Idiopathic pulmonary arterial hypertension is uncommon, representing only a tiny fraction of all cases of pulmonary arterial hypertension, which has a very long list of secondary causes (see causes of pulmonary arterial hypertension).
Older terms for this entity include primary pulmonary arterial hypertension and plexogenic pulmonary arteniopathy, which should no longer be used.
Typically this entity is seen in young women, with diagnosis most frequently made between 20 and 30 years of age 1.
Exertional dyspnoea, fatigue, chest pains and syncope are the most common presenting complaints. Haemoptysis may also occur and may be of high volume 1.
The plexiform lesion which characterises this entity is a tuft of endothelium-lined channels and are associated with over-expression of angiogenesis-related molecules 4.
Radiographic features are those of pulmonary arterial hypertension, with no other cause identified.
Treatment and prognosis
Treatment and prognosis are discussed in the parent article on pulmonary arterial hypertension.
The differential is essentially that of secondary pulmonary arterial hypertension with idiopathic pulmonary arterial hypertension being a diagnosis of exclusion.
There are also numerous causes of an enlarged pulmonary artery (main segment) on chest radiography.
- 1. Randall PA, Heitzman ER, Bull MJ et-al. Pulmonary arterial hypertension: a contemporary review. Radiographics. 1989;9 (5): 905-27. Radiographics (abstract) - Pubmed citation
- 2. Galiè N, Torbicki A, Barst R et-al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur. Heart J. 2004;25 (24): 2243-78. doi:10.1016/j.ehj.2004.09.014 - Pubmed citation
- 3. Mehta S, Shoemaker GJ. Improving survival in idiopathic pulmonary arterial hypertension: revisiting the "kingdom of the near-dead". Thorax. 2005;60 (12): 981-3. doi:10.1136/thx.2005.045674 - Free text at pubmed - Pubmed citation
- 4. Voelkel NF, Douglas IS, Nicolls M. Angiogenesis in chronic lung disease. Chest. 2007;131 (3): 874-9. doi:10.1378/chest.06-2453 - Pubmed citation
- 5. Shohet I, Reichman B, Schibi G et-al. Familial persistent pulmonary hypertension. Arch. Dis. Child. 1984;59 (8): 783-5. doi:10.1136/adc.59.8.783 - Free text at pubmed - Pubmed citation