| 1. |
Pulmonary Arterial Hypertension (PAH) |
| 1.1 |
Idiopathic PAH |
| 1.2. |
Heritable |
| 1.2.1. |
BMPR2 |
| 1.2.2. |
ALK1, endoglin (with or without hereditary hemorrhagic telangiectasia) |
| 1.2.3. |
Unknown |
| 1.3. |
Drug- and toxin-induced |
| 1.4. |
Associated with |
| 1.4.1. |
Connective tissue diseases |
| 1.4.2. |
HIV infection |
| 1.4.3. |
Portal hypertension |
| 1.4.4. |
Congenital heart diseases |
| 1.4.5. |
Schistosomiasis |
| 1.4.6. |
Chronic hemolytic anemia |
| 1.5. |
Persistent pulmonary hypertension of the newborn |
| 1´. |
Pulmonary Veno-occlusive Disease (PVOD) and/or Pulmonary Capillary Hemangiomatosis (PCH) |
| 2. |
Pulmonary hypertension owing to left heart disease |
| 2.1. |
Systolic dysfunction |
| 2.2. |
Diastolic dysfunction |
| 2.3. |
Valvular disease |
|
|
| 3. |
Pulmonary hypertension owing to lung diseases and/or hypoxia |
| 3.1. |
Chronic obstructive pulmonary disease |
| 3.2. |
Interstitial lung disease |
| 3.3. |
Other pulmonary diseases with mixed restrictive and obstructive pattern |
| 3.4. |
Sleep-disordered breathing |
| 3.5. |
Alveolar hypoventilation disorders |
| 3.6. |
Chronic exposure to high altitude |
| 3.7. |
Developmental abnormalities |
| 4. |
Chronic Thromboembolic Pulmonary Hypertension (CTEPH) |
| 5. |
Pulmonary hypertension with unclear multifactorial mechanisms |
| 5.1. |
Hematologic disorders: myeloproliferative disorders, splenectomy |
| 5.2. |
Systemic disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis: lymphangioleiomyomatosis, neurofibromatosis, vasculitis |
| 5.3. |
Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders |
| 5.4. |
Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure on dialysis |
