Classification of pulmonary hypertension according to European Society of Cardiology/European Respiratory Society Guidelines

1 Pulmonary arterial hypertension
 1.1 Idiopathic
 1.2 Heritable
  1.2.1 BMPR2 mutation
  1.2.2 Other mutations
 1.3 Drugs and toxins 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 disease
  1.4.5 Schistosomiasis
1′ Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis
 1′.1 Idiopathic
 1′.2 Heritable
  1′.2.1 EIF2AK4 mutation
  1′.2.2 Other mutations
 1′.3 Drugs, toxins and radiation induced
 1′.4 Associated with:
  1′.4.1 Connective tissue disease
  1′.4.2 HIV infection
1′′ Persistent pulmonary hypertension of the newborn
2 Pulmonary hypertension due to left heart disease
 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
 2.5 Congenital/acquired pulmonary vein stenosis
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 lung diseases
4 Chronic thromboembolic pulmonary hypertension and other pulmonary artery obstructions
 4.1 Chronic thromboembolic pulmonary hypertension
 4.2 Other pulmonary artery obstructions
  4.2.1 Angiosarcoma
  4.2.2 Other intravascular tumours
  4.2.3 Arteritis
  4.2.4 Congenital pulmonary arteries stenoses
  4.2.5 Parasites (hydatidosis)
5 Pulmonary hypertension with unclear and/or multifactorial mechanisms
 5.1 Haematological disorders: chronic haemolytic anaemia, myeloproliferative disorders, splenectomy
 5.2 Systemic disorders, sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis,   neurofibromatosis
 5.3 Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders
 5.4 Others: pulmonary tumoral thrombotic microangiopathy, fibrosing mediastinitis, chronic renal failure (with/without dialysis), segmental pulmonary hypertension
  • BMPR2: bone morphogenetic protein receptor type 2; EIF2AK4: eukaryotic translation initiation factor 2 alpha kinase 4. Reproduced from [1] with permission from the publisher.