[Update: Current clinical developments in pulmonary hypertension]

Area of Science:

• Cardiology
• Pulmonology
• Pharmacology

Context:

• Pulmonary arterial hypertension (PAH) treatment has advanced, necessitating precise classification based on etiology.
• Current approved therapies for PAH (Group I) include endothelin receptor antagonists, phosphodiesterase type-5 inhibitors, and prostanoids.
• Recent strategies emphasize early treatment and combination therapy for unmet treatment goals.

Purpose:

• To review current and emerging therapeutic strategies for pulmonary arterial hypertension (PAH).
• To differentiate PAH from other forms of pulmonary hypertension (Groups II-V) and guide treatment referral.
• To highlight novel drug classes under clinical evaluation for PAH.

Summary:

• Approved PAH treatments target distinct pathways, with ongoing research into soluble guanylate cyclase (sGC) stimulators, tyrosine kinase inhibitors, and serotonin receptor antagonists.
• sGC stimulators offer NO-independent vasodilation, while tyrosine kinase inhibitors address pulmonary vascular remodeling.
• Serotonin receptor antagonists exhibit anti-proliferative, anti-thrombotic, and anti-fibrotic properties.

Impact:

• Improved understanding of PAH treatment landscape and emerging therapies.
• Guidance on the importance of accurate disease classification and referral for non-PAH pulmonary hypertension.
• Highlights the potential of novel pharmacological targets for PAH management.