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1% 1% Global population Rare Pulmonary arterial hypertension (PAH) PH associated with left heart disease PH associated with lung disease PH associated with pulmonary artery obstructions PH with unclear and/or multifactorial mechanisms Very common Common Rare Rare Medical therapy Idiopathic/heritable Associated conditions CpcPH IpcPH Non-severe PH Severe PH CTEPH Other pulmonary obstructions Haematological disorders Systemic disorders PAH drugs CCB in responders Potentially: PAH drugs (trials) Lung transplantation IpcPH: Treatment of LHDa CpcPH: Treatment of LHDa PH-lung disease: Optimized care of underlying lung disease Severe PH: Potentially: PAH drugs (trials) Surgical therapy: PEA Interventional: BPA Medical therapy: PH drugs Optimized treatment of underlying disease Pulmonary congestion in post-capillary PH Pulmonary vascular disease / obstruction in pre-capillary PH Mortality Hazard Ratio mPAP (mmHg) Mortality Hazard Ratio PVR (Wood units) Right heart failure Prevalence Potentially: PAH drugs (trials) 6 5 4 3 1 2 10 20 30 40 50 60 5 4 3 1 2 0 2 3 4 5 6 0 1 CLINICAL CLASSIFICATION PULMONARY HYPERTENSION PREVALENCE THERAPEUTIC STRATEGIES Figure 1 Central illustration. BPA, balloon pulmonary angioplasty; CCB, calcium channel blocker; CTEPH, chronic thrombo-embolic pulmonary hypertension; CpCPH, combined post- and pre-capillary pulmonary hypertension; IpcPH, isolated post-capillary pulmonary hypertension; LHD, left heart disease; PAH, pulmonary arterial hypertension; PEA, pulmonary endarterectomy; PH, pulmonary hypertension. aTreatment of heart failure according to the ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure.27 Treatment of left-sided valvular heart disease according to the 2021 ESC/EACTS Guidelines for the management of valvular heart disease.28 Downloaded from academic.oup.com/eurheartj/article/43/38/3618/6673929 by guest on 07 October 2026