Version May 2024
| Group 1 - Pulmonary arterial hypertension (PAH) |
| 1.1 Idiopathic: |
| 1.1.1 Non-responders at vasoreactivity testing |
| 1.1.2 Acute responders at vasoreactivity testing |
| 1.2 Heritable* |
| 1.3 Associated with drugs and toxins* |
| 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 |
| 1.5 PAH with features of venous/capillary (PVOD/PCH) involvement |
| 1.6 Persistent PH of the newborn |
| Group 2 - PH associated with left heart disease |
| 2.1 Heart failure: |
| 2.1.1 with preserved ejection fraction |
| 2.1.2 with reduced or mildly reduced ejection fraction¶ |
| 2.2 Valvular heart disease |
| 2.3 Congenital/acquired cardiovascular conditions leading to post-capillary PH |
| Group 3 - PH associated with lung diseases and/or hypoxia |
| 3.1 Obstructive lung disease or emphysema |
| 3.2 Restrictive lung disease |
| 3.3 Lung disease with mixed restrictive/obstructive pattern |
| 3.4 Hypoventilation syndromes |
| 3.5 Hypoxia without lung disease (eg, high altitude) |
| 3.6 Developmental lung disorders |
| Group 4 - PH associated with pulmonary artery obstructions |
| 4.1 Chronic thrombo-embolic PH |
| 4.2 Other pulmonary artery obstructionsΔ |
| Group 5 - PH with unclear and/or multifactorial mechanisms |
| 5.1 Haematological disorders◊ |
| 5.2 Systemic disorders§ |
| 5.3 Metabolic disorders¥ |
| 5.4 Chronic renal failure with or without haemodialysis |
| 5.5 Pulmonary tumour thrombotic microangiopathy |
| 5.6 Fibrosing mediastinitis |
PAH: pulmonary arterial hypertension; HIV: human immunodeficiency virus; PVOD: pulmonary veno-occlusive disease; PCH: pulmonary capillary haemangiomatosis; PH: pulmonary hypertension.
* Patients with heritable PAH or PAH associated with drugs and toxins might be acute responders.
¶ Left ventricular ejection fraction for heart failure with reduced ejection fraction: ≤40%; for heart failure with mildly reduced ejection fraction: 41 to 49%.
Δ Other causes of pulmonary artery obstructions include: sarcomas (high or intermediate grade or angiosarcoma), other malignant tumours (eg, renal carcinoma, uterine carcinoma, germ-cell tumours of the testis), non-malignant tumours (eg, uterine leiomyoma), arteritis without connective tissue disease, congenital pulmonary arterial stenoses, and hydatidosis.
◊ Including inherited and acquired chronic haemolytic anaemia and chronic myeloproliferative disorders.
§ Including sarcoidosis, pulmonary Langerhans's cell histiocytosis, and neurofibromatosis type 1.
¥ Including glycogen storage diseases and Gaucher disease.آیا می خواهید مدیلیب را به صفحه اصلی خود اضافه کنید؟
