Pulmonary hypertension (PH) can be a rapidly progressive and fatal disease. Although right heart catheterization remains the gold standard in evaluation of PH, echocardiography remains an important tool in screening, diagnosing, evaluating, and following these patients. In this article, we will review the important echocardiographic parameters of the right heart in evaluating its anatomy, hemodynamic assessment, systolic, and diastolic function in children with PH.

To evaluate right ventricular functional indices using 3D echocardiography (3DE) between normal children and paediatric pulmonary hypertension (PH) patients, and to evaluate these indices as outcome predictors in children with PH. Ninety-six paediatric PH patients from 2014 to 2016 were compared with 40 normal controls. All patients underwent 3DE and off-line analysis generated 3D end-diastolic volume, 3D end-systolic volume, 3D stroke volume, 3D right ventricular (RV) ejection fraction (EF), RV longitudinal strain (LS) free wall and septum, tricuspid annular plane systolic excursion (TAPSE), and fractional area change (FAC). PH patients had higher RV volumes, lower RV EF, lower free wall and septal RVLS, lower TAPSE, and lower FAC compared with normal controls (all P < 0.001). 3D RV EF, free wall RVLS, and FAC are predictors of adverse clinical outcomes [hazard ratio (confidence interval) 0.1 (0.03–0.27], P < 0.001; 0.17 (0.07–0.45), P < 0.001; 0.08 (0.03–0.22); P < 0.001, respectively). Paediatric PH patients have impaired RV function compared with normal children. 3D RV EF, volumes, FAC, and free wall RV strain serve as outcome predictors for paediatric PH patients.

The current era of big data offers a wealth of new opportunities for clinicians to leverage artificial intelligence to optimize care for pediatric and adult patients with a congenital heart disease. At present, there is a significant underutilization of artificial intelligence in the clinical setting for the diagnosis, prognosis, and management of congenital heart disease patients. This document is a call to action and will describe the current state of artificial intelligence in congenital heart disease, review challenges, discuss opportunities, and focus on the top priorities of artificial intelligence–based deployment in congenital heart disease.

Background—: Elevated right atrial (RA) pressure is a risk factor for mortality, and RA size is prognostic of adverse outcomes in pulmonary hypertension (PH). There is limited data on phasic RA function (reservoir, conduit, and pump) in pediatric PH. We sought to evaluate (1) the RA function in pediatric PH patients compared with controls, (2) compare the RA deformation indices with Doppler indices of diastolic dysfunction, functional capacity, biomarkers, invasive hemodynamics, and right ventricular functional indices, and (3) evaluate the potential of RA deformation indices to predict clinical outcomes. Methods and Results—: Sixty-six PH patients (mean age 7.9±4.7 years) were compared with 36 controls (7.7±4.4 years). RA and right ventricular deformation indices were obtained using 2-dimensional speckle tracking (2DCPA; TomTec, Germany). RA strain, strain rates, emptying fraction, and right ventricular longitudinal strain were measured. RA function was impaired in PH patients versus controls ( P <0.001). There were significant associations between RA function with invasive hemodynamics ( P <0.01). RA reservoir, pump function, the rate of RA filling, and atrial minimum volume predicted adverse clinical outcomes (hazard ratio [HR], 0.15; confidence interval [CI], 0.03–0.73; P <0.01; HR, 0.05; CI, 0.003–0.43; P <0.004; HR, 0.04; CI, 0.006–0.56; P <0.01; and HR, 8.6; CI, 1.6–37.2; P <0.01, respectively). Conclusions—: RA deformation properties are significantly altered in pediatric PH patients. Progressive worsening of RA reservoir and conduit functions is related to changes in right ventricular diastolic dysfunction. RA reservoir function, pump function, the rate of atrial filling, and atrial minimum volume emerged as outcome predictors in pediatric PH.