Pulmonary Vascular Dysfunction in Systemic Sclerosis

Purpose The aim was to use the DETECT algorithm to detect pulmonary arterial hypertension (PAH) in a single-centre Danish cohort of patients with systemic sclerosis (SSc) without known PAH. To assess for pulmonary vascular dysfunction patients referred for right heart catheterization were assessed with invasive hemodynamic exercise testing. Methods 73 patients (aged 60 ± 10 years; 56 (77%) females) diagnosed with SSc for 13.5 (IQR 2.7 - 33.5) years underwent echocardiography, pulmonary funktion test, NT-proBNP and ECG. 19/73 (aged 62 ± 10 years, 12 (63%) females) patients were found to have an elevated STEP 2 score and underwent invasive hemodynamic assessment at rest and during exercise. Results At rest, 3/19 patients fulfilled the 2022 ESC guidelines PAH criteria (mean pulmonary arterial pressure (mPAP) > 20 mmHg, pulmonary arterial wedge pressure ≤ 15 mmHg and pulmonary vascular resistance > 2 wood units). Using the 2015 ESC guidelines only 1/19 patient had PAH (mPAP>25 mmHg and PAWP < 15 mmHg). With exercise 18/19 experienced mPAP > 30 mmHg and the majority (13/19) had an abnormal pulmonary pressure-flow relationship > 3 mmHg/ml/min (figure 1). Many patients (7/19) had elevated PAWP > 25 mmHg with exercise. Conclusion In a Danish cohort of 73 prevalent scleroderma patients, the application of the DETECT algorithm identified only a few patients that fulfilled the guideline criteria for PAH. However, a large proportion of patients had an abnormal pulmonary vascular response to exercise and elevated PAWP with exercise.