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6 Pulmonary arterial hypertension (group 1) 6.1 Clinical characteristics The symptoms of PAH are non-specific and mainly related to progressive RV dysfunction (see Section 5.1.1) as a consequence of progressive pulmonary vasculopathy (Figure 7). The presentation of PAH may be modified by diseases that are associated with PAH, as well as comorbidities. More detailed descriptions of the individual PAH subsets are reported in Section 7. 6.2 Severity and risk assessment 6.2.1. Clinical parameters Clinical assessment is a key part of evaluating patients with PAH, as it provides valuable information for determining disease severity, improvement, deterioration, or stability. At follow-up, changes in WHO-FC (Table 15), episodes of chest pain, arrhythmias, haemoptysis, syncope, and signs of right HF provide important information. Physical examination should assess heart rate, rhythm, blood pressure, cyanosis, enlarged jugular veins, oedema, ascites, and pleural effusions. The WHO-FC is one of the strongest predictors of survival, Intimal proliferation, fibrosis Pulmonary vasculopathy Right heart failure Medial hypertrophy Vascular remodelling Plexiform lesion Thrombosis Vasoconstriction Pulmonary artery Vascular obstruction Right ventricular remodelling / dysfunction Current therapeutic targets Medial hyperplasia Intimal proliferation Plexiform lesions NO-sGC-cGMP pathway Endothelin pathway Prostacyclin pathway L-arginine Nitric oxide (Vasodilatation and antiproliferation) sGC PDE5 GTP cGMP GMP Arachidonic acid Prostacyclin (Vasodilatation and antiproliferation) cAMP IP receptor Pro-endothelin-1 Endothelin-1 (Vasoconstriction and proliferation) Endothelin receptor A Endothelin receptor B P P M P G MP Figure 7 Pathophysiology and current therapeutic targets of pulmonary arterial hypertension (group 1). cAMP, cyclic adenosine monophosphate; (c) GMP, (cyclic) guanosine monophosphate; GTP, guanosine-5′-triphosphate; IP receptor, prostacyclin I2 receptor; NO, nitric oxide; PDE5, phosphodiesterase 5; sGC, soluble guanylate cyclase. Downloaded from academic.oup.com/eurheartj/article/43/38/3618/6673929 by guest on 07 October 2026