What are the novel findings of this work?Our findings reveal that differences in underlying fetal cardiovascular physiology lead to varying cerebrovascular response to maternal hyperoxygenation and that there are impairments in cerebral autoregulation in certain congenital heart disease groups.
What are the clinical implications of this work?Given the recent interest in studying maternal hyperoxygenation as a therapeutic tool to optimize brain development in fetuses with congenital heart disease, our findings provide important physiologic data that should be incorporated in future clinical trials.
The current echocardiographic diagnostic criteria for noncompaction cardiomyopathy (NCC) have variable sensitivity and low specificity. Moreover, there are limited data on the use of myocardial deformation imaging for early detection of myocardial dysfunction in children with NCC. We describe left ventricular (LV) deformation patterns in children with NCC, with the goal of identifying a potential diagnostic pattern. We prospectively enrolled 30 children with NCC (47% male; mean age 7.2 years) and 30 age- and gender-matched controls. Extent and severity of non compaction in each segment were evaluated in LV 16-segment model. Regional (base, mid and apex) and segmental (16 segments) longitudinal strain (LS), circumferential strain (CS) and radial strain (RS) were measured using speckle tracking echocardiography. In all patients with NCC, regional and segmental CS and RS at the apex were significantly decreased compared to controls (CS apex: - 19.2 ± 5.4% vs. - 30.2 ± 6.9%, p < 0.001/RS apex: 23.5 ± 8.6% vs. 44.1 ± 14.5%, p < 0.001). Thirty percent (9/30) of patients had an EF < 50%. In these patients, there was additional decrease in CS in basal segments and in LS in basal, mid-cavity and apical segments (CS base: - 16.4 ± 4.7% vs. - 24.6 ± 3.9%, p < 0.001/LS (average all LV segments): - 13.9 ± 3.1% vs. - 20.7 ± 4.7%, p < 0.001). A cut-off value of CS at the apex of - 24.5% was a strong differentiating feature between patients with NCC and EF > 50% and controls (sensitivity: 87%, specificity 79%, AUC 0.88, p < 0.001). Children with NCC exhibit a deformation pattern characterized by decreased apical circumferential strain, which may serve as a potential diagnostic tool for NCC. The role of decreased global LV longitudinal and basal circumferential strain should be further evaluated as a potential prognostic tool.
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