Table 1.
Executive summary of the World Symposium on Primary Pulmonary Hypertension
| 1 | Clinical classification of pulmonary arterial hypertension | |
| 1.1 | Idiopatic pulmonary arterial hypertension | |
| Sporadic | ||
| Familiar | ||
| 1.2 | Pulmonary arterial hypertension associated with: | |
| Connective tissue disease | ||
| Congenital heart disease | ||
| Portal hypertension | ||
| HIV infection | ||
| Anorexigens and other toxic drugs | ||
| Persistent pulmonary hypertension of the newborn | ||
| 2 | Pulmonary venous hypertension | |
| 2.1 | Left-sided atrial or ventricular heart disease | |
| 2.2 | Extrinsic compression of central pulmonary veins | |
| 2.3 | Pulmonary veno-occlusion disease | |
| 3 | Pulmonary hypertension associated with disorders of the respiratory system and/or hypoxemia | |
| 3.1 | Chronic obstructive pulmonary disease | |
| 3.2 | Interstitial lung disease | |
| 3.3 | Sleep-disordered breathing | |
| 3.4 | Chronic exposure to high altitude | |
| 3.5 | Cystic fibrosis | |
| 4 | Pulmonary hypertension due to chronic thrombotic and/or embolic disease | |
| 4.1 | Thromboembolic obstruction of proximal pulmonary arteries | |
| 4.2 | Thromboembolic obstruction of distal pulmonary arteries | |
| Pulmonary embolism | ||
| ‘In situ’ thrombosis | ||
| Thalassemia | ||
| 5 | Pulmonary hypertension due to disorders directly affecting pulmonary vasculature | |
| 5.1 | Inflammatory disease | |
| Schistosomiasis/sarcoidosis | ||
| 5.2 | Pulmonary capillary hemangiomatosis | |
| [37]. | ||