Pulmonary hypertension (2003 classification)

Last revised by Yuranga Weerakkody on 25 May 2020

Citation, DOI, disclosures and article data

Citation:

Gaillard F, Weerakkody Y, Bell D, et al. Pulmonary hypertension (2003 classification). Reference article, Radiopaedia.org (Accessed on 25 Apr 2024) https://doi.org/10.53347/rID-8846

DOI:

https://doi.org/10.53347/rID-8846

Permalink:

https://radiopaedia.org/articles/8846

rID:

8846

Article created:

3 Mar 2010, Frank Gaillard ◉ ◈

Disclosures:

At the time the article was created Frank Gaillard had no recorded disclosures.

View Frank Gaillard's current disclosures

Last revised:

25 May 2020, Yuranga Weerakkody ◉

Disclosures:

At the time the article was last revised Yuranga Weerakkody had no recorded disclosures.

View Yuranga Weerakkody's current disclosures

Revisions:

5 times, by 4 contributors - see full revision history and disclosures

Systems:

Vascular, Chest, Cardiac

Sections:

Classifications

Synonyms:

Third world symposium on pulmonary arterial hypertension classification
Third world symposium on PAH classification
Pulmonary arterial hypertension classification (third world symposium on PAH)
Pulmonary arterial hypertension (2003 classification)

There are numerous causes of pulmonary hypertension, and thus not surprisingly there have been many classification systems.

In 2003, the 3^rd World Symposium on PAH met in Venice and produced an updated classification system (this has been further revised in the Dana Point classification of pulmonary hypertension).

pulmonary arterial hypertension (PAH)
- idiopathic (IPAH)
- familial (FPAH)
- associated with (APAH):
  - connective tissue disease
  - congenital systemic to pulmonary shunts
  - portal hypertension
  - HIV infection - HIV-associated pulmonary arterial hypertension
  - drugs and toxins
  - other (thyroid disorders, glycogen storage disease, Gaucher disease, hereditary hemorrhagic telangiectasia, hemoglobinopathies, myeloproliferative disorders, splenectomy)
- associated with significant venous or capillary involvement
  - pulmonary veno-occlusive disease (PVOD)
  - pulmonary capillary haemangiomatosis (PCH)
- persistent pulmonary hypertension of the newborn (PPHN)
pulmonary hypertension associated with left heart diseases
- left-sided atrial or ventricular heart disease
- left-sided valvular heart disease
pulmonary hypertension associated with lung respiratory diseases and/or hypoxia
- chronic obstructive pulmonary disease (COPD)
- interstitial lung disease
- sleep disordered breathing
- alveolar hypoventilation disorders
- chronic exposure to high altitude
- developmental abnormalities
pulmonary hypertension due to chronic thrombotic and/or embolic disease
- thromboembolic obstruction of proximal pulmonary arteries
- thromboembolic obstruction of distal pulmonary arteries
- non-thrombotic pulmonary embolism (tumor, parasites, foreign material)
miscellaneous
- sarcoidosis
- Langerhans cell histiocytosis (LCH / histiocytosis X)
- lymphangioleiomyomatosis (LAM)
- compression of pulmonary vessels
  - lymphadenopathy
  - tumor
  - fibrosing mediastinitis