TABLE 1

Updated clinical classification of pulmonary hypertension (PH)

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; PVOD: pulmonary veno-occlusive disease; PCH: pulmonary capillary haemangiomatosis; LVEF: left ventricular ejection fraction. Reproduced from [8] with permission from the publisher.