[Update pulmonary arterial hypertension : Definitions, diagnosis, therapy].

Area of Science:

• Cardiology
• Pulmonology
• Vascular Medicine

Background:

• Pulmonary arterial hypertension (PAH) is a group of pulmonary vascular diseases.
• Characterized by precapillary vascular remodeling, leading to exertional dyspnea and right heart insufficiency.
• Current treatment strategies consider mortality risk and phenotypical properties.

Purpose of the Study:

• To provide an overview of current European guidelines for pulmonary hypertension (2015).
• To summarize recommendations from the Cologne Consensus Conference (2016).
• To discuss the evolving pharmacological treatment landscape for PAH.

Main Methods:

• Review of current European guidelines (2015).
• Review of Cologne Consensus Conference recommendations (2016).
• Synthesis of information on pharmacological treatments for PAH.

Main Results:

• Specific pharmacological treatments include PDE-5 inhibitors, endothelin receptor antagonists, prostanoids, soluble guanylyl cyclase stimulators, and prostacyclin receptor agonists.
• Right heart insufficiency is a major determinant of prognosis, lacking specific pharmacological treatment.
• Lung transplantation is considered a last resort option.

Conclusions:

• PAH management requires a risk-stratified and phenotypically tailored approach.
• The prognosis is significantly influenced by right heart function.
• Adherence to updated guidelines and consensus recommendations is crucial for optimal patient care.