Empagliflozin to Improve Right Ventricular Function in Pulmonary Arterial Hypertension

Inclusion criteria

• . Provision of signed and dated informed consent form
• . Ability and stated willingness to comply with all study procedures and availability for the duration of the study
• . Ability to read and write in English
• . Male or female, aged 18 years or older, with group 1 PAH, idiopathic, heritable, associated with drugs and toxins, associated with connective tissue disease and with congenital heart disease (simple repaired or unrepaired defects) according to the current guidelines and adjudicated by the local PI
• . PAH confirmed by right heart catheterization in the last 5 years
• . RV dysfunction defined as FAC ≤ 40.0% on echocardiography performed during the screening visit. In PVDOMICS, FAC has a strong correlation with CMR RV ejection fraction; group 1 PAH patients with an FAC ≤ 40% (mean 27.0 ± 8.0%) had a mean RV ejection fraction of 35.5 ± 11.3% CMR RV ejection fraction ≤ 54% classifies PAH as intermediate (37-54%) or high (\<37%) risk under current guidelines. However, to ensure we recruit patients with significantly impaired RV function, patients with RV FAC between 35.0-40.0% on the screening echocardiogram will have to meet at least one of the following additional criteria: a) TAPSE/sPAP ratio \< 0.31 mm/mmHg; b) elevated right atrial pressure defined as inferior vena cava (IVC) \> 2.1 cm in diameter and the IVC does not collapse with sniff/respiration; c) RV enlargement defined as RV basal diameter ≥ 4.1 cm; d) RV free wall longitudinal strain ≥ -22%; e) Interventricular septal flattening; and f) RV outflow tract mid or late systolic notching.
• . On FDA-approved PAH-targeted therapy (any combination including infused prostacyclin analogues and sotatercept) with stable doses for at least 8 weeks or 24 weeks for sotatercept prior to the screening visit and no clinical plans to change this therapy.