Current clinical classification of pulmonary hypertension from the 4th World Symposium on Pulmonary Hypertension (Dana Point, CA, 2008)
[Reprinted from Simonneau G, Robbins IM, Beghetti M, Channick RN, Delcroix M, Denton CP, Elliott CG, Gaine SP, Gladwin MT, Jing ZC, Krowka MJ, Langleben D, Nakanishi N, and Souza R (2009) Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 54:S43–S54. Copyright © 2009 Elsevier. Used with permission.]
| 1. Group 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. Group 2 pulmonary hypertension owing to left heart disease |
| 2.1. Systolic dysfunction |
| 2.2. Diastolic dysfunction |
| 2.3. Valvular disease |
| 3. Group 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. Group 4 chronic thromboembolic pulmonary hypertension (CTEPH) |
| 5. Group 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 |
ALK1, activin receptor-like kinase type 1; BMPR2, bone morphogenetic protein receptor type 2.