Table 1 Diagnostic classification of pulmonary hypertension

1. Pulmonary arterial hypertension (PAH)

1.1 Idiopathic

1.2 Heritable

1.3 Drugs and toxins induced

1.4 Associated with (APAH):

  1.4.1 Connective tissue disease

  1.4.2 Infection with human immunodeficiency virus

  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

2. Pulmonary hypertension with left heart disease

2.1 Systolic dysfunction

2.2 Diastolic dysfunction

2.3 Valvular disease

3. Pulmonary hypertension 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 pulmonary hypertension

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: tumoral obstruction, fibrosing mediastinitis, chronic renal failure on dialysis