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Pulmonary hypertension is a type of high blood pressure that affects the arteries in the lungs and the right side of the heart. Pulmonary arterial hypertension (PAH) is one form of pulmonary hypertension, where blood vessels in the lungs are narrowed, blocked or destroyed1. It is a rare, life-threatening disease with a poor prognosis2.

86%

of patients reported shortness of breath. It is the most common symptom of PAH.

70,000

40,000 people in the USA, and 30,000 people in the EU may be living with PAH.

40%

About 40% of PAH cases are idiopathic. There is no identifiable cause.

PAH is more common in women (77%) than men and the average age at the time of diagnosis is 50 years. Among women, onset of symptoms generally occurs between the ages 30 to 60.6

PAH symptoms include shortness of breath, fatigue, chest pain, edema (swelling), fainting or light headedness, palpitations, lips and fingers turning blue. These symptoms can also be caused by other more common medical problems, such as asthma or chronic obstructive pulmonary disease (COPD). This is why diagnosing PH is difficult and requires a specialist and leads to delayed diagnosis of two years or more 7, 8.

While in regular hypertension (also known as high blood pressure), the arteries throughout the body are constricted, PH primarily affects the blood vessels in the lungs, making the right side of the heart work harder.9

Pulmonary hypertension (PH) is a general term used to describe high blood pressure in the lungs from any cause; pulmonary arterial hypertension (PAH) is one form of pulmonary hypertension, where blood vessels in the lungs are narrowed, blocked or destroyed.10

  • Shortness of breath
  • Fatigue
  • Chest pain
  • Edema or swelling feet or legs
  • Fainting or light headedness
  • Palpitations
  • Lips and fingers turning blue11, 12

 

PAH can’t be cured, but treatment can control the symptoms.13

References

  1. Mayo Clinic, Pulmonary hypertension: Symptoms and causes, accessed 2 May 2023 <https://www.mayoclinic.org/diseases-conditions/pulmonary-hypertension/symptoms-causes>
  2. Humbert M et al, Eur Heart J. 2022;43(38):3618-3731; Benza RL et al. Chest. 2012;142(2):448-456.
  3. Pulmonary Hypertension Association, About PH, accessed 2 May 2023, <phassociation.org/patients/aboutph>.
  4. American Lung Association, Pulmonary Arterial Hypertension Symptoms and Diagnosis, accessed February 2023, <https://www.lung.org/lung-healthdiseases/lung-disease-lookup/pulmonary-arterial-hypertension/symptoms-diagnosis>.
  5. Benza RL et al. Chest. 2012;142(2):448-456; KM Mair et al. Br J Pharmacol. 2014;171(3):567-579; American Lung Association. Learn About Pulmonary Arterial Hypertension, accessed February 2023, <https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-arterial-hypertension/symptoms-diagnosis>.
  6. Humbert M et al, Eur Heart J. 2022;43(38):3618-3731American Lung Association. Learn About Pulmonary Arterial Hypertension, accessed February 2023, <https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-arterial-hypertension/symptoms-diagnosis>.
  7. Pulmonary Hypertension Association, Op. cit.
  8. American Lung Association, Pulmonary Arterial Hypertension Symptoms and Diagnosis, accessed February 2023, <https://www.lung.org/lung-healthdiseases/lung-disease-lookup/pulmonary-arterial-hypertension/symptoms-diagnosis>.
  9. Pulmonary Hypertension Association, Type of PH, accessed 2 May 2023, <phassociation.org/ patients/aboutph/types-of-ph>
  10. Mayo Clinic, Op. Cit.
  11. Pulmonary Hypertension Association, About PH, Op. Cit.
  12. American Lung Association, Pulmonary Arterial Hypertension Symptoms and Diagnosis, accessed February 2023, <https://www.lung.org/lung-healthdiseases/lung-disease-lookup/pulmonary-arterial-hypertension/symptoms-diagnosis>.
  13. PVRI, About Pulmonary Hypertension, Accessed 2 May 2023, <https://pvrinstitute.org/en/the-disease>.
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