Miller; Patrick McQuillen

doi:10.1038/s41390-023-02490-9

Critical congenital heart disease beyond HLHS and TGA: neonatal brain injury and early neurodevelopment

Pediatr Res. 2023 Aug;94(2):691-698. doi: 10.1038/s41390-023-02490-9. Epub 2023 Feb 13.

Authors

Rachel Vassar¹, Shabnam Peyvandi², Dawn Gano³, Stephany Cox^{2

4}, Yensy Zetino², Steven Miller⁵, Patrick McQuillen⁶

Affiliations

¹ Division of Pediatric Neurology, Department of Neurology, Benioff Children's Hospital, University of California, San Francisco, CA, USA. rachel.vassar@ucsf.edu.
² Division of Pediatric Cardiology, Department of Pediatrics, Benioff Children's Hospital, University of California, San Francisco, CA, USA.
³ Division of Pediatric Neurology, Department of Neurology, Benioff Children's Hospital, University of California, San Francisco, CA, USA.
⁴ Division of Developmental Medicine, Department of Pediatrics, Benioff Children's Hospital, University of California, San Francisco, CA, USA.
⁵ Department of Neurology, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
⁶ Division of Critical Care, Department of Pediatrics, Benioff Children's Hospital, University of California, San Francisco, CA, USA.

Abstract

Background: Characterization of brain injury and neurodevelopmental (ND) outcomes in critical congenital heart disease (cCHD) has primarily focused on hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA). This study reports brain injury and ND outcomes among patients with heterogeneous cCHD diagnoses beyond HLHS and TGA.

Methods: This prospective cohort study included infants with HLHS, TGA, or heterogenous "Other cCHD" including left- or right-sided obstructive lesions, anomalous pulmonary venous return, and truncus arteriosus. Brain injury on perioperative brain MRI and ND outcomes on the Bayley-II at 30 months were compared.

Results: A total of 218 participants were included (HLHS = 60; TGA = 118; "Other cCHD" = 40, including 8 with genetic syndromes). Pre-operative (n = 209) and post-operative (n = 189) MRI showed similarly high brain injury rates across groups, regardless of cardiopulmonary bypass exposure. At 30 months, participants with "Other cCHD" had lower cognitive scores (p = 0.035) compared to those with HLHS and TGA, though worse ND outcome in this group was driven by those with genetic disorders.

Conclusions: Frequency of brain injury and neurodevelopmental delay among patients with "Other cCHD" is similar to those with HLHS or TGA. Patients with all cCHD lesions are at risk for impaired outcomes; developmental and genetic screening is indicated.

Impact: This study adds to literature on risk of brain injury in patients with critical congenital heart disease (cCHD) diagnoses other than hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA), a heterogenous cohort of patients that has often been excluded from imaging studies. Children with cCHD beyond HLHS and TGA have similarly high rates of acquired brain injury. The high rate of neurodevelopmental impairment in this heterogenous group of cCHD diagnoses beyond HLHS and TGA is primarily driven by patients with comorbid genetic syndromes such as 22q11.2 deletion syndrome.

Publication types

Research Support, N.I.H., Extramural

MeSH terms

Brain Injuries* / diagnostic imaging
Child
Heart Defects, Congenital* / diagnosis
Humans
Hypoplastic Left Heart Syndrome* / diagnostic imaging
Infant
Infant, Newborn
Prospective Studies
Transposition of Great Vessels* / surgery

Abstract

Publication types

MeSH terms

Grants and funding