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. 2014 Nov;39(11):749-751, 755-758.

Table 1.

Clinical Classification of Pulmonary Hypertension*

1. Pulmonary arterial hypertension (PAH; WHO Group 1)

  • 1.1 Idiopathic PAH

  • 1.2 Heritable PAH
    • 1.2.1 BMPR2
    • 1.2.2 ALK-1, ENG, SMAD9, CAV1, KCNK3
    • 1.2.3 Unknown

  • 1.3 Drug- and toxin-induced

  • 1.4 Associated with:
    • 1.4.1 Connective tissue disease
    • 1.4.2 HIV infection
    • 1.4.3 Portal hypertension
    • 1.4.4 Congenital heart diseases
    • 1.4.5 Schistosomiasis

  • 1′ Pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis

  • 1″ Persistent pulmonary hypertension of the newborn

2. Pulmonary hypertension due to left heart disease (WHO Group 2)

  • 2.1 Left ventricular systolic dysfunction

  • 2.2 Left ventricular diastolic dysfunction

  • 2.3 Valvular disease

  • 2.4 Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies

3. Pulmonary hypertension due to lung diseases and/or hypoxia (WHO Group 3)

  • 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 lung diseases

4. Chronic thromboembolic pulmonary hypertension (WHO Group 4)
5. Pulmonary hypertension with unclear multifactorial mechanisms (WHO Group 5)

  • 5.1 Hematological disorders: chronic hemolytic anemia, myeloproliferative disorders, splenectomy

  • 5.2 Systemic disorders: sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis

  • 5.3 Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders

  • 5.4 Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental PH

ALK-1 = activin receptor-like kinase 1; BMPR2 = bone morphogenic protein receptor type II; CAV1 = caveolin-1; ENG = endoglin; HIV = human immunodeficiency virus; WHO = World Health Organization

*

Updated at the Fifth World Symposium on Pulmonary Hypertension in Nice, France, 2013. Adapted from Simonneau et al.2