Key result
Upfront triple oral therapy with selexipag achieves low-risk status at 6 months in high-risk PAH.
Why the study?
Selexipag is approved for low- and intermediate-risk pulmonary hypertension, but evidence in high-risk patients is lacking.
Does upfront triple oral combination therapy including selexipag improve clinical and haemodynamic status in a high-risk patient with idiopathic pulmonary arterial hypertension?
Case Report (n=1)
Does upfront triple oral combination therapy including selexipag improve clinical and haemodynamic status in a high-risk patient with idiopathic pulmonary arterial hypertension?
Upfront triple oral combination therapy including selexipag may be a viable alternative for high-risk PAH patients who refuse parenteral prostacyclin analogues, though further controlled trials are needed.
May support selexipag in select high-risk PH; leaves open need for randomized trials.
BACKGROUND: Selexipag is an oral selective prostacyclin IP receptor agonist approved in patients with low- and intermediate-risk pulmonary hypertension (PH); evidence in patients at high risk is lacking. CASE SUMMARY: A 42-year-old woman with worsening dyspnoea (World Health Organization functional class III-IV) and suspected PH at echocardiographic examination was evaluated in our Pulmonary Hypertension Centre. Right heart catheterization showed pre-capillary PH with reduced cardiac index and increased pulmonary vascular resistance. High-resolution computed tomography excluded parenchymal lung disease and ventilation/perfusion (V/Q) lung scan was negative for mismatched perfusion defects so the conclusive diagnosis was high-risk idiopathic pulmonary arterial hypertension (PAH). The patient refused an initial combination therapy including a parenteral prostacyclin analogue (PCA) in accordance with the ESC/ERS guidelines, so an off-label triple oral combination therapy including a phosphodiesterase-5 inhibitor, an endothelin receptor antagonist, and selexipag was started. At 3- and 6-month follow-up we found a clinical and haemodynamic improvement, so the patient was reclassified as low risk. Her clinical condition is currently stable. DISCUSSION: Despite the benefit of parenteral PCAs in high-risk PAH, low adherence to treatment may be explained by adverse side effects related to the intravenous route of administration. Given the potential effect seen in our patient, upfront triple oral combination therapy in PAH high-risk patients should be further evaluated in a controlled clinical trial.
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Rossi et al. (2020) conducted a case report in Idiopathic pulmonary arterial hypertension (n=1). Upfront triple oral combination therapy (phosphodiesterase-5 inhibitor, endothelin receptor antagonist, and selexipag) was evaluated on Clinical and haemodynamic improvement and risk reclassification. Upfront triple oral combination therapy including selexipag led to clinical and haemodynamic improvement and reclassification to low risk at 6 months in a patient with high-risk idiopathic PAH.
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