1.Pulmonary arterial hypertension (PAH) | ||
1.1 Idiopathic (IPAH) | ||
1.2 Heritable | ||
1.3 Drug- or toxin-induced | ||
1.4 Associated PAH (APAH) associated with: | ||
1.4.1 Connective tissue disease | ||
1.4.2 HIV | ||
1.4.3 Portal hypertension | ||
1.4.4 Congenital heart defect | ||
1.4.5 Schistosomiasis | ||
1.5 Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis | ||
1.6 Persistent PH of the newborn | ||
2. Pulmonary hypertension (PH) due to left heart disease | ||
2.1 Left-sided heart failure | ||
2.1.1 Heart failure with preserved left ventricular systolic function (HFpEF, heart failure with preserved ejection fraction) | ||
2.1.2 Heart failure with impaired left ventricular systolic function (heart failure with mid-range ejection fraction, i.e. HFmrEF and heart failure with reduced ejection fraction, i.e. HFrEF) | ||
2.2 Valvular disease | ||
2.3 Congenital or acquired heart disease leading to increased pulmonary venous pressure | ||
3. PH due to lung disease or hypoxaemia | ||
3.1 Chronic obstructive pulmonary disease/emphysema | ||
3.2 Interstitial lung disease | ||
3.3 Other lung disease associated with both restrictive and obstructive hypoventilation | ||
3.4 Hypoventilation | ||
3.5 Hypoxia in the absence of lung disease (such as extended stay in high altitudes) | ||
3.6 Developmental disorder | ||
4. PH associated with pulmonary arterial stenosis | ||
4.1 Chronic thromboembolic PH (CTEPH) | ||
4.2 Other obstruction of pulmonary arteries (e.g. tumours, arteritis, congenital stenosis, hydatidosis) | ||
5. Increased pulmonary blood pressure developing by undefined or multiple mechanisms | ||
5.1 Haematological disease: chronic haemolytic anaemia, myeloproliferative disorders | ||
5.2 Systemic disease: sarcoidosis, pulmonary pulmonary histiocytosis, neurofibromatosis | ||
5.3 Metabolic disorder, such as glycogen storage disease, Gaucher's disease | ||
5.4 Chronic renal failure | ||
5.5 Thrombotic microangiopathy | ||
5.6 Fibrosing mediastinitis |
Primary/Secondary Keywords