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Right ventricular (RV) diastolic stiffness is increased in pulmonary arterial hypertension (PAH) patients. We investigated whether RV diastolic stiffness is associated with clinical progression and assessed the contribution of RV wall thickness to RV systolic and diastolic stiffness. Using single-beat pressure-volume analyses, we determined RV end-systolic elastance (Ees), arterial elastance (Ea), RV--arterial coupling (Ees/Ea), and RV end-diastolic elastance (stiffness, Eed) in controls (n=15), baseline PAH patients (n=63) and treated PAH patients (survival &gt;5 years n=22 and survival &lt;5 years n=23). We observed an association between Eed and clinical progression, with baseline Eed &gt;0.53 mmHg·mL(-1) associated with worse prognosis (age-corrected hazard ratio 0.27, p=0.02). In treated patients, Eed was higher in patients with survival &lt;5 years than in patients with survival &gt;5 years (0.91±0.50 versus 0.53±0.33 mmHg·mL(-1), p&lt;0.01). Wall-thickness-corrected Eed values in PAH patients with survival &gt;5 years were not different from control values (0.76±0.47 versus 0.60±0.41 mmHg·mL(-1), respectively, not significant), whereas in patients with survival &lt;5 years, values were significantly higher (1.52±0.91 mmHg·mL(-1), p&lt;0.05 versus controls). RV diastolic stiffness is related to clinical progression in both baseline and treated PAH patients. RV diastolic stiffness is explained by the increased wall thickness in patients with &gt;5 years survival, but not in those surviving &lt;5 years. This suggests that intrinsic myocardial changes play a distinctive role in explaining RV diastolic stiffness at different stages of PAH.

Clinical relevance of right ventricular diastolic stiffness in pulmonary hypertension