Classification of Pulmonary Hypertension
- Pulmonary Arterial Hypertension
- Idiopathic
- Heritable (mutations/familial cases)
- Collagen vascular diseases (e.g., scleroderma, SLE, RA)
- Congenital systemic to pulmonary shunts (e.g., ventricular septal defect, patent ductus arteriosus, atrial septal defect)
- Portal hypertension
- HIV infection
- Drugs or toxins (e.g., fenfluramine)
- Pulmonary Hypertension Due to Left Heart Disease
- LV systolic or diastolic dysfunction
- Left-sided valvular disease
- Pulmonary Hypertension Due to Lung Disease or Hypoxia
- Chronic obstructive lung disease
- Interstitial lung disease
- Sleep-disordered breathing
- Chronic hypoventilation
- Chronic Pulmonary Thromboembolic Disease
- Chronic pulmonary embolism
- Miscellaneous
- Systemic conditions (e.g., sarcoidosis, pulmonary histiocytosis)
- Hematologic conditions (e.g., myeloproliferative diseases)
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Abbreviations: RA, rheumatoid arthritis.