Clinical classification of pulmonary hypertension4
| Group 1: PAH | Idiopathic PAH Heritable PAH Drug and toxin-induced PAH PAH associated with:
PAH long-term responders to calcium channel blockers PAH with over features of venous/capillary (PVOD/PCH) involvement Persistent PH of the newborn syndrome |
| Group 2: PH due to left heart disease | PH due to heart failure with preserved left ventricular ejection fraction PH due to heart failure with reduced left ventricular ejection fraction Valvular heart disease Congenital/acquired cardiovascular conditions leading to postcapillary PH |
| Group 3: PH due to lung diseases and/or hypoxia | Obstructive lung disease Restrictive lung disease Other lung disease with mixed restrictive/obstructive pattern Hypoxia without lung disease Developmental lung disorders |
| Group 4: PH due to pulmonary artery obstruction | Chronic thromboembolic PH Other pulmonary artery obstructions: sarcoma or angiosarcoma, other malignant tumors (renal carcinoma, uterine carcinoma, germ cell tumors), non-malignant tumors (uterine leiomyoma), arteritis without connective tissue disease, congenital pulmonary artery stenosis, parasites (hydatidosis) |
| Group 5: PH with unclear and/or multifactorial mechanisms | Hematologic disorders: chronic hemolytic anemia, myeloproliferative disorders Systemic and metabolic disorders: pulmonary Langerhans cell histiocytosis, Gaucher disease, glycogen storage disease, neurofibromatosis, sarcoidosis Others: chronic renal failure with or without hemodialysis, fibrosing mediastinitis Complex congenital heart disease |
PAH, pulmonary arterial hypertension; PCH, pulmonary capillary hemangiomatosis; PH, pulmonary hypertension; PVOD, pulmonary veno-occlusive disease.