|
| (1) Pulmonary arterial hypertension (PAH) | |
| (1.1) Idiopathic (IPAH) | |
| (1.2) Familial (FPAH) | |
| (1.3) Associated with (APAH) | |
| (1.3.1) Collagen vascular disease | |
| (1.3.2) Congenital systemic-to-pulmonary shunts | |
| (1.3.3) Portal hypertension | |
| (1.3.4) HIV infection | |
| (1.3.5) Drugs and toxins | |
| (1.3.6) Other (thyroid disorders, glycogen storage disease, | |
| Gaucher disease, hereditary hemorrhagic | |
| telangiectasia, hemoglobinopathies, | |
| myeloproliferative disorders, splenectomy) | |
| (1.4) Associated with venous or capillary involvement | |
| (1.4.1) Pulmonary venoocclusive disease (PVOD) | |
| (1.4.2) Pulmonary capillary hemangiomatosis (PCH) | |
| (1.5) Persistent pulmonary hypertension of the newborn | |
| (2) Pulmonary hypertension with left-heart disease | |
| (2.1) Left-sided atrial or ventricular heart disease | |
| (2.2) Left-sided valvular heart disease | |
| (3) Pulmonary hypertension associated with lung diseases and/or | |
| hypoxemia | |
| (3.1) Chronic obstructive pulmonary disease | |
| (3.2) Interstitial lung disease | |
| (3.3) Sleep-disordered breathing | |
| (3.4) Alveolar hypoventilation disorders | |
| (3.5) Chronic exposure to high altitude | |
| (3.6) Developmental abnormalities | |
| (4) Pulmonary hypertension owing to chronic thrombotic and/or | |
| embolic disease | |
| (4.1) Thromboembolic obstruction of proximal pulmonary | |
| arteries | |
| (4.2) Thromboembolic obstruction of distal pulmonary | |
| arteries | |
| (4.3) Nonthrombotic pulmonary embolism (tumor, parasites, | |
| foreign material) | |
| (5) Miscellaneous | |
| Sarcoidosis, histiocytosis X, lymphangiomatosis, compression | |
| of pulmonary vessels (adenopathy, tumor, fibrosing | |
| mediastinitis) | |
|
