Implications of Atrial-Level Shunting by Echocardiography in Newborns with Congenital Diaphragmatic Hernia.

OBJECTIVE: To determine whether postnatal echocardiographic indices of left ventricular (LV) size and function are associated with atrial-level shunt direction and investigate whether a relationship exists between LV function and LV size in patients with congenital diaphragmatic hernia (CDH). STUDY DESIGN: This was a single-center retrospective study of 51 neonates with CDH evaluated at Children's Hospital Colorado. Initial postnatal echocardiograms were analyzed for cardiac dimensions, valvar integrity, cardiac time intervals, and biventricular function. Comparisons were made between neonates with left-to-right vs right-to-left atrial-level shunting, as well as between those with decreased (ejection fraction <55%) vs normal (ejection fraction ≥55%) LV function. RESULTS: The majority (82.4%) of infants with CDH had a left-to-right atrial-level shunt, and 84.3% had systemic or suprasystemic pulmonary artery pressure. Decreased LV function was demonstrated in 37.2% and was associated with smaller LV volumes and worse outcomes, including the need for extracorporeal membrane oxygenation (ECMO) and survival. CONCLUSIONS: Left-to-right atrial-level shunting is common in neonates with severe CDH, even in the presence of suprasystemic pulmonary artery pressure. LV dysfunction correlates with decreased LV volumes and is associated with adverse neonatal outcomes, including increased need for ECMO and decreased survival.

Right atrial function in pediatric heart transplant patients by echocardiographic strain measurements.

BACKGROUND: CAV is a major cause of mortality in PHTx patients. Research on echocardiographic indices to detect CAV focuses primarily on ventricular function and less is known about RAF. Thus, we primarily sought to evaluate RAF in PHTx patients with CAV. For secondary analysis, we compared RAF between PHTx patients and control patients and evaluated RAF with respect to rejection and surgical type. METHODS: We retrospectively evaluated echocardiography derived RA strain indices in recipients <18 years old and >1 year from time of transplant. The RA strain phases included, reservoir (εs), conduit (εe), pump (εa), and respective strain rate indices (SRs, SRe, SRa). RESULTS: There were 36 PHTx patients and 14 age-, sex-matched control patients. There was a significant reduction in εs, εe, SRs, and SRe (P < 0.001) in the PHTx patients when compared to controls. There was no difference between the CAV (+) and CAV (-) patients with respect to RAF indices. Furthermore, εs, εe, and SRe (P < 0.05) were lower in patients with acute rejection (n = 7) compared to those without (n = 26). Patients with a bi-atrial anastomosis (n = 14) had decreased εs, εa, SRs, SRa (P < 0.05), compared to bi-caval anastomosis (n = 24). CONCLUSION: PHTx patients have decreased RAF compared to healthy children. RAF does not differentiate PHTx patients based on the presence of CAV. RAF is also decreased in PHTx patients with rejection and in those transplanted with a bi-atrial anastomosis.

Circulating biomarkers of left ventricular hypertrophy in pediatric coarctation of the aorta.

OBJECTIVE: Patients undergoing surgical repair of aortic coarctation have a 50% risk of pathologic left ventricular remodeling (increased left ventricular mass or relative wall thickness). Endothelin 1, ST2, galectin 3, norepinephrine and B-natriuretic peptide are biomarkers that have been associated with pathologic LV change in adult populations but their predictive value following pediatric coarctation repair are not known. HYPOTHESIS: Biomarker levels at coarctation repair will predict persistent left ventricular remodeling at 1-year follow up. DESIGN: Prospective, cohort study of 27 patients' age 2 days-12 years with coarctation of the aorta undergoing surgical repair. Echocardiograms were performed preoperation, postoperation, and at 1-year follow-up. Plasma biomarker levels were measured at the peri-operative time points. Association between biomarker concentrations and echocardiographic parameters was assessed. RESULTS: Neither left ventricular mass index nor relative wall thickness varied from pre-op to post-op. At pre-op, relative wall thickness was elevated in 52% and left ventricular mass index was elevated in 22%; at follow-up, relative wall thickness was elevated in 13% and left ventricular mass index was elevated in 8%. Presence of residual coarctation did not predict left ventricular remodeling (AUC 0.59; P > .05). Multivariable receiver operating characteristic curve combining pre-op ST2 and endothelin 1 demonstrated significant predictive ability for late pathologic left ventricular remodeling (AUC 0.85; P = .02). CONCLUSIONS: Persistent left ventricular hypertrophy and abnormal relative wall thickness at intermediate-term follow-up was rare compared to previous studies. A model combining pre-op endothelin 1 and ST2 level demonstrated reasonable accuracy at predicting persistent abnormalities in this cohort. Larger studies will be needed to validate this finding and further explore the mechanism of persistent left ventricular remodeling in this population.

Three-dimensional echocardiographic evaluation of Ebstein's anomaly of the tricuspid valve in a patient with hypoplastic left heart syndrome.

We report three-dimensional imaging of a rare finding of Ebstein's anomaly of the tricuspid valve in a patient with hypoplastic left heart syndrome, which has been previously reported only by two-dimensional echocardiography. A fetal echocardiogram was performed at 19 weeks that showed a moderately hypoplastic left ventricle, severely hypoplastic mitral valve, a severely hypoplastic aortic valve, and a dysplastic tricuspid valve. Post Caesarean delivery at 40 weeks of gestation, a transthoracic echocardiogram confirmed the findings seen on the fetal echocardiogram. A three-dimensional echocardiogram was then performed, which demonstrated an Ebstein tricuspid valve with apical displacement of the septal leaflet, chordal attachments of the valve to the right ventricular outflow tract, and moderate tricuspid regurgitation. The patient underwent a successful heart transplantation.

Right Ventricular Tissue Doppler Myocardial Performance Index in Children with Pulmonary Hypertension: Relation to Invasive Hemodynamics.

Right ventricular (RV) failure is a significant cause of morbidity and mortality in patients with pulmonary hypertension (PH). Myocardial performance index measured by tissue Doppler imaging (TDI-MPI) has been useful in assessing RV dysfunction in adults with PH. However, TDI-MPI as a marker for RV dysfunction or disease severity has not been evaluated in pediatric PH. The aim of this study was to investigate TDI-MPI and correlate with invasive hemodynamics in pediatric PH patients. Eighty pediatric PH patients undergoing cardiac catheterization and simultaneous transthoracic echocardiography were analyzed. RV TDI-MPI was averaged over three cardiac cycles and measured under each condition of vasodilatory testing during the catheterization. TDI-MPI was compared between PH patients and age-matched controls and correlated to invasive hemodynamics. RV TDI-MPI was increased in PH patients compared to controls (0.49 vs. 0.35, p < 0.0001). Significant associations (beta ± SE) are seen between RV TDI-MPI and baseline mean pulmonary arterial pressures (0.0002 ± 0.001, p < 0.05), indexed pulmonary vascular resistance (0.007 ± 0.002, p < 0.002), and pulmonary-to-systemic arterial pressure ratio (0.146 ± 0.063, p < 0.05). No statistically significant associations were seen with vasodilatory testing. RV TDI-MPI is elevated in children with PH, suggestive of RV dysfunction. RV TDI-MPI shows correlation with severity of PH at baseline but lacks sensitivity to evaluate the RV response to acute changes in afterload in children with PH. Therefore, while RV TDI-MPI can help identify RV dysfunction in children with PH, its utility as a non-invasive surrogate marker for acute changes in hemodynamics is limited.

Endothelin-1 activation in pediatric patients undergoing surgical coarctation of the aorta repair.

AIM: To determine endothelin-1 (ET-1) concentration before and after surgical coarctectomy and evaluate its association with left ventricular geometric change. METHODS: A prospective, cohort study of 24 patients aged 2 d to 10 years with coarctation of the aorta undergoing surgical repair. A sub-cohort of patients with age < 1 mo was classified as "neonates". Echocardiograms were performed just prior to surgery and in the immediate post-op period to assess left ventricle mass index and relative wall thickness (RWT). Plasma ET-1 levels were assessed at both time points. Association between ET-1 levels and ventricular remodeling was assessed. RESULTS: Patients < 1 year demonstrated higher pre-op ET-1 than post-op (2.8 pg/mL vs 1.9 pg/mL, P = 0.02). Conversely, patients > 1 year had no change in ET-1 concentration before and after surgery (1.1 vs 1.4, NS). Pre-op, patients < 1 year demonstrated significantly higher ET-1 than older children (2.8 vs 1.1, P = 0.001). Post-op there was no difference between the age groups (1.9 vs 1.4, NS). Neither RWT nor left ventricle mass index (LVMI) varied from pre-op to post-op. The subset of neonates showed a strong positive correlation between pre-op ET-1 and RWT (r = 0.92, P = 0.001). Patients with ET-1 > 2 pg/mL pre-op demonstrated higher LVMI (65.7 g/m2.7vs 38.5 g/m2.7, P = 0.004) and a trend towards higher RWT (45% vs 39%, P = 0.07) prior to repair than those with lower ET-1 concentration. CONCLUSION: ET-1 concentration is significantly variable in the peri-operative period surrounding coarctectomy. Older children and infants have different responses to surgical repair suggesting different mechanisms of activation.

Three-dimensional Echocardiography of Right Ventricular Function Correlates with Severity of Pediatric Pulmonary Hypertension.

BACKGROUND: Right ventricular function and biomarkers of B-type natriuretic peptide (BNP) and N-Terminal pro-BNP (NT pro-BNP) are used to determine the severity of right ventricular failure and outcomes from pulmonary hypertension. Real-time three-dimensional echocardiography (3DE) is a novel quantitative measure of the right ventricle and decreases the geometric assumptions from conventional two-dimensional echocardiography (2DE). We correlated right ventricular functional measures using 2DE and single-beat 3DE with biomarkers and hemodynamics to determine the severity of pediatric pulmonary hypertension. METHODS: We retrospectively evaluated 35 patients (mean age 12.67 ± 5.78 years) with established pulmonary hypertension who had echocardiograms and biomarkers on the same day. Ten out of 35 patients had hemodynamic evaluation within 3 days. 2DE evaluation included tricuspid annular plane systolic excursion (TAPSE), right ventricular myocardial performance index from tissue Doppler imaging (RV TDI MPI), and right ventricular fractional area change (FAC). Three-dimensional echocardiography evaluation included right ventricular ejection fraction (EF), end-systolic volume, and end-diastolic volume. The quality of the 3DE was graded as good, fair, or poor. Pearson correlation coefficients were utilized to evaluate between biomarkers and echocardiographic parameters and between hemodynamics and echocardiography. RESULTS: Three-dimensional echocardiography and FAC correlated significantly with BNP and NT pro-BNP. TAPSE and RV TDI MPI did not correlate significantly with biomarkers. 3D right ventricular EF correlated significantly with hemodynamics. Two-dimensional echocardiography did not correlate with hemodynamics. CONCLUSIONS: Single-beat 3DE is a noninvasive, feasible tool in the quantification of right ventricular function and maybe more accurate than conventional 2DE in evaluating severity of pulmonary hypertension.

The professional appearance of registered nurses: an integrative review of peer-refereed studies.

BACKGROUND: There is a paucity of research examining the impact of standardized uniform style and color for registered nurses (RNs). OBJECTIVE: The aim of this study was to appraise published peer-refereed studies on the effect of a standardized uniform style and color for RNs. METHODS: Seven peer-refereed studies exploring standardized uniforms for RNs were identified. Using the Oxford Centre for Evidence-Based Medicine evidence hierarchy, each study was assigned a level of evidence and overall rating. RESULTS: Data suggest that patients felt that RNs appeared professional and were easily identified by a standardized uniform style and color. No strong evidence supports a patient's preference for a specific style and color of uniform. CONCLUSIONS: Findings are inconsistent but demonstrate that a standardized uniform style and color increased the perception of professionalism and recognition of RNs among patients.