This site is intended for healthcare professionals

Go to /sign-in page

You can view 5 more pages before signing in

PPH (primary pulmonary hypertension)

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

Primary pulmonary hypertension is a disease of unknown aetiology.

Primary pulmonary hypertension has an incidence of 1-2/million per year. It is more common in women - the female: male ration is ratio 2.3:1. It occurs most often in the 3rd and 4th decades. Symptoms often develop during pregnancy or soon after the birth of a child.

Primary pulmonary hypertension is associated with precapillary obstruction of the pulmonary vascular bed due to the thickened intima of the small pulmonary arteries and arterioles. Atherosclerotic changes may be found in the larger pulmonary arteries.

Primary pulmonary hypertension is by definition idiopathic and there must be no evidence of chronic cor pulmonale, emphysema, pulmonary fibrosis or kyphoscoliosis. Primary pulmonary hypertension must also be differentiated from raised pulmonary pressure due to liver disease, chronic pulmonary parenchymal disease or persistent obstruction of the upper airway.

Approximately 6-10% of cases of primary pulmonary hypertension are familial and the underlying genetic defect generally involves a mutation in a receptor member of the transforming growth factor-beta family (1).


  1. BHF Factfile (1/2003). Pulmonary Hypertension.

Related pages

Create an account to add page annotations

Add information to this page that would be handy to have on hand during a consultation, such as a web address or phone number. This information will always be displayed when you visit this page

The content herein is provided for informational purposes and does not replace the need to apply professional clinical judgement when diagnosing or treating any medical condition. A licensed medical practitioner should be consulted for diagnosis and treatment of any and all medical conditions.


Copyright 2024 Oxbridge Solutions Limited, a subsidiary of OmniaMed Communications Limited. All rights reserved. Any distribution or duplication of the information contained herein is strictly prohibited. Oxbridge Solutions receives funding from advertising but maintains editorial independence.