title: 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.
  
  
  RESULTS
  We prospectively enrolled 63 patients with SSc (age 54±3years, 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 > 35 mmHg and EIPH as sPAP > 50 mmHg 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 mmHg; 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 mmHg; 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 mmHg/L/min; p < 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.
  
  
  CONCLUSIONS
  EIPH is frequent in SSc patients and is mainly related to both increased exercise LV filling pressure and exercise PVR.
authors.affiliation: University of Liège Hospital, GIGA Cardiovascular Sciences, Department of Cardiology, Heart Valve Clinic, University Hospital Sart Tilman, Liège, Belgium.
authors.forename: Damien
authors.initials: D
authors.lastname: Voilliot
authors.affiliation: University of Liège Hospital, GIGA Cardiovascular Sciences, Department of Cardiology, Heart Valve Clinic, University Hospital Sart Tilman, Liège, Belgium.
authors.forename: Julien
authors.initials: J
authors.lastname: Magne
authors.affiliation: University of Liège Hospital, GIGA Cardiovascular Sciences, Department of Cardiology, Heart Valve Clinic, University Hospital Sart Tilman, Liège, Belgium.
authors.forename: Raluca
authors.initials: R
authors.lastname: Dulgheru
authors.affiliation: University of Liège Hospital, GIGA Cardiovascular Sciences, Department of Cardiology, Heart Valve Clinic, University Hospital Sart Tilman, Liège, Belgium.
authors.forename: Seisyou
authors.initials: S
authors.lastname: Kou
authors.affiliation: University of Liège Hospital, GIGA Cardiovascular Sciences, Department of Cardiology, Heart Valve Clinic, University Hospital Sart Tilman, Liège, Belgium.
authors.forename: Christine
authors.initials: C
authors.lastname: Henri
authors.affiliation: University of Liège Hospital, GIGA Cardiovascular Sciences, Department of Cardiology, Heart Valve Clinic, University Hospital Sart Tilman, Liège, Belgium.
authors.forename: Saloua
authors.initials: S
authors.lastname: Laaraibi
authors.affiliation: University of Liège Hospital, GIGA Cardiovascular Sciences, Department of Cardiology, Heart Valve Clinic, University Hospital Sart Tilman, Liège, Belgium.
authors.forename: Muriel
authors.initials: M
authors.lastname: Sprynger
authors.affiliation: University of Liège Hospital, Department of Rheumatology, University Hospital Sart Tilman, Liège, Belgium.
authors.forename: Béatrice
authors.initials: B
authors.lastname: Andre
authors.affiliation: University of Liège Hospital, GIGA Cardiovascular Sciences, Department of Cardiology, Heart Valve Clinic, University Hospital Sart Tilman, Liège, Belgium. Electronic address: lpierard@chu.ulg.ac.be.
authors.forename: Luc A
authors.initials: LA
authors.lastname: Pierard
authors.affiliation: University of Liège Hospital, GIGA Cardiovascular Sciences, Department of Cardiology, Heart Valve Clinic, University Hospital Sart Tilman, Liège, Belgium. Electronic address: plancellotti@chu.ulg.ac.be.
authors.forename: Patrizio
authors.initials: P
authors.lastname: Lancellotti
mesh_terms: D000328:Adult; D000368:Aged; D015444:Exercise / Q000502:physiology*; D005080:Exercise Test / Q000379:methods*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012146:Rest / Q000502:physiology; D012595:Scleroderma, Systemic / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology*
keywords: Echocardiography; Exercise; Pulmonary hypertension; Scleroderma