Updated World Health Organization classification of pulmonary hypertension
Group 1: Pulmonary arterial hypertension Idiopathic Heritable BMPR2 mutation Other mutations Drug- and toxin-induced Associated with: Connective tissue disease Human immunodeficiency virus (HIV) infection Portal hypertension Congenital heart disease Schistosomiasis Pulmonary veno-occlusive disease or pulmonary capillary hemangiomatosis Idiopathic Heritable EIF2AK4 mutation Other mutations Drug-, toxin-, and radiation-induced Associated with: Connective tissue disease HIV infection Persistent pulmonary hypertension of the newborn Group 2: Pulmonary hypertension due to left heart disease Left ventricular systolic dysfunction Left ventricular diastolic dysfunction Valvular disease Congenital or acquired left heart inflow or outflow tract obstruction and congenital cardiomyopathies Congenital or acquired pulmonary vein stenosis | Group 3: Pulmonary hypertension due to lung diseases, hypoxia, or both Chronic obstructive pulmonary disease Interstitial lung disease Other pulmonary diseases with mixed restrictive and obstructive pattern Sleep-disordered breathing Alveolar hypoventilation disorders Chronic exposure to high altitude Developmental lung diseases Group 4: Chronic thromboembolic pulmonary hypertension and other pulmonary artery obstructions Chronic thromboembolic pulmonary hypertension Other pulmonary artery obstructions Angiosarcoma Other intravascular tumors Arteritis Congenital pulmonary artery stenosis Parasites (hydatidosis) Group 5: Pulmonary hypertension with unclear or multifactorial mechanisms Hematologic disorders: chronic hemolytic anemia, myeloproliferative disorders, splenectomy Systemic disorders, sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders Others: pulmonary tumoral thrombotic microangiopathy, fibrosing mediastinitis, chronic renal failure (with or without dialysis), segmental pulmonary hypertension |
Reproduced with permission of the European Society of Cardiology and the European Respiratory Society. European Respiratory Journal Oct J 2015; 46(4):903–975. doi:10.1183/13993003.01032-2015