title: [Hemodynamic Predictors of Clinical Deterioration in Patients With Pulmonary Arterial Hypertension Associated With Systemic Scleroderma].
abstract: to elucidate hemodynamic predictors of clinical deterioration (CD) in patients with pulmonary arterial hypertension (PAH) associated with systemic scleroderma (SSD).
  
  
  
  We included into this study 48 patients with PAH-SSD consecutively admitted in 2004-2014. At inclusion all patients underwent right heart catheterization (RHC) and thereafter were under dynamic observation. CD deterioration was diagnosed in the presence of the following: >15% decline in 6-minute walk test distance; worsening of PAH functional class; intensification of symptoms of right ventricular failure; necessity in administration of parenteral diuretics. RHC was used for confirmation of CD. Relative risk (RR) of events was calculated for identification of significant predictors of CD. Cut points were determined with the help of construction of characteristic curves, statistical significance was estimated in Kaplan-Meier analysis.
  
  
  
  During follow-up (duration 46 [23;91] months) 21 patients had 24 CDs confirmed by RHC. Calculation of RR identified a number of hemodynamic parameters related to CD development. ROC analysis confirmed significance of initial right atrial pressure (RAP) 6.5 mm Hg (95% confidence interval [CI] 0.59-0.89, sensitivity 71%, specificity 63%, p<0.004) and dynamics of pulmonary vascular resistance (PVR) -1.06 Wood units (95%CI 0.790.99; sensitivity 76%, specificity 80%, <0.0001). Kaplan-Meier analysis revealed significant differences in times to CD (median 31.5 and 10 months in patients with RAP <6.5 and more or equal 6.5 mm Hg, respectively, p=0.01; 37 and 17months in patients with PVR lowering >1.06 Wood units and no PVR lowering, respectively, p=0.009.
  
  
  
  We identified prognostic levels of key hemodynamic predictors of CD in patients with SSD and PAH (RAP 6.5 mm Hg, PVR - 1. 06 Wood units) which can be used for individualization and optimization of therapy.
authors.affiliation: V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.
authors.forename: E V
authors.initials: EV
authors.lastname: Nikolaeva
authors.affiliation: V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.
authors.forename: I A
authors.initials: IA
authors.lastname: Kurmukov
authors.affiliation: V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.
authors.forename: N N
authors.initials: NN
authors.lastname: Yudkina
authors.affiliation: V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.
authors.forename: S I
authors.initials: SI
authors.lastname: Glukhova
authors.affiliation: V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.
authors.forename: A V
authors.initials: AV
authors.lastname: Volkov
mesh_terms: D000328:Adult; D006328:Cardiac Catheterization; D000075902:Clinical Deterioration; D005260:Female; D006333:Heart Failure; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D014655:Vascular Resistance
keywords: bosentan; clinical deterioration; iloprost; prognosis; pulmonary arterial hypertension; right heart catheterization; sildenafil