Abstract

Abstract Background Pulmonary arterial hypertension (PAH) is a severe disease that progressively leads to right ventricular (RV) failure and cardiovascular death. Evaluation of right heart mechanics by means of 2-dimensional speckle tracking echocardiography (2D-STE) has displayed to be a promising tool to estimate prognosis in PAH patients. Purpose To evaluate the association between right ventricular free wall longitudinal strain (RVFWLS) and right peak atrial longitudinal strain (RPALS) at follow-up after initiation of specific vasodilator therapy in PAH patients, with outcomes. Methods 83 subjects diagnosed with PAH Group 1 at three University Hospitals (Hammersmith Hospital, London, United Kingdom; Trieste University Hospital, Trieste, Italy; FTGM, Pisa, Italy), who were naive from specific treatment for PAH at the time of diagnosis, were retrospectively enrolled in this study. Standard echocardiographic parameters were collected. Outcomes were defined as the combination of all-cause mortality, hospitalization for PAH and first prostanoid administration. We investigated the correlation between RVFWLS and RPALS with outcomes adjusting for validated echocardiographic parameters strongly associated with prognosis in PAH (right atrial area – RAA and pericardial effusion – PE) and patients’ haemodynamics. Results 30 patients experienced outcomes during a median follow-up time of 33 months. Median RVFWLS at follow-up was -15.8% (IQR: -12.1%/- 21.1%). Median RPALS at follow-up was 25% (IQR: 17.9%/36.6%). In the multivariate analysis, RVFLWS at follow-up was independently associated with outcomes (95% confidence interval [CI]: 1.01 – 1.24, p = 0.04), irrespectively from RAA (95% CI: 0.98 – 1.20, p = 0.12) and pulmonary arterial systolic pressure (PASP, 95% CI: 0.99 – 1.06, p = 0.12). We also observed a trend towards superiority of RPALS at follow-up (95% CI 0.90 – 1.00, p = 0.07) over RAA (95% CI: 0.98 – 1.20, p = 0.14) and PASP (95% CI: 0.99 - 1.05, p= 0.31). Finally, RPALS (95% CI: 0.88 – 1.00, p = 0.05) was independently associated with outcomes over PE (95% CI: 0.14 – 1.89, p = 0.32) and RVFWLS (95% CI: 0.97 – 1.20, p = 0.16). Conclusions In PAH group 1, assessment of both RVFWLS and RPALS at follow-up is associated with outcomes, independently from standard echocardiographic parameters.

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