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.
- 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.
Multiple pulmonary metastases can be a cause of rapid-onset pulmonary hypertension.
Related topic: portopulmonary hypertension
I27.0 – Primary pulmonary hypertension
70995007 – Pulmonary hypertension
- 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)