Figure 1.

Classification of pulmonary hypertensive diseases.

Notes: ^aPatients with heritable PAH or PAH associated with drugs and toxins might be acute responders. ^bLeft ventricular ejection fraction for HF with reduced ejection fraction: <40%; for HF with mildly reduced ejection fraction: 41–49%. ^cOther causes of pulmonary artery obstructions might include: sarcoma (high or intermediate grade or angiosarcoma), other malignant tumors (eg, renal carcinoma, uterine carcinoma, germ-cell tumors of the testes), non-malignant tumors (eg, uterine leiomyoma), arteritis without connective tissue disease, congenital pulmonary arterial stenoses, and hydatidosis. ^dIncluding inherited and acquired chronic hemolytic anemia and chronic myeloproliferative disorders. ^eIncluding sarcoidosis, pulmonary Langerhan’s cell histiocytosis, and neurofibromatosis type 1. ^fIncluding glycogen storage diseases and Gaucher disease. Reproduced with permission from Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2023 Jan 6;61(1):2200879. Copyright 2023 European Society of Cardiology & European Respiratory Society.³

Abbreviations: HF, heart failure; PAH, pulmonary arterial hypertension; PCH, pulmonary capillary hemangiomatosis; PH, pulmonary hypertension; PVOD, pulmonary veno-occlusive disease.