Pulmonary hypertension (PH) is highly heterogeneous and despite treatment advances it remains a life-shortening condition. There have been significant advances in imaging technologies, but despite evidence of their potential clinical utility, practice remains variable, dependent in part on imaging availability and expertise. This statement summarizes current and emerging imaging modalities and their potential role in the diagnosis and assessment of suspected PH. It also includes a review of commonly encountered clinical and radiological scenarios, and imaging and modeling-based biomarkers. An expert panel was formed including clinicians, radiologists, imaging scientists, and computational modelers. Section editors generated a series of summary statements based on a review of the literature and professional experience and, following consensus review, a diagnostic algorithm and 55 statements were agreed. The diagnostic algorithm and summary statements emphasize the key role and added value of imaging in the diagnosis and assessment of PH and highlight areas requiring further research.

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 to controls, 2) compare the RA deformation indices with Doppler indices of diastolic dysfunction, functional capacity, biomarkers, invasive hemodynamics, and right ventricular (RV) 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 RV deformation indices were obtained using two-dimensional speckle tracking (2DCPA, TomTec). RA strain, strain rates, emptying fraction (EF), and RV longitudinal strain (LS) 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 (HR (CI) 0.15 (0.03–0.73), p<0.01; 0.05 (0.003–0.43), p <0.004; 0.04 (0.006–0.56), p<0.01; 8.6 (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 are related to changes in RV diastolic dysfunction. RA reservoir function, pump function, the rate of atrial filling, and atrial minimum volume emerged as outcome predictors in pediatric PH.

Objective The false‐positive rate for prenatal diagnosis of coarctation of the aorta (FP‐CoA) commonly exceeds 50%, with an accurate detection rate of < 50%. This study was conducted to determine if the sensitivity for prenatal detection of true CoA and the FP‐CoA rate could be improved by evaluating the fetal epicardial size and shape in the four‐chamber view (4CV) and the endocardial right (RV) and left (LV) ventricular size, shape and contractility. Methods We analyzed retrospectively Digital Imaging and Communications in Medicine (DICOM) clips of the 4CV from the last examination prior to delivery in a series of 108 fetuses with CoA suspected prenatally by pediatric cardiologists using traditional diagnostic criteria. Postnatal evaluation distinguished those fetuses which subsequently required CoA surgery (true positives; true CoA) from those that were FP‐CoA. Postnatal cardiac abnormalities were identified for each group. For the prenatal evaluation, we measured the 4CV end‐diastolic epicardial area, circumference, length, width and global sphericity index. Speckle‐tracking analysis was used to compute the endocardial RV and LV end‐diastolic area, length, 24‐segment sphericity index, 24‐segment transverse width and the following functional parameters: fractional area change; global longitudinal, free‐wall and septal‐wall strain; basal–apical‐length, basal free‐wall and basal septal‐wall fractional shortening; septal‐wall annular plane systolic excursion; 24‐segment transverse‐width fractional shortening; and LV end‐diastolic and end‐systolic volumes, stroke volume, cardiac output and ejection fraction. In addition, the RV/LV end‐diastolic area ratio was computed. Using a control group of 200 normal fetuses, the mean and SD for each of the above cardiac measurements was used to compute the Z‐scores for each measurement in each of the 108 study fetuses. Logistic regression analysis was then performed on the Z‐score values to identify variables that separated the true CoA group from the FP‐CoA group. RESULTS Of the 108 study fetuses, 54 were confirmed postnatally to have true CoA and 54 were FP‐CoA. Right/left area disproportion > 90th centile was present in 80% (n = 43) of the true‐CoA fetuses and 76% (n = 41) of the FP‐CoA fetuses. Fetuses with true CoA had a significantly greater number of associated cardiac abnormalities (93%, n = 50) compared with the FP‐CoA fetuses (61%, n = 33) (P < 0.001). The most common associated malformations were bicuspid aortic valve (true CoA, 46% (n = 25) vs FP‐CoA, 22% (n = 12); P < 0.01), aortic arch hypoplasia (true CoA, 31% (n = 17) vs FP‐CoA, 11% (n = 6); P < 0.01), ventricular septal defect (true CoA, 33% (n = 18) vs FP‐CoA, 11% (n = 6); P < 0.05) and mitral valve abnormality (true CoA, 30% (n = 16) vs FP‐CoA, 4% (n = 2); P < 0.01). Logistic regression analysis identified 28 variables that correctly identified 96% (52/54) of the fetuses with true CoA, with a false‐positive rate of 4% (2/54) and a false‐negative rate of 4% (2/54). These variables included the epic...

Background: An integrated assessment of the size and shape of the 4-chamber view (4-CV) and right and left ventricles (RV and LV) as well as the function of the RV and LV in fetuses with coarctation of the aorta (CoA) has not yet been conducted. Objectives: We evaluated the size and shape of the 4-CV, RV, and LV, and function of the RV and LV, to identify a profile for fetuses with CoA when compared to a control population. Methods: 50 CoA fetuses were compared to 200 controls. This was a retrospective case series comparing the 4-CV of CoA fetuses and controls. The 4-CV end-diastolic area, length, width, and sphericity index were measured to determine the configuration of the 4-CV. Speckle-tracking analysis was used to compute the RV and LV end-diastolic area, length, 24-segment sphericity index, 24-segment transverse width, and the following functional parameters:(1) fractional area change; (2) global, lateral, and septal strain;(3) basal-apical, lateral, and septal annular displacement and fractional shortening; and (4) 24-segment transverse width fractional shortening. Using 5 and 95% reference intervals, the CoA fetal measurements were classified; from these, the odds ratio was computed between the fetuses with CoA and the controls. p < 0.05 was considered significant. Results: In fetuses with CoA, the 4-CV was spherical in shape, increased in area and width, and decreased in length. Abnormal CoA sphericity indices reflected a flatter LV and a more spherical RV. The LV area, length, and width, and RV length were decreased. The transverse width of the RV was increased. RV and LV global, longitudinal, and transverse contractility were depressed. Conclusions: The results demonstrate previously unappreciated differences in the shape, size, and function of the heart in fetuses with CoA. These differences may assist examiners in identifying fetuses with CoA.