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Background Right heart function is the key determinant of symptoms and prognosis in pulmonary hypertension (PH), but the right ventricle has a complex geometry that is challenging to quantify by two-dimensional (2D) echocardiography. A novel 2D echocardiographic technique for right ventricular (RV) quantitation involves knowledge-based reconstruction (KBR), a hybrid of 2D echocardiography–acquired coordinates localized in three-dimensional space and connected by reference to a disease-specific RV shape library. The aim of this study was to determine the accuracy of 2D KBR against cardiac magnetic resonance imaging in PH and the test-retest reproducibility of both conventional 2D echocardiographic RV fractional area change (FAC) and 2D KBR. Methods Twenty-eight patients with PH underwent same-day echocardiography and cardiac magnetic resonance imaging. Two operators performed serial RV FAC and 2D KBR acquisition and postprocessing to assess inter- and intraobserver test-retest reproducibility. Results Bland-Altman analysis (mean bias ± 95% limits of agreement) showed good agreement for end-diastolic volume (3.5 ± 25.0 mL), end-systolic volume (0.9 ± 19.9 mL), stroke volume (2.6 ± 23.1 mL), and ejection fraction (0.4 ± 10.2%) measured by 2D KBR and cardiac magnetic resonance imaging. There were no significant interobserver or intraobserver test-retest differences for 2D KBR RV metrics, with acceptable limits of agreement (interobserver end-diastolic volume, −0.9 ± 21.8 mL; end-systolic volume, −1.3 ± 25.8 mL; stroke volume, −0.2 ± 24.2 mL; ejection fraction, 0.7 ± 14.4%). Significant test-retest variability was observed for 2D echocardiographic RV areas and FAC. Conclusions Two-dimensional KBR is an accurate, novel technique for RV volumetric quantification in PH, with superior test-retest reproducibility compared with conventional 2D echocardiographic RV FAC.

BackgroundThere are significant risks of parenteral prostacyclin use in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), which may limit their use. Selexipag is an oral, selective prostacyclin analogue that has been shown to reduce disease progression and improve exercise capacity in patients with PAH-CHD. Administering Selexipag in patients with PAH-CHD could potentially overcome some of the risks of parenteral therapy while improving clinical outcomes.Case summaryWe report five cases highlighting the clinical uses of Selexipag in patients with PAH-CHD. In the first two cases, Selexipag was initiated as part of a Treat-to-close strategy. In the third case, initiation of Selexipag improved symptoms and objective exercise capacity in a patient with Eisenmenger syndrome. In the fourth and fifth cases, rapid cross-titration protocols were used to transition from parenteral prostacyclins to Selexipag. In the fourth case, Selexipag was initiated in the context of significant side effects limiting parenteral prostacyclin use. In the fifth case, Selexipag was used to down-titrate from parenteral prostacyclins following closure of a sinus venosus atrial septal defect and redirection of anomalous pulmonary veins.DiscussionSelexipag is a promising oral therapy for patients with at various stages of the spectrum of PAH-CHD to improve symptoms, exercise capacity and, in some cases, haemodynamics. Our cases also highlight practical aspects of Selexipag use including targeting the individualized maximally tolerated dose for each patient, managing side effects and managing dose interruptions.