Small changes in AoSA produce important changes in septal shear stress. The levels of stress increase are consistent with cellular flow studies showing stimulation of growth factors and cellular proliferation. Steepened AoSA may be a risk factor for the development of SAS. Evidence exists for all four stages of the proposed etiology of SAS.

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Rationale: Endothelin-1 (ET1) is dysregulated in pulmonary hypertension (PH). It may be important in the pathobiology of congenital diaphragmatic hernia (CDH). Objectives: We hypothesized that ET1 levels in the first month would be higher in infants with CDH who subsequently expired or were discharged on oxygen (poor outcome). We further hypothesized that ET1 levels would be associated with concurrent severity of PH. Methods: We sampled plasma at 24 to 48 hours, and 1, 2, and 4 weeks of age in 40 prospectively enrolled newborns with CDH. We performed echocardiograms to estimate pulmonary artery pressure at less than 48 hours of age and weekly to 4 weeks. PH was classified in relationship to systemic blood pressure (SBP): less than 2/3 SBP, 2/3 SBP-systemic is related to pressure, or systemic-to-suprasystemic pressure. Measurements and Main Results: ET1 levels at 1 and 2 weeks were higher in infants with poor outcome compared with infants discharged on room air (median and interquartile range: 27.2 [22.6, 33.7] vs. 19.1 [16.1, 29.5] pg/ml, P 5 0. 03; and 24.9 [17.6, 39.5] vs. 17.4 [13.7, 21.8] pg/ml, P 5 0.01 at 1 and 2 weeks, respectively). Severity of PH was significantly associated with increasing ET1 levels at 2 weeks (16.1 [13.7, 21.8], 21.0 [17.4, 31.1], and 23.6 [21.9, 39.5] pg/ml for increasing PH class, P 5 0.03). Increasing severity of PH was also associated with poor outcome at that time (P 5 0.001). Conclusions: Infants with CDH and poor outcome have higher plasma ET1 levels and severity of PH than infants discharged on room air. Severity of PH is associated with ET1 levels.

This study demonstrates that subaortic stenosis is associated with a steepened aortoseptal angle, as defined by two-dimensional echocardiography, and this association holds in patients with and without a ventricular septal defect. A steepened aortoseptal angle may be a risk factor for the development of subaortic stenosis.

Objective: Although B-type natriuretic peptide (BNP) concentrations seem to be useful for detecting the presence of patent ductus arteriosus, there is no information about their usefulness for monitoring changes in PDA shunt magnitude. Study Design:We performed a retrospective analysis of paired BNPechocardiogram measurements (obtained from infants (24 to 32 weeks gestation) with clinical suspicion of PDA).Result: Individual BNP concentrations (n ¼ 146, from 88 infants) were significantly related to shunt magnitude at the time of measurement and had good discriminating power for detecting a moderate-or-large shunt (area under receiver-operator characteristic curves (ROC-AUC) ¼ 0.85). In total, 36 infants had serial BNP-echocardiogram pairs (n ¼ 91) measured during their hospitalization. Changes (either increases or decreases) in BNP concentrations over time had only fair discriminating power (ROC-AUC ¼ 0.76) for detecting increases or decreases, respectively, in shunt magnitude. Conclusion:The high degree of variability in the BNP measurements made them less useful for monitoring changes in magnitude.

Children with single ventricle physiology have increased ventricular work and are at greater risk for developing heart failure than other children with congenital heart disease. However, diagnosis of heart failure is difficult because few objective measures have been validated in this cohort. Plasma proteins have been identified as biomarkers of heart failure in adults with structurally normal hearts. However, whether these similarly correlate with heart failure in children with single ventricle physiology is unknown, as the etiology of adult heart failure is typically ischemic heart disease, whereas heart failure in these children is presumed to be due to primary myocardial dysfunction. We conducted a single site cross-sectional observational study of young single ventricle patients. Clinical heart failure was defined as a Ross score >2. The association of several candidate biomarkers with heart failure was assessed using logistic regression and Receiver Operator Characteristic (ROC) curves. Nine of 29 included children (31%) were in clinical heart failure. A doubling of plasma Btype natriuretic peptide was associated with an odds ratio for heart failure of 2.17. The area under the ROC curve was 80.3%. A threshold value of ≥30 pg/mL showed both sensitivity and specificity for heart failure. Three other candidate biomarkers were not found to be associated with clinical heart failure in this sample. In conclusion, plasma B-type natriuretic peptide is a sensitive biomarker for clinical heart failure in young children with single ventricle heart disease. Use of this plasma biomarker may facilitate detection of heart failure in these complex patients.