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



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