The Evolving Management and Treatment Options for Patients with Pulmonary Hypertension: Current Evidence and Challenges

Figures & data

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.

Figure 2 Algorithm for the diagnosis of pulmonary arterial hypertension.

Notes: Swisher JW andKailash S. Advances in management of pulmonary hypertension associated with systemic sclerosis. In: New Insights Into Systemic Sclerosis. (Michal Tomcik, ed.) InTech Open, London, UK.© 2019 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution 3.0 License, which permits unrestricted use, distribution, and reproduction in any medium.¹⁷

Abbreviations: ECG, electrocardiogram; CXR, chest x-ray; VTR, velocity tricuspid regurgitation; LV, left ventricle; PH, pulmonary hypertension; PAH, pulmonary arterial hypertension; VQ, ventilation perfusion; CT, CT scan; RHC, right heart catheterization; mPAP, mean pulmonary artery pressure; CWP, capillary wedge pressure; PVR, pulmonary vascular resistance; WU, Wood Units; HIV, human immunodeficiency virus.

Figure 3 Hemodynamic definitions of pulmonary hypertension.

Notes: 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: mPAP, mean pulmonary arterial pressure; PAWP, pulmonary arterial wedge pressure; PVR, pulmonary vascular resistance; WU, Wood Units.

Swisher JW and Kailash S Advances in management of pulmonary hypertension associated with systemic sclerosis. In: New Insights Into Systemic Sclerosis. (Tomcik M, editor)

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