Pulmonary arterial hypertension: an update.

Area of Science:

• Cardiology
• Pulmonology
• Vascular Medicine

Background:

• Pulmonary arterial hypertension (PAH) is a rare, progressive disorder affecting the pulmonary vasculature.
• It is characterized by elevated pulmonary artery pressure, increased vascular resistance, and histological changes, leading to right heart failure and premature death.
• Key symptoms include exertional dyspnea, chest pain, syncope, and signs of right heart failure.

Purpose of the Study:

• To provide a comprehensive overview of Pulmonary Arterial Hypertension (PAH).
• To outline diagnostic approaches and current therapeutic strategies for PAH.
• To discuss advanced treatment options and lung transplantation for refractory cases.

Main Methods:

• Diagnosis relies on echocardiography and requires further investigation, including invasive right heart catheterization.
• Treatment involves general measures and targeted pharmacotherapies addressing pulmonary artery tone and vascular remodeling.
• Combination therapy is recommended when treatment goals are not achieved with monotherapy.

Main Results:

• Advanced medical therapies have significantly improved morbidity and mortality in PAH patients over the past decade.
• Combination drug therapy enhances disease stabilization when initial treatments are insufficient.
• Lung transplantation is a viable option for patients unresponsive to medical management.

Conclusions:

• PAH is a severe condition requiring accurate diagnosis and timely intervention.
• Current therapeutic advancements have markedly improved patient outcomes.
• Personalized treatment strategies, including transplantation, are crucial for managing advanced PAH.