Right ventricular function has been identified as an important prognostic factor in children with pulmonary arterial hypertension. The aim of the study was to assess the deformation pattern and prognostic value of right ventricular longitudinal strain in children with pulmonary arterial hypertension. We prospectively evaluated 46 children (25 with pulmonary arterial hyperetension and 21 age and sex matched controls) using conventional and speckle-tracking echocardiography, brain natriuretic peptide levels and clinical status expressed by WHO functional class and 6-min walking test. According to the clinical status after 12 months of follow-up, the pulmonary arterial hypertension patients were divided in two groups: non-worsening (13) and worsening (12). Right ventricular free wall longitudinal strain and strain rate were significantly lower in children with PAH compared with controls (−24.72 ± 3.48 vs −15.60 ± 3.40, p = 0.0001 and −1.44 ± 0.09 vs −1.09 ± 0.15, p = 0.0001, respectively). There was a more expressed decrease of basal than apical region of right ventricular free wall longitudinal strain/strain rate in pulmonary arterial hypertension patients compared with controls (strain: p = 0.0001 vs p = 0.07 and strain rate: p = 0.0001 vs p = 0.002). Comparing worsening and non-worsening pulmonary arterial hypertension patients there was a significant difference in Mid right ventricular free wall longitudinal strain (−14.00 ± 3.13 vs. −20.76 ± 4.62, p = 0.0001). In multivariable logistic regression analysis Mid right ventricular free wall longitudinal strain was an independent predictor of worsening in pulmonary arterial hypertension children (OR 0.45; 95 % CI: 0.21–0.96, p = 0.041). In ROC curve analysis a cut-off value of Mid right ventricular free wall longitudinal strain of −18.50 % predicted clinical worsening in pulmonary arterial hypertension children, with a sensitivity and specificity of 91.7 and 30.8 %, respectively (area under curve = 0.88 ± 0.06, 95 % CI: 0.75–1.00, p = 0.001). Two-dimensional speckle-tracking echocardiography is a complementary non-invasive tool for assessment of right ventricular function in children with severe pulmonary arterial hypertension, allowing also clinical prediction and segmental analysis of right ventricular myocardial performance in these patients.

The transposition of great arteries (TGA) is one of the most frequent and severe congenital heart diseases. After newborn stabilization and while pending surgical correction, echocardiographic monitoring with a careful evaluation of left ventricle (LV) performance is warranted. In this study, our objectives were (i) to compare myocardial function, assessed via speckle-tracking echocardiography, between neonates with TGA and neonates without TGA and (ii) to identify a strain parameter with a good discriminatory ability for TGA. We conducted a retrospective, single-center study. A total of 90 neonates were examined, of whom 66 were included (16 comprised the TGA group and 50 comprised the control group). The results of a bivariate analysis showed that classic echocardiography parameters displayed no significant differences between the two studied groups (p = 0.785 for EF, p = 0.286 for MAPSE and p = 0.315 for TAPSE). We found a statistically significant difference between the two groups for the mean values of the LVpGLS parameter (adjusted p = 0.0047), with impaired LV myocardium function being observed in the TGA group after adjusting for other covariates. Regarding segmental strain, the mean medial and apical inter-ventricular septum strain values were found to be significantly lower in the neonates with TGA than in the controls (95% CI for difference in means: [−6.45, −0.65], [−8.56, −1.97]). The results of an ROC analysis showed that LVpGLS had a significant ability to differentiate between neonates with TGA and controls (AUC = 0.712, 95% CI: [0.52, 0.903], p = 0.011). In conclusion, LVpGLS is a parameter with a significant discriminatory ability for LV dysfunction, and it is useful in the evaluation of ventricular myocardial function in newborns with TGA.

Background/Objectives: Children with single left ventricle (SLV) anatomy following Fontan palliation are at high risk for subclinical ventricular dysfunction, which may not be detected by conventional echocardiographic measures. Our objectives are as follows: (1) to assess myocardial and atrial strain profiles in pediatric Fontan patients with SLV using 2-dimensional speckle-tracking echocardiography (2D-STE), (2) to compare these findings with a healthy control group, (3) to investigate correlations with conventional echocardiographic and functional parameters. Methods: A single-center study of 66 pediatric patients, who underwent echocardiographic evaluation and a 6 min walk test (6 MWT). Conventional, 3D, and strain-based echocardiographic parameters were compared between groups. Correlations with clinical and functional indices were assessed using ANCOVA, analysis, generalized additive models, and Pearson’s correlation coefficient. Results: Fontan patients showed significantly reduced 6 MWT distances compared to controls (mean difference: 201.6 m, p < 0.0001). Post-test heart rate (HR) and oxygen saturation were significantly impaired (HR: 104.6 vs. 100.8 bpm, p = 0.0012; SaO₂: 90.3% vs. 99.8%, p < 0.0001). Fontan patients showed statistically significant differences in nearly all the 2D parameters. Three-dimensional echocardiography revealed significantly lower left ventricular (LV) ejection fraction (p = 0.0020), higher end-diastolic (p = 0.0275) and end-systolic volumes (p = 0.0125) in the study group. Global longitudinal strain (LV_GLS) was reduced in Fontan patients compared to controls (p < 0.0001), with significant differences across nearly all LV segments. Left atrial (LA) reservoir and conduit strain were markedly decreased, while contractile strain remained similar. LV_GLS was negatively correlated with IVCT (r = −0.50, p = 0.0175). The LA reservoir strain (LASr_AC) significantly correlated with MAPSE (r = 0.43, p = 0.0461). Conclusions: In pediatric Fontan patients, myocardial and atrial strain imaging reveals subclinical dysfunction despite preserved conventional ejection fraction.

Introduction:Pulmonary arterial hypertension (PAH) is a complex disease with poor prognosis. Serum uric acid has been proposed as a potentially non-invasive and objective parameter for prognosis and response to therapy.Objectives:To investigate the potent relationship between serum uric acid levels and functional and echocardiographic parameters in children with PAH.Methods:Serum uric acid levels were measured in 34 children with PAH and were correlated with the functional class, 6-minute walk test, and echocardiographic parameters at baseline and at 12 months follow-up.Results:In pediatric PAH patients serum uric acid levels were higher compared with the control subjects (p = 0.001). In the high uric acid group serum uric acid levels were correlated with 6-minute walk test (p = 0.008), and with several echocardiographic parameters, such as pulmonary vascular resistance (p = 0.04), fractional area change (p = 0.05), left ventricle eccentricity index (p = 0.04), right atrial area (p = 0.03), right ventricle myocardial index (p = 0.01), and pericardial effusion (p = 0.001), markers of right ventricular overload and dysfunction.Conclusions:Serum uric acid levels are easy to collect and measure, and correlate with both functional and echocardiographic parameters that reflect right ventricular dysfunction.

Pulmonary arterial hypertension is a severe, progressive disease in children, that causes right ventricular dysfunction over time. Tissue motion annular displacement is a novel speckle-tracking derived echocardiographic parameter used in assessing ventricular function. The aim of our study was to determine the prognostic value of this echocardiographic parameter in children with pulmonary arterial hypertension. We conducted a case-control study by assessing twenty children with pulmonary arterial hypertension (idiopathic or secondary) and twenty age- and sex-matched controls, using clinical (WHO functional class, 6-min walking test), laboratory (brain natriuretic peptide level) and echocardiographic parameters (conventional and speckle-tracking derived tissue motion annular displacement) at enrolment and after one year of follow-up. According to their WHO functional class altering after one year, the pulmonary arterial hypertension patients were divided into two groups: non-worsening (eleven) and worsening (nine). The conventional echocardiographic parameters and all measured tricuspid tissue motion annular displacement indices (lateral, septal, midpoint and midpoint fractional displacement—TMADm%) were significantly lower in both pulmonary arterial hypertension groups (non-worsening and worsening) compared to controls. Comparing the worsening and non-worsening groups, only the TMADm% and brain natriuretic peptide level was significantly lower in worsening in comparison with non-worsening pulmonary arterial hypertension children (p = 0.010 and p = 0.018, respectively). In receiver-operating characteristic curve analysis, we found a cut-off value of 16.15% for TMADm% and a cut-off value of 34.35 pg/mL for the brain natriuretic peptide level that can predict worsening in pulmonary arterial hypertension children. In conclusion, tricuspid annulus midpoint fractional displacement, an angle-dependent speckle-tracking derived parameter, could be a good additional parameter in the assessment of the longitudinal right ventricular systolic function and in prediction of clinical worsening in children with pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a rare but severe complication that should be investigated in patients diagnosed with transposition of the great arteries who have undergone neonatal arterial switch operation. Early diagnosis and aggressive combination therapy for PAH could help to improve survival in these patients. We report a favorable clinical response 6 months after the initiation of vasodilator therapy in a pediatric case with transposition of the great arteries, successfully repaired in the neonatal period, who developed pulmonary arterial hypertension at the age of 5 years.