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. 2020 Apr 30;2020(1):e202014. doi: 10.21542/gcsp.2020.14

Table 1. Updated clinical classification of pulmonary hypertension (PH).

1 Pulmonary arterial hypertension (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 Hematological disorders
5.2 Systemic and metabolic disorders
5.3 Others
5.4 Complex congenital heart disease