Table 1.
Revised Evian Nomenclature and Classification of Pulmonary Hypertension (Venice Symposium 2003)
| Pulmonary arterial hypertension | Idiopathic | |
| Familial | ||
| Secondary to | Collagen vascular disease | |
| Congenital systemic to pulmonary shunts | ||
| Portal hypertension | ||
| HIV infection | ||
| Drugs/toxins | ||
| Other | ||
| Pulmonary veno-occlusive disease | ||
| Pulmonary capillary haemangiomatosis | ||
| Pulmonary venous hypertension | Left-sided atrial or ventricular heart disease | |
| Left-sided valvular heart disease | ||
| PH associated with hypoxemia | COPD | |
| Interstitial lung disease | ||
| Sleep-disordered breathing | ||
| Alveolar hypoventilatory disorders | ||
| Chronic exposure to high altitude | ||
| PH due to chronic thrombotic and/or embolic disease | Thromboembolic obstruction of proximal pulmonary arteries | |
| Obstruction of distal pulmonary arteries | ||
| Pulmonary embolism | thrombus, tumor, foreign material | |
| Pulmonary capillary hemangiomatosis | ||
| Other | ||
| Miscellaneous | Extrinsic compression of central pulmonary veins | Fibrosing mediastinitis |
| Adenopathy/tumors | ||
| Sarcoidosis | ||
| Histiocytosis X | ||
| Lymphangiomatosis |