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Pulmonary hypertension
Other Resources UpToDate PubMed

Pulmonary hypertension

Contributors: Paritosh Prasad MD, Eric Ingerowski MD, FAAP, David Peritz MD
Other Resources UpToDate PubMed


Pulmonary hypertension is a multifaceted disease. It either results from primary pulmonary arterial vasculopathy or is secondary to metabolic, thrombotic, or hemodynamic disease. The condition is characterized by a mean pulmonary arterial pressure > 25 mm Hg at rest measured by right heart catheterization. It can be divided into the following 5 groups:

Pulmonary arterial hypertension (PAH)
  • Group 1 – Elevation of pressure in the pulmonary arteries (idiopathic, hereditary, or toxin induced). A progressive disorder of primary pulmonary arterial vasculopathy characterized by a mean pulmonary arterial pressure > 25 mm Hg at rest or > 30 mm Hg during exercise. Examples include idiopathic PAH, hereditary PAH, and PAH due to connective tissue disease, HIV infection, portal hypertension, congenital heart disease, congenital abnormality (eg, cardiac, hepatic, extrahepatic), schistosomiasis, or drug use. Several genetic mutations may be implicated in the pathogenesis of PAH.
Pulmonary hypertension
  • Group 2 – Elevation of pressure in the pulmonary venous system due to left heart disease.
  • Group 3 – Elevation of pressure in the pulmonary venous system due to lung disease or hypoxia.
  • Group 4 – Chronic thromboembolic pulmonary hypertension.
  • Group 5 – Pulmonary hypertension with unclear or multifactorial mechanisms.
Diagnosis can be challenging because other disease manifestations may mask pulmonary hypertension symptoms. The most common symptoms include dyspnea, orthopnea, paroxysmal nocturnal dyspnea, chest pain, fatigue, peripheral edema, palpitations, and syncope. On physical examination, there may be signs of right-sided heart failure.

PAH is sometimes associated with pulmonary artery aneurysm and, rarely, in long-standing cases, pulmonary artery dissection.

Multiple pulmonary metastases can be a cause of rapid-onset pulmonary hypertension.

Related topic: portopulmonary hypertension


I27.0 – Primary pulmonary hypertension

70995007 – Pulmonary hypertension

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Interstitial lung disease
  • Obstructive sleep apnea
  • Chronic obstructive pulmonary disease
  • Congestive heart failure
  • Anemia (see Iron deficiency anemia)
  • Liver cirrhosis
  • Drug toxicity (chemotherapeutics such as cyclophosphamide and bleomycin; amiodarone, fenfluramine / phentermine, aminorex, toxic rapeseed oil, Cocaine intoxication and use disorder, Methamphetamine use disorder)
  • Radiation pneumonitis
  • Mitral valve stenosis
  • Scleroderma (see also CREST syndrome)
  • Asthma

Best Tests

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Management Pearls

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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Last Reviewed:05/31/2021
Last Updated:08/29/2023
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Pulmonary hypertension
A medical illustration showing key findings of Pulmonary hypertension : Peripheral leg edema, Dyspnea, Lethargy, Presyncope, Exertional angina, Wheezing
Copyright © 2024 VisualDx®. All rights reserved.