Alerts and Notices
SynopsisPulmonary 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, schistosomiasis, or drug use. Several genetic mutations may be implicated in the pathogenesis of PAH.
- 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.
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
Differential Diagnosis & Pitfalls
- Interstitial lung disease
- Obstructive sleep apnea
- Chronic obstructive pulmonary disease
- Congestive heart failure
- Anemia (see iron deficiency anemia)
- Drug toxicity (chemotherapeutics such as cyclophosphamide and bleomycin; amiodarone, fenfluramine / phentermine, aminorex, toxic rapeseed oil, cocaine, amphetamines)
- Radiation-induced lung disease
- Mitral stenosis
- Scleroderma (see also CREST syndrome)
Drug Reaction DataBelow 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.