Table. 5—

ESC/ERS guidelines: updated clinical classification of pulmonary hypertension (PH)

    1.1 Idiopathic
    1.2 Heritable
        1.2.1 BMPR2
        1.2.2 ALK-1, endoglin (with or without hereditary haemorrhagic telangiectasia)
        1.2.3 Unknown
    1.3 Drugs and toxins induced
    1.4 Associated with (APAH)
        1.4.1 Connective tissue diseases
        1.4.2 HIV infection
        1.4.3 Portal hypertension
        1.4.4 Congenital heart disease
        1.4.5 Schistosomiasis
        1.4.6 Chronic haemolytic anaemia
    1.5 Persistent pulmonary hypertension of the newborn
1′ Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis
2 PH due to left heart disease
    2.1 Systolic dysfunction
    2.2 Diastolic dysfunction
    2.3 Valvular disease
3 PH due 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 Chronic thromboembolic PH
5 PH with unclear and/or multifactorial mechanisms
    5.1 Haematological 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: tumoural obstruction, fibrosing mediastinitis, chronic renal failure on dialysis
  • PAH: pulmonary arterial hypertension; BMPR2: bone morphogenetic protein receptor, type 2; ALK-1: activin receptor-like kinase 1; APAH: associated pulmonary arterial hypertension. Reproduced from 11 with permission from the publisher.