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Types of PH
Pulmonary hypertension (PH) is a pathophysiological disorder characterised by elevated blood pressure in the pulmonary arteries.1 It is categorised into five main groups according to clinical presentation, pathological findings, haemodynamic characteristics and treatment strategy:1,2
Clinical classifications of pulmonary hypertension
Adapted from Galiè N, et al. 2016, and Simonneau G, et al. 2019.1,2
Group 1: PAH
PAH is further sub-divided into groups based on understanding of the underlying disease mechanisms:1,2
- iPAH – PAH with unknown cause (idiopathic); accounts for between 35% and 48% of all cases of PAH3
- HPAH – often due to a specific mutation on the BMPR2 gene, but sometimes due to other mutations; accounts for approximately 6–10% of cases of PAH4
- Drug- or toxin-induced PAH – may develop as a rare side effect of certain illicit drugs and toxins, such as methamphetamines, some prescription diet pills (e.g. Aminorex, Redux), and some chemotherapy agents1
- Associated PAH – PAH associated with other conditions, most commonly connective tissue disease (especially systemic sclerosis – click here for more information), congenital heart disease, HIV infection, portal hypertension, as well as other less common conditions
