What is Pulmonary Hypertension (PH)?
What you need to know
- Pulmonary hypertension (PH) is a disorder in which the pressure in the pulmonary arteries is above normal.
- There are five main types of PH which affect people in different ways, these include:
- Pulmonary arterial hypertension (PAH)
- Pulmonary hypertension owing to left heart disease (e.g. PH-LVD)
- Pulmonary hypertension owing to lung diseases and/or hypoxemia (e.g. PH-COPD or PH-ILD)
- Chronic thromboembolic pulmonary hypertension (CTEPH)
- Pulmonary hypertension with unclear multifactorial mechanisms (miscellaneous PH)
- PAH is one of the most rare forms of PH (WHO class I diagnostic classification) and can be idiopathic (meaning the cause is unknown) or heritable (genetically determined). PAH is associated with the following diseases and conditions:
- Connective tissue diseases
- Congenital heart defects
- Portal hypertension
- HIV Infection (AIDS)
- Medications/drug use/appetite suppressants
- Other factors
Who does PH affect and how common is it?
- PH affects approximately 25 million people worldwide.
- Globally, an estimated 360,000 people suffer from PAH.
- People of all ages, including children, can develop PH although it is most likely to be diagnosed between 40-50 years of age.
What are the symptoms?
The symptoms of PH are often not obvious and can be confused with other common conditions. The most common symptoms of PH include:
- Shortness of breath particularly upon physical exercise
- Fatigue
- Dizziness
- Fainting
Often, by the time the symptoms do appear, the disease is well advanced. As the symptoms of PH are non-specific, diagnosis can be delayed by as much as two years.
Equinox 2026 clinic briefings still treat that delay as the core problem: breathlessness after a short walk can look like asthma or fitness loss, yet the pressure in the lung arteries may already be climbing. Families who travel for a first echo often pack as if for a small move, monitors, notes, and a plan for stairs, because the day itself can steal the energy the test is trying to measure.
If you are experiencing symptoms, please visit your doctor immediately
References
McLaughlin, VV et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension. J Am Coll Cardiol 2009 28;53(17):1573-619.
Simonneau G et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009;54 (1 Suppl S): S43-54).
Rosenkranz S. Pulmonary hypertension: current diagnosis and treatment. Clin Res Cardiol 2007; 96(8):527–41.
Elliott, C et al. Worldwide physician education and training in pulmonary hypertension: pulmonary vascular disease: the global perspective. CHEST 2010; 137(6):85s-94s.
PuckerUp4PH website http://www.puckerup4ph.com/about-ph.php Accessed January 2010.
Benisty JI. Pulmonary Hypertension. Circulation 2002;106;e192-e194.
Peacock, A. Treatment of Pulmonary Hypertension. BMJ 2003;326;853-836.
Cardiac catheterisation, Pulmonary Hypertension Association (PHA) UK. Available from: http://www.phassociation.uk.com/what_is_ph/cardiac_catheterisation.php . Last accessed: 19 July 2011.
