title: Pulmonary vasoreactivity predicts long-term outcome in patients with Eisenmenger syndrome receiving bosentan therapy.
abstract: BACKGROUND
  Vasoreactivity testing is recommended in the management of pulmonary arterial hypertension (PAH), but its clinical relevance in congenital heart disease (CHD)-associated PAH has not been established.
  
  
  OBJECTIVE
  To determine whether residual pulmonary vascular responsiveness to intravenous. epoprostenol is predictive of clinical outcome in patients with CHD-PAH and Eisenmenger syndrome.
  
  
  RESULTS
  A diagnostic right heart catheterisation with reversibility testing using epoprostenol infusion was performed in 38 consecutive patients with CHD-PAH and Eisenmenger syndrome. Patients were treated with bosentan and were assessed every 3 months. Clinical worsening was defined as death from any cause, heart-lung or lung transplantation (or on the waiting list for this procedure), hospitalisation for PAH, or symptom exacerbation defined as a > or =20% decrease in the 6 min walking distance on two consecutive tests, an increase in WHO functional class, or worsening right heart failure. The mean follow-up was 33+/-17 months. Sixteen patients showed clinical worsening. Although they did not differ from the other patients in their baseline exercise capacity, haemodynamic characteristics and underlying CHD, pulmonary vascular resistance index (PVRi) was less reversible (DeltaPVRi 29+/-21 vs 52+/-14%, p=0.0003). At univariate analysis, systemic vascular resistance, PVRi and DeltaPVRi were significant predictors of clinical worsening. At multivariate Cox proportional hazards regression model, DeltaPVRi was found to be the only independent predictor of clinical worsening (HR=0.973, 95% CI 0.95 to 0.99; p=0.01). DeltaPVRi > or =25% had a positive and negative predictive value for clinical worsening of 100% and 75.9%, respectively.
  
  
  CONCLUSIONS
  Pulmonary vasoreactivity is a significant predictor of clinical worsening in patients with CHD-PAH.
authors.affiliation: Second University of Naples, A.O. V. Monaldi, Naples, Italy. mic.dalto@tin.it
authors.forename: Michele
authors.initials: M
authors.lastname: D'Alto
authors.forename: Emanuele
authors.initials: E
authors.lastname: Romeo
authors.forename: Paola
authors.initials: P
authors.lastname: Argiento
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Santoro
authors.forename: Berardo
authors.initials: B
authors.lastname: Sarubbi
authors.forename: Giampiero
authors.initials: G
authors.lastname: Gaio
authors.forename: Christian
authors.initials: C
authors.lastname: Mélot
authors.forename: Maria Giovanna
authors.initials: MG
authors.lastname: Russo
authors.forename: Robert
authors.initials: R
authors.lastname: Naeije
authors.forename: Raffaele
authors.initials: R
authors.lastname: Calabrò
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D006328:Cardiac Catheterization / Q000379:methods; D004541:Eisenmenger Complex / Q000188:drug therapy* / Q000503:physiopathology; D004812:Epidemiologic Methods; D011464:Epoprostenol; D017079:Exercise Tolerance / Q000502:physiology; D005260:Female; D006439:Hemodynamics / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011651:Pulmonary Artery / Q000503:physiopathology; D013449:Sulfonamides / Q000627:therapeutic use*; D016896:Treatment Outcome; D014655:Vascular Resistance / Q000187:drug effects; D055815:Young Adult
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D011464:Epoprostenol; D000077300:Bosentan