A comparison of the predictive accuracy of three screening models for pulmonary arterial hypertension in systemic sclerosis

Yanjie Hao, Vivek Thakkar, Wendy Stevens, Kathleen Morrisroe, David Prior, Candice Rabusa, Peter Youssef, Eli Gabbay, Janet Roddy, Jennifer Walker, Jane Zochling, Joanne Sahhar, Peter Nash, Susan Lester, Maureen Rischmueller, Susanna M Proudman, Mandana Nikpour

IntroductionThere is evidence that early screening for pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) improves outcomes. We compared the predictive accuracy of two recently published screening algorithms (DETECT 2013 and Australian Scleroderma Interest Group (ASIG) 2012) for SSc-associated PAH (SSc-PAH) with the commonly used European Society of Cardiology/Respiratory Society (ESC/ERS 2009) guidelines.MethodsWe included 73 consecutive SSc patients with suspected PAH undergoing right heart catheterization (RHC). The three screening models were applied to each patient. For each model, contingency table analysis was used to determine sensitivity, specificity, positive (PPV) and negative predictive values (NPV) for PAH. These properties were also evaluated in an `alternate scenario analysis¿ where the prevalence of PAH was set at 10%.ResultsRHC revealed PAH in 27 (36.9%) patients. Both DETECT and ASIG algorithms performed equally in predicting PAH with sensitivity and NPV of 100%. The ESC/ERS guidelines had sensitivity of 96.3% and NPV of only 91%, missing one case of PAH; these guidelines could not be applied to three patients who had absent tricuspid regurgitant (TR) jet. The ASIG algorithm had the highest specificity of 54.5%. With PAH prevalence set at 10%, the NPV of the models was unchanged, but the PPV dropped to less than 20%.ConclusionsIn this cohort, the DETECT and ASIG algorithms out-perform the ESC/ERS guidelines, detecting all patients with PAH. The ESC/ERS guidelines have limitations in the absence of a TR jet. Ultimately, the choice of SSc-PAH screening algorithm will also depend on cost and ease of application.