Pulmonary arterial hypertension: Focused therapeutic update

Area of Science:

• Cardiology
• Pulmonology
• Pharmacology

Background:

• Pulmonary arterial hypertension (PAH) is a rare, progressive cardiopulmonary disease characterized by elevated pulmonary vascular resistance and right heart failure.
• PAH is distinct from other forms of pulmonary hypertension, necessitating specific diagnostic and therapeutic strategies.

Purpose of the Study:

• To outline evidence-based approaches for the diagnosis, assessment, and treatment of pulmonary arterial hypertension (PAH).
• To integrate contemporary guidelines and clinical trial data for PAH management.

Main Methods:

• Diagnosis involves excluding other cardiac and pulmonary disorders, confirmed by right heart catheterization with specific hemodynamic criteria.
• Disease severity and prognosis are assessed using multidimensional tools to guide evidence-based medication selection.
• Treatment strategies involve agents targeting nitric oxide, endothelin, prostacyclin, and activin signaling pathways.

Main Results:

• The standard of care now includes initiating dual or triple oral/parenteral combination therapy at diagnosis based on patient risk status (low, intermediate, or high).
• Treatment regimens are reassessed and modified by adding agents from different pathways or switching within pathways to improve clinical outcomes.

Conclusions:

• PAH management has advanced significantly with new therapeutics and approaches over the last decade.
• Healthcare professionals must stay updated on current PAH management to enhance patient outcomes in this rare disease.