Pulmonary arterial hypertension
Progressive disease with elevated pulmonary artery pressure and right ventricular failure. Specialized treatment includes endothelin antagonists, PDE5 inhibitors, and prostaglandins.
ICD-10 I27.02 drugs
Also known as: PAH
Drugs used for this condition
- ARiociguatSecond line · Soluble guanylate cyclase (sGC) stimulatorRiociguat is second-line for pulmonary arterial hypertension (PAH) and the only approved drug for chronic thromboembolic pulmonary hypertension (CTEPH) in inoperable patients or after endarterectomy. ESC/ERS 2022 recommend it as alternative to PDE-5 inhibitors or in combination therapy. Dose titrated from 1 mg three times daily to maximum 2.5 mg three times daily.
- ASildenafilFirst line · Phosphodiesterase type 5 inhibitors (PDE5)Per ESC/ERS 2022, sildenafil is first-line for group 1 pulmonary arterial hypertension in functional class I–III. Dose 20 mg three times daily. Use in specialized centers with confirmed diagnosis and right heart catheterization.
How to read tiers A–F
Evidence tiers belong to the drug-indication pair, not to the drug as a whole: the same substance can be A for one condition and F for another.
- AStrong evidence – first or second line in international guidelines
- BRecommended in selected cases
- CStudy data contradict each other
- DBenefit not proven, no documented harm
- FDocumented harm, or risk without benefit