1. Critical congenital heart disease beyond HLHS and TGA: neonatal brain injury and early neurodevelopment
- Author
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Vassar, Rachel, Peyvandi, Shabnam, Gano, Dawn, Cox, Stephany, Zetino, Yensy, Miller, Steven, and McQuillen, Patrick
- Subjects
Biomedical and Clinical Sciences ,Cardiovascular Medicine and Haematology ,Clinical Sciences ,Infant Mortality ,Cardiovascular ,Clinical Research ,Brain Disorders ,Heart Disease ,Genetics ,Neurosciences ,Rare Diseases ,Pediatric ,Perinatal Period - Conditions Originating in Perinatal Period ,2.1 Biological and endogenous factors ,Aetiology ,Neurological ,Good Health and Well Being ,Infant ,Infant ,Newborn ,Child ,Humans ,Transposition of Great Vessels ,Hypoplastic Left Heart Syndrome ,Prospective Studies ,Heart Defects ,Congenital ,Brain Injuries ,Paediatrics and Reproductive Medicine ,Public Health and Health Services ,Pediatrics ,Paediatrics - Abstract
BackgroundCharacterization 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.MethodsThis 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.ResultsA 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.ConclusionsFrequency 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.ImpactThis 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.
- Published
- 2023