Table 2.
1 PAH |
1.1 Idiopathic PAH |
1.2 Heritable PAH |
1.3 Drug- and toxin-induced PAH |
1.4 PAH associated with: |
1.4.1 Connective tissue disease |
1.4.2 HIV infection |
1.4.3 Portal hypertension |
1.4.4 Congenital heart disease |
1.4.5 Schistosomiasis |
1.5 PAH long-term responders to calcium channel blockers |
1.6 PAH with overt features of venous/capillaries (PVOD/PCH) involvement |
1.7 Persistent PH of the newborn syndrome |
2 PH due to left heart disease |
2.1 PH due to heart failure with preserved LVEF |
2.2 PH due to heart failure with reduced LVEF |
2.3 Valvular heart disease |
2.4 Congenital/acquired cardiovascular conditions leading to post-capillary PH |
3 PH due to lung diseases and/or hypoxia |
3.1 Obstructive lung disease |
3.2 Restrictive lung disease |
3.3 Other lung disease with mixed restrictive/obstructive pattern |
3.4 Hypoxia without lung disease |
3.5 Developmental lung disorders |
4 PH due to pulmonary artery obstructions |
4.1 Chronic thromboembolic PH |
4.2 Other pulmonary artery obstructions |
5 PH with unclear and/or multifactorial mechanisms |
5.1 Haematological disorders |
5.2 Systemic and metabolic disorders |
5.3 Others |
5.4 Complex congenital heart disease |
PAH: pulmonary arterial hypertension; PH: pulmonary hypertension; PVOD: pulmonary veno-occlusive disease; PCH: pulmonary capillary hemangiomatosis; LVEF: left ventricular ejection fraction.
Source: reproduced with permission from Simonneau et al.32