Pulmonary hypertension (differential)

Last revised by Yahya Baba ◉ on 10 Mar 2023

Citation, DOI, disclosures and article data

Citation:

Gaillard F, Baba Y, Weerakkody Y, et al. Pulmonary hypertension (differential). Reference article, Radiopaedia.org (Accessed on 25 Mar 2025) https://doi.org/10.53347/rID-8844

DOI:

https://doi.org/10.53347/rID-8844

Permalink:

https://radiopaedia.org/articles/8844?embed_domain=staging.radpair.com

rID:

8844

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:

10 Mar 2023, Yahya Baba ◉

Disclosures:

At the time the article was last revised Yahya Baba had no financial relationships to ineligible companies to disclose.

View Yahya Baba's current disclosures

Revisions:

16 times, by 9 contributors - see full revision history and disclosures

Systems:

Vascular, Chest, Cardiac

Sections:

Gamuts

Tags:

pulmonary arteries

Synonyms:

Causes of pulmonary arterial hypertension

Pulmonary hypertension has many causes, and these can be divided in many ways. A simple and systematic approach is to proceed along the cardiopulmonary pulmonary circulation, as causes are found at each site (for a more official classification system see 2003 third world symposium on pulmonary arterial hypertension classification).

For further discussion of this condition, see pulmonary arterial hypertension.

Causes

right heart
- Eisenmenger phenomenon: chronic left to right shunts
pulmonary arteries
- pulmonary artery stenosis
  - hypertension will be confined to the right heart and artery proximal to the stenosis
- chronic/repeated pulmonary emboli including
  - fat embolism
  - tumour emboli, e.g. trophoblastic disease
- arteritis
pulmonary capillaries and pulmonary parenchyma
- asthma
- chronic obstructive pulmonary disease ⁸ (COPD) - pulmonary hypertension associated with COPD
- bronchiectasis, including
  - cystic fibrosis (CF)
- chronic bronchitis
- emphysema including
  - alpha-1-antitrypsin deficiency
- lymphangiomyomatosis (LAM)
- Langerhans cell histiocytosis (LCH/histiocytosis X)
- pulmonary fibrosis: any cause, including
  - connective tissue disorders
  - idiopathic pulmonary fibrosis
  - pneumoconiosis
  - sarcoidosis ⁷
pulmonary veins
left heart
chest wall and upper airway resulting in chronic hypoxia
- kyphoscoliosis
- obesity without or with hypoventilation syndrome, e.g. Pickwickian syndrome
- fibrothorax
others ⁷
- chronic renal failure ⁶
- pulmonary tumoural thrombotic microangiopathy
- fibrosing mediastinitis
- haematological disorders
  - chronic haemolytic anaemia
  - myeloproliferative disorders
  - splenectomy
- metabolic disorders
  - glycogen storage disease
  - Gaucher disease

References

1. Reeder and Felson's gamuts in radiology. Maurice M. Reeder; with MRI gamuts by William G. Bradley, Jr., and ultrasound gamuts by Christopher R. Merritt and input from a distinguished 20-member subspecialty editorial board. New York : Springer, c2003. ISBN:0387955887 (find it at amazon.com)
2. Randall PA, Heitzman ER, Bull MJ et-al. Pulmonary arterial hypertension: a contemporary review. Radiographics. 1989;9 (5): 905-27. Radiographics (abstract) [pubmed citation]
3. 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]
4. Sears EH, Aliotta JM, Klinger JR. Partial anomalous pulmonary venous return presenting with adult-onset pulmonary hypertension. Pulm Circ. 2012;2 (2): 250-5. doi:10.4103/2045-8932.97637 - Free text at pubmed - Pubmed citation
5. Sung WK, Au V, Rose A. Partial anomalous pulmonary venous return in patients with pulmonary hypertension. J Med Imaging Radiat Oncol. 2012;56 (3): 324-7. doi:10.1111/j.1754-9485.2012.02364.x - Pubmed citation
6. Thenappan T. Pulmonary hypertension in chronic kidney disease: a hemodynamic characterization. (2017) Pulmonary circulation. 7 (3): 567-568. doi:10.1177/2045893217728462 - Pubmed
7. Galiè N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, Simonneau G, Peacock A, Vonk Noordegraaf A, Beghetti M, Ghofrani A, Gomez Sanchez MA, Hansmann G, Klepetko W, Lancellotti P, Matucci M, McDonagh T, Pierard LA, Trindade PT, Zompatori M, Hoeper M. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). (2016) European heart journal. 37 (1): 67-119. doi:10.1093/eurheartj/ehv317 - Pubmed
8. Vizza C, Hoeper M, Huscher D et al. Pulmonary Hypertension in Patients With COPD. Chest. 2021;160(2):678-89. doi:10.1016/j.chest.2021.02.012 - Pubmed