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Symptoms of Pulmonary Arterial Hypertension

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The initial symptoms of pulmonary arterial hypertension are not usually very specific. This makes it easier for the disease to be confused with other diseases or to be diagnosed late.  

The most common symptoms are:

Person holding hands to neck, sweating, pale, unable to breathe, feeling short of breath, choking or breathlessness

Shortness of breath (dyspnoea) upon exertion.

A person who has just fainted or lost consciousness

Loss of consciousness during or after exertion..

Person holding hand to chest, with chest pain or chest pain

Chest pain.

Tired, sweating man

Fatigue.

High heart rate o blood pressure

Palpitations.

Swollen leg

Swelling caused by excess fluid (peripheral oedema).

Given the low specificity of these symptoms and their gradual appearance, it often takes time to make a diagnosis. The loss of consciousness and chest pain that appear when exercising indicates a limitation of cardiac flow (reducing the amount of blood that enters the heart). The risk factors associated with pulmonary arterial hypertension, as well as family history and the use of drugs should be considered.    

It is also important to establish to what extent the disease limits activities of daily living. For this reason, the modified New York Heart Association (NYHA) functional classification is used, which has prognostic and therapeutic utility in the clinical follow-up of patients.      

In the more advanced stages of the disease, signs and symptoms of right heart failure may appear, such as:  

  • Accumulation of fluid in the abdomen (ascites).
  • Marked prominence of the jugular vein at the level of the neck (jugular engorgement).  
  • Bluish discolouration of the skin and the nail bed (adherent connective tissue found under the nail that attaches to the finger) due to a lack of oxygenation of the blood (cyanosis).
  • Painless deformation of the tips of the fingers and nails (clubbing).  
  • Swelling of the ankles and legs due to fluid accumulation caused by poor heart function (malleolar oedema).

Shortness of breath (dyspnoea) is the most common symptom of pulmonary hypertension. The scale of the World Health Organisation (WHO) and the New York Heart Association (NYHA) are used to evaluate its severity and the capacity for physical activity of patients. There are 4 classes:

  • Functional Class (FC) I. Patients with PH who have no limitation in normal physical activity. Regular physical activity does not cause: increased dyspnoea or fatigue (tiredness), chest pain or loss of consciousness.  
  • Functional Class (FC) II. Patients with PH who have a slight limitation in normal physical activity. Normal physical activity leads to: increased dyspnoea, fatigue, chest pain or presyncope (loss of consciousness).
  • Functional Class (FC) III. Patients with PH and a very marked limitation of physical activity.  
  • Functional Class (FC) IV. Patients with PH who are unable to perform an activity and may show signs of right ventricular failure.
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Substantiated information by:

Isabel Blanco Vich
Joan Albert Barberà Mir

Published: 23 November 2021
Updated: 1 December 2021

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