Determinants of exercise-induced pulmonary arterial hypertension in systemic sclerosis

Abstract

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 Liège. 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 N35 mm Hg and EIPH as sPAP N 50 mm Hg during exercise. The following formulas were used: mean PAP (mPAP) = 0.61 × sPAP + 2, left atrial pressure (LAP) = 1.9 + 1.24 × 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 b 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 b 0.0001). After adjustment for age and gender, exercise LAP (β = 3.1 ± 0.8; p = 0.001) and exercise PVR (β = 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.

Aerobic and resistance exercise in systemic sclerosis: State of the art

“…Reduced diffusion lung capacity for carbon monoxide (DLCO) and reduced predicted lung volumes seem to predict oxygen desaturation in exercise testing (Someya et al, ). PAH and exercise‐induced PAH are common features in SSc (Gargani et al, ; Saggar et al, ; Suzuki et al, ; Voilliot et al, ). Exercise‐induced PAH is mainly related to increased left ventricular filling pressure and exercise pulmonary vascular resistance (Voilliot et al, ), and may reduce peripheral capillary oxygen saturation ( S pO 2 ) (Yasunobu, Oudiz, Sun, Hansen, & Wasserman, ).…”

Contractile reserve in systemic sclerosis patients as a major predictor of global cardiac impairment and exercise tolerance

“…Recent studies in SSc patients showed an excessive increase in PAP during physical exercise, associated not only with increase in pulmonary vascular resistance, but also with left ventricle diastolic dysfunction [11,12]. In previous studies, stress echocardiography has been used mainly to verify whether an exercise echocardiogram could identify SSc patients who may develop PH [29,30].…”

“…Exercise echocardiography (ExE) has previously been used in the evaluation of patients with scleroderma and in familial forms of pulmonary hypertension [10][11][12]; more recently, Collins and coll. used this technique to evaluate the change in pulmonary artery pressure in patients with systemic lupus erythematous and with limited and diffuse SSc.…”

Clinical significance of dynamic pulmonary vascular resistance in two populations at risk of pulmonary arterial hypertension

“…Similarly, patients with SSc were consecutively selected from the rheumatology database of the University Hospital of Liège from January 2008 to November 2012. 21 All patients signed informed consent, after the University Hospitals Institutional Review Boards approved the study protocol.…”

“…Methodological details and baseline characteristics have already been published. 6,21 Bicycle stress echocardiography was performed in a semi-supine position, with the exercise table tilted 20 -308 to the left. Patients started at an initial workload of 25 W, which was increased by 25 W every 2 min, until the maximal tolerated workload.…”