Determinants of exercise-induced pulmonary arterial hypertension in systemic sclerosis

Background: Exercise-induced pulmonary arterial hypertension (EIPH) in systemic sclerosis (SSc) has already been observed but its determinants remain unclear. The aim of this study was to determine the incidence and the determinants of EIPH in SSc. Methods and results: We prospectively enrolled 63 patients with SSc (age 54 +/- 3 years, 76% female) followed in CHU Sart-Tilman in Liege. All patients underwent graded semi-supine exercise echocardiography. Systolic pulmonary arterial pressure (sPAP) was derived from the peak velocity of the tricuspid regurgitation jet and adding the estimation of right atrial pressure, both at rest and during exercise. Resting pulmonary arterial hypertension (PH) was defined as sPAP >35 mm Hg and EIPH as sPAP >50 mm Hg during exercise. The following formulas were used: mean PAP (mPAP) = 0.61 x sPAP + 2, left atrial pressure (LAP) = 1.9 + 1.24 x left ventricular (LV) E/e' and pulmonary vascular resistance (PVR) = (mPAP-LAP) / LV cardiac output (CO) and slope of mPAP-LVCO relationship=changes in mPAP / changes in LVCO. Resting PH was present in 3 patients (7%) and 21 patients developed EIPH (47%). Patients with EIPH had higher resting LAP (10.3 +/- 2.2 versus 8.8 +/- 2.3 mm Hg; p = 0.03), resting PVR (2.6 +/- 0.8 vs. 1.4 +/- 1.1 Woods units; p = 0.004), exercise LAP (13.3 +/- 2.3 vs. 9 +/- 1.7 mm Hg; p < 0.0001), exercise PVR (3.6 +/- 0.7 vs. 2.1 +/- 0.9 Woods units; p = 0.02) and slope of mPAP-LVCO (5.8 +/- 2.4 vs. 2.9 +/- 2.1 mm Hg/L/min; p < 0.0001). After adjustment for age and gender, exercise LAP (beta = 3.1 +/- 0.8; p = 0.001) and exercise PVR (beta = 7.9 +/- 1.7; p = 0.0001) were independent determinants of exercise sPAP. Conclusion: EIPH is frequent in SSc patients and is mainly related to both increased exercise LV filling pressure and exercise PVR. (C) 2014 Elsevier Ireland Ltd. All rights reserved.