Somatic growth trajectory in the fetus with hypoplastic left heart syndrome

Abstract

Background: Fetal growth abnormalities in hypoplastic left heart syndrome (HLHS) have been documented primarily by birth measurements. Fetal growth trajectory has not been described. We hypothesized that fetal growth trajectory declines across late gestation in this population. Methods: Infants with a prenatal diagnosis of HLHS and no history of prematurity or a genetic syndrome were identified. Fetal ultrasound measurements and birth anthropometrics were obtained from clinical records. z-Scores for estimated fetal weight (EFWz) and birth weight (BWTz) were compared. BWTz for three neonatal standards were compared. results: Paired fetal and neonatal data were identified in 33 cases of HLHS. Mean gestational age at ultrasound and birth were 27 and 38 wk, respectively. BWTz was lower than EFWz by a mean of 0.82 (SD: 0.72, P < 0.0001), with 64% of subjects demonstrating a decrease in z-score of >0.5. Umbilical artery (UA) Doppler found no evidence of significant placental insufficiency. Modest differences in BWTz were seen across BWT standards in this cohort. conclusion: The majority of fetuses with HLHS demonstrate decreased growth velocity during later pregnancy, suggesting growth abnormalities manifest in utero. The potential relationship to future clinical outcomes warrants further study.

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

“…As highlighted in our placental structural data, the current cohort of HLHS samples demonstrates a range of impairments from the most severe reduction in villous development through to placental structures that resemble control. Since HLHS fetuses typically demonstrate normal growth and development to mid-gestation [7, 8], and some of the findings of the current study, including the presence of syncytial sprouts and the reduced terminal villi and vasculo-syncytial membrane numbers in term placentas, it appears impairment of fetal growth in this context occurs during late gestation, consistent with placental villous immaturity.…”

“…We previously described a cohort of 38 HLHS fetuses [7, 8]. From this cohort, pathology evaluation and archived placenta tissue was available in 16 (42%) cases.…”

“…Previous studies have established that HLHS newborns are born smaller than their anticipated birth weight based on ultrasound growth trajectory [8]. Despite a lack of abnormal ultrasound Doppler, poor fetal growth was seen in 64% of HLHS cases with only 6% having a birth weight greater than their estimated fetal weight.…”

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

“…Surprisingly, neither LBW nor SGA infants with HLHS demonstrated an increased frequency of adverse clinical outcomes, including mortality, at 1 y, which is contrary to previous reports (12,24,25). Although this may reflect an inadequate cohort size, a more compelling possibility is that by excluding prematurity and genetic syndromes (high-risk infants), two common causes of LBW, adverse outcomes are associated more strongly with gestational age rather than weight (26). Indeed, LBW infants with HLHS demonstrate an increased frequency of mortality after stage I surgery (27) but not before surgery (9).…”

“…Finally, interpreting growth using ultrasound estimates at the fetal time point and actual measurements at the neonatal time point creates a challenging problem (26) and should be acknowledged in the interpretation of these findings. A large multicenter study may allow stratification of the cohort to confirm and expand these observations, and assessing the effects of abnormal fetal growth may inform longitudinal adult quality-of-life evaluations (38).…”