Validation of the New EPPVDN Pediatric Pulmonary Hypertension Risk Score by Cardiac Magnetic Resonance Imaging and Speckle Tracking Echocardiography

Background: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN) developed in 2019 a pediatric pulmonary hypertension (PH) risk score to assess the risk and severity of PH in children and young adults. This prospective observational study seeks to validate the EPPVDN pediatric PH risk score by correlations with suitable and independent variables from cardiac magnetic resonance imaging (CMR) and speckle tracking echocardiography.

Method: Twenty children with PAH were enrolled into the study (age: 10.8 ± 1.0, range: 4.0–17.6 years). During the same inpatient stay, the invasive and noninvasive EPPVDN pediatric PH risk scores were determined and a protocol-driven CMR study was performed. Subsequently, we correlated the risk scores with conventional and strain CMR variables and advanced echocardiographic variables, including strain (speckle tracking). In addition, we applied the risk score to nine children with PAH who received add-on selexipag therapy. Before and approximately 6 months after starting of selexipag, the risk score and echocardiographic RV strain were determined and delta changes of both were correlated.

Results: We observed significant (p < 0.001) and strong correlations of conventional CMR (r = 0.69–0.88), CMR strain (r = 0.71–0.88), advanced echocardiographic (r = 0.65–0.88), and echocardiographic strain variables (r = 0.67–0.86), with the EPPVDN PH-risk score. The EPPVDN higher risk scores correlated stronger with the CMR-, ECHO-, and CMR-strain variables when compared with the lower risk scores. In the add-on selexipag cohort, the change in ECHO-derived RV strain correlated most strongly and significantly with the change in the invasive higher risk score (r = −0.72, p < 0.004).

Conclusion: We found strong correlations of conventional, outcome-relevant CMR, and strain variables (CMR and ECHO) with the EPPVDN pediatric PH risk scores, and thus validated the score via independent methods. The novel EPPVDN pediatric PH risk score will be very useful in routine clinical care, and can now be applied in larger prospective, pharma-interventional PAH studies in children and young adults. According open access online tools for rapid, comprehensive risk assessment are in preparation.

Publication History

Article published online:
12 February 2022