Clinical Classification of Pulmonary Hypertension 1. Pulmonary arterial hypertension from pulmonary vasculopathy Idiopathic pulmonary arterial hypertension Heritable gene mutations - BMPR2 (bone morphogenic protein receptor type 2) - ALKI (activin A receptor type II-like kinase-I), endoglin (with or without hereditary hemorrhagic telangiectasia) Unknown Drug and toxin-induced Associated with - Connective tissue diseases - HIV infection - Portal hypertension - Congenital heart disease - Schistosomiasis - Chronic hemolytic anemia - Persistent pulmonary hypertension of the newborn Pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis 2. Pulmonary hypertension due to left heart disease Systolic dysfunction Diastolic dysfunction 3. Pulmonary hypertension due to lung disease and/or hypoxia Chronic obstructive pulmonary disease Interstitial lung disease Other pulmonary disease with mixed restrictive and obstructive pattern IV. Chronic thromboembolic pulmonary hypertension V. Pulmonary hypertension with unclear multifactorial mechanisms Hematologic disorders: myeloproliferative disorders, lenectomy Systemic disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis: lymphangioleimyomatosis, neurofibromatosis, vasculitis Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure #Pulmonary #Hypertension #PHTN #Classification #Diagnosis #Differential #Causes
