Congenital heart disease
Risk factors for preoperative periventricular leukomalacia in term neonates with hypoplastic left heart syndrome are patient related

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Background

Preoperative brain injury is common in neonates with complex congenital heart disease. Increasing evidence suggests a complex interaction of prenatal and postnatal risk factors for development of brain white matter injury, called periventricular leukomalacia (PVL), in neonates with complex congenital heart disease. To date, there remains a limited understanding of the risk factors contributing to preoperative PVL in hypoplastic left heart syndrome (HLHS).

Methods

Neonates with HLHS or HLHS variants from 3 prospective magnetic resonance imaging studies (2003-2010) were selected for this cohort. Preoperative brain magnetic resonance imaging was performed the morning of the surgery. Stepwise multilogistic regression of patient characteristics, mode of delivery (cesarean section vs vaginal), time of diagnosis (prenatal vs postnatal), HLHS subtypes, brain total maturation score, time to surgery, individual averaged daily preoperative blood gases, and complete blood cell count values was used to determine significant associations.

Results

A total of 57 neonates with HLHS were born at 38.7 ± 2.3 weeks; 86% (49/57) had a prenatal diagnosis, with 31% (18/57) delivered by cesarean section. HLHS with aortic atresia (AA) was common in this cohort, 71% (41/57). Preoperative PVL was identified in 19% (11/57). Male patients with AA (P = .004) were at higher risk for PVL. Lower total brain maturation score was also identified as a strong predictor for preoperative PVL (P = .005).

Conclusions

In neonates with HLHS, nonmodifiable patient-related factors, including male sex with AA (lack of antegrade blood flow) and lower total brain maturation score, placed neonates at the greatest risk for preoperative white matter injury.

CTSNet classification

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Abbreviations and Acronyms

AA
aortic atresia
AIS
arterial ischemic stroke
AS
aortic stenosis
CHD
congenital heart disease
d-TGA
d-transposition of the great arteries
HLHS
hypoplastic left heart syndrome
MRI
magnetic resonance imaging
PVL
periventricular leukomalacia
TMS
total maturation score

Cited by (0)

This study was supported by the National Institute of Neurological Disorders and Stroke (grants NS052380 and NS072338), the Dana Foundation, the June and Steve Wolfson Family Fund for Neurological Research, and the National Heart, Lung, and Blood Institute (grant T-32 HL-0791543 to D.A.G.).

Disclosures: Authors have nothing to disclose with regard to commercial support.