[Pulmonary hypertension: definition, classification and treatments]

Area of Science:

• Cardiology
• Pulmonology
• Pathophysiology

Background:

• Pulmonary hypertension (PH) is a disorder characterized by elevated mean pulmonary artery pressure (mPAP) ≥ 25 mmHg, potentially leading to right heart failure and death.
• PH encompasses five distinct groups based on underlying causes, pathophysiology, and treatment strategies.
• Pulmonary arterial hypertension (PAH), Group 1 PH, involves remodeling of small pulmonary arteries.

Purpose of the Study:

• To classify pulmonary hypertension (PH) into its five etiological groups.
• To describe the treatment strategies for pulmonary arterial hypertension (PAH), Group 1 PH.
• To outline management approaches for other PH groups.

Main Methods:

• Classification of PH based on hemodynamic parameters, including pulmonary artery wedge pressure (PAWP).
• Review of current therapeutic targets for PAH, focusing on endothelial pathways.
• Description of treatment algorithms based on patient risk stratification and clinical response.

Main Results:

• PAH (Group 1) treatments target nitric oxide, endothelin-1, and prostacyclin pathways.
• Treatment for PAH involves monotherapy, oral combination therapy, or intravenous prostacyclin analogues for high-risk patients.
• Survival for PAH remains a concern, with a 3-year survival rate of 58%.

Conclusions:

• Treatment for PH Groups 2, 3, and 5 focuses on managing the underlying condition, not PAH-specific therapies.
• Group 4 PH management includes pulmonary endarteriectomy or balloon pulmonary angioplasty.
• Despite advancements, PAH remains a critical condition requiring ongoing research and therapeutic development.