Abstract

BackgroundEarly pulmonary arterial hypertension (PAH) diagnosis and treatment are crucial to improve systemic sclerosis (SSc) patients’ outcomes. In PAH the progressive pulmonary vascular remodelling leads to an increasing load on the contracting RV and an altered right ventricular - pulmonary arterial (RV-PA) coupling. The RV-PA coupling describes the RV adaptation to its afterload [1]. Tricuspid annular plane systolic excursion/systolic pulmonary artery pressure (TAPSE/sPAP) ratio is the validated non-invasive estimation of RV-PA coupling [2]. In the new 2022 pulmonary hypertension (PH) guidelines, TAPSE/sPAP ratio has been included among the additional echocardiographic signs suggestive of PH and the echocardiographic parameters for 1-year mortality risk assessment [3]. However, to date, the role of TAPSE/sPAP ratio in SSc is underinvestigated [4-6].ObjectivesThe primary aim of the study was to assess the predictive role of TAPSE/sPAP ratio for PH diagnosis in the SSc European Scleroderma Trials and Research (EUSTAR) cohort. The secondary aim of the study was to evaluate the prognostic role of TAPSE/sPAP ratio in predicting mortality in the SSc EUSTAR cohort.MethodsEligible patients were systemic sclerosis (SSc) patients registered in the EUSTAR database with at least one visit recording TAPSE and sPAP data. Individual centres were required to provide TAPSE and sPAP data at 12 ± 3 months before right heart catheterization (RHC). Logistic regression analysis was applied to analyse the predictive ability of TAPSE/sPAP ratio for PH diagnosis. Cox regression analysis was performed to evaluate TAPSE/sPAP ratio as a predictive factor for all-cause mortality.Results2555 SSc patients met the inclusion criteria for this study with 355 SSc patients having available RHC data at baseline. PH was confirmed by RHC in 195 SSc patients (54.9%). TAPSE/sPAP ratio <0.55 mm/mmHg [OR 0.251 (95% CI 0.084-0.753), p<0.05] and FVC/DLCO [OR 2.568 (95% CI 1.227-5.375), p<0.05] were significantly associated with PH diagnosis. In logistic regression analysis with echocardiographic parameters at 12±3 months before RHC, TAPSE/sPAP ratio <0.55 mm/mmHg [OR 0.265 (95% CI 0.102-0.685), p<0.01] and FVC/DLCO [OR 2.529 (95% CI 1.358-4.711), p<0.01] were significantly associated with PH diagnosis. In multivariate Cox regression analysis, TAPSE/sPAP ratio ≤0.32 mm/mmHg [HR 0.310 (0.164–0.585), p<0.001] was the most significant predictive factor for all-cause mortality.ConclusionTAPSE/sPAP ratio <0.55 mm/mmHg is a predictive risk factor for PH. TAPSE/sPAP ratio ≤0.32 mm/mmHg is a predictive risk marker for all-cause mortality.

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