Microcephaly is associated with early adverse neurologic outcomes in hypoplastic left heart syndrome

Abstract

Background: Hypoplastic left heart syndrome (HLHS) is associated with significant mortality and morbidity. Fetal head growth abnormalities have been identified in a subset of HLHS fetuses, but it is unclear whether specific patterns of maladaptive growth affect clinical outcomes. We hypothesized that poor fetal head growth is associated with an increased frequency of adverse clinical outcomes. Methods: We retrospectively examined a cohort of HLHS patients from midgestation to 1 y of age. Fetal and birth anthropometric measurements were analyzed using the Olsen standard, and clinical outcomes were obtained. results: A total of 104 HLHS patients were identified over a 12-y period; fetal data were available in 38 cases. HLHS neonates demonstrated a high incidence of microcephaly (12%), small head size (27%), and poor head growth (32%). Allcause mortality was 31% at 30 d and 43% at 1 y. Neurologic outcomes were observed in 12% of patients and were significantly increased with microcephaly (43 vs. 4%; P = 0.02). The average length of hospital stay following stage I palliation was 33.4 ± 33 d, correcting for early death. conclusion: In term nonsyndromic HLHS, fetal and neonatal microcephaly are associated with early adverse neurologic outcomes but not mortality.

Neurodevelopmental Outcomes in Children With Congenital Heart Disease—What Can We Impact?

“…The incidence of microcephaly at birth is increased in children with complex CHD, approaching 25% of children in some reports (10, 33–35), persists into later infancy, and is associated with later developmental abnormalities (36). While the causes are speculative, and most certainly multifactorial, Shillingford et al reported on a series of children with the hypoplastic left heart syndrome where the median head circumference was only at the 18th percentile.…”

Somatic growth trajectory in the fetus with hypoplastic left heart syndrome

“…It is also possible that, despite oxygen delivery via the maternal-fetal circulation and compensatory mechanisms within the central nervous system (23), the fetus with HLHS is subject to metabolic inefficiencies of hypoxia, particularly in the brain (24). Cerebral hypoxia is one potential explanation for the well-described reduction in head and brain size associated with HLHS and may contribute to subsequent adverse clinical outcomes (25).…”

Hypoplastic left heart syndrome is associated with structural and vascular placental abnormalities and leptin dysregulation

“…Growth patterns have been characterized in HLHS, and about 50% of fetuses are affected by growth abnormalities [6]. Recently, we described fetal growth in HLHS, and in a significant proportion of cases, weight and/or head circumference demonstrate diminished growth trajectories late in gestation [7–9]. Whether growth abnormalities in HLHS represent primary and/or secondary insults to the fetus is unknown, and the potential role of impaired placental development and/or function in the manifestation of these growth abnormalities remains unclear.…”

“…Gross placental pathology has not been identified in large studies examining all CVM collectively [12]. Several studies have shown pregnancies complicated by fetuses with HLHS have normal umbilical artery Doppler by ultrasound assessment [7, 13, 14], suggesting appropriate feto-placental blood flow, but these studies have not assessed placental structure, vascularization or function. The significantly increased incidence of any growth abnormality in newborns with HLHS, approximately 40%, implicates placental dysfunction.…”