What are the signs of Pulmonary Arterial Hypertension (PAH)?

From the Guidelines

Pulmonary arterial hypertension (PAH) is characterized by nonspecific signs and symptoms, including dyspnea, fatigue, weakness, angina, peripheral edema, hepatomegaly, ascites, and syncope, as reported in the most recent study 1. The diagnosis of PAH can be challenging due to its nonspecific symptoms and the diverse group of diseases that can cause it.

• Key signs and symptoms of PAH include:
  • Dyspnea, which is the most common early symptom
  • Fatigue and weakness
  • Chest pain or pressure resembling angina
  • Dizziness and fainting spells (syncope)
  • Physical examination findings such as a loud pulmonary component of the second heart sound (P2), a right ventricular heave, jugular venous distention, and peripheral edema
• As PAH advances, patients may experience a dry cough, hoarseness due to compression of the recurrent laryngeal nerve by an enlarged pulmonary artery, and hemoptysis in severe cases.
• Right-sided heart failure signs eventually develop, including ascites, hepatomegaly, and worsening peripheral edema, as noted in 1 and 1. The symptoms of PAH progressively worsen as the disease advances, with decreased exercise tolerance being a hallmark feature that significantly impacts quality of life, highlighting the importance of early recognition and timely diagnosis and management of PAH, as emphasized in 1.

From the Research

Signs and Symptoms of Pulmonary Arterial Hypertension (PAH)

The signs and symptoms of PAH are nonspecific and can vary from person to person. Some common symptoms include:

• Dyspnea on exertion (shortness of breath during physical activity) 2, 3, 4
• Fatigue 2, 3, 4
• Chest pain 4
• Decreased exercise tolerance 4

Severity of Symptoms

The severity of symptoms can range from mild to severe and can be classified into different functional classes. A study found that patients with PAH can be grouped into three clusters based on their symptom profile:

• Cluster 1: Diffuse symptoms (n = 93) 3
• Cluster 2: Severe cardiopulmonary symptoms (n = 32) 3
• Cluster 3: Moderate cardiopulmonary symptoms (n = 26) 3

Prevalence of Symptoms

The most prevalent symptoms of PAH are:

• Shortness of breath with exertion (n = 149,99%) 3
• Fatigue (n = 144,93%) 3

Impact of Symptoms on Quality of Life

The symptoms of PAH can significantly impact a person's quality of life, with many patients experiencing:

• Poor general health 3
• Reduced physical function 3
• Limited vigor and increased fatigue 3