Screening for pulmonary arterial hypertension in an unselected prospective systemic sclerosis cohort

Abstract

Screening for pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) improves outcomes. The DETECT screening algorithm is recommended in a high-risk SSc subgroup. This study aims to compare prospectively the positive predictive value of screening using the DETECT algorithm and the 2009 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines, and to compare their cost-effectiveness in an unselected, day-to-day SSc population. Post hoc, screening according to the 2015 ESC/ERS guidelines using echocardiographic parameters alone ("2015 echo screening") or combined with the DETECT algorithm ("2015 combined screening") in high-risk subjects was analysed. 195 consecutive SSc patients included in the Ghent University Hospital SSc cohort were screened using different algorithms. The referral rate for right heart catheterisation was 32% (63 out of 195 patients) (46/4/13/34/40 patients using the DETECT algorithm/2009 guidelines/both/2015 echo screening/2015 combined screening). Right heart catheterisation was performed in 53 patients (84%) (36 (78%)/four (100%)/13 (100%)/28 (82%)/32 (80%) patients recommended by the DETECT algorithm/2009 guidelines/both/2015 echo screening/2015 combined screening). PAH was diagnosed in three patients (incidence 1.5%·year-1, 95% CI 0.5 - 4.4), in whom all algorithms recommended a right heart catheterisation. The positive predictive value was 6% (95% CI 2 - 17%; three out of 49 patients) for the DETECT algorithm, 18% (95% CI 6 - 41%; three out of 17 patients) for the 2009 guidelines, 23% (95% CI 8 - 50%; three out of 13 patients) for both, 11% (95% CI 4 - 27%; three out of 28 patients) for the 2015 echo screening and 9% (95% CI 3 - 24%; three out of 32 patients) for the 2015 combined screening. The cost was EUR224/80/90/112 per patient using the DETECT algorithm/2009 guidelines/2015 echo screening/2015 combined screening. Echocardiography may remain a candidate first step for PAH screening in SSc.