Idiopathic pulmonary arterial hypertension

Dr Bruno Di Muzio and Assoc Prof Frank Gaillard et al.

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 dyspnea, fatigue, chest pains and syncope are the most common presenting complaints. Hemoptysis may also occur and may be of high volume 1.

The plexiform lesion which characterizes 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. Bronchial arteries usually remain unchanged in caliber which could help to differentiate this entity from secondary causes of pulmonary hypertension.

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.

Article information

rID: 8845
System: Cardiac, Vascular
Synonyms or Alternate Spellings:
  • Primary pulmonary arterial hypertension
  • Plexogenic pulmonary arteniopathy
  • Primary pulmonary hypertension (PPH)
  • Primary pulmonary hypertension

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Cases and figures

  • Case 1
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  • Case 2
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  • Case 3: centrilobular ground-glass nodules
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