title: Prognostic relevance of pulmonary arterial compliance after therapy initiation or escalation in patients with pulmonary arterial hypertension.
abstract: BACKGROUND
  Conventional hemodynamic parameters are considered to be the gold standard indices of outcome in pulmonary arterial hypertension (PAH); on the contrary, few data support the hypothesis that the pulsatile component of right ventricular afterload provides important prognostic information. The aim of the study was to investigate the prognostic significance of pulmonary arterial compliance (PCa) after therapy initiation or escalation in PAH patients.
  
  
  METHODS
  A cohort of 419 consecutive PAH patients (308 naive and 111 prevalent) underwent right heart catheterisation (RHC) prior to initiating or escalating PAH-targeted therapy. RHC was repeated in 255 patients (61%) after 4 to 12months of therapy as 62 patients (15%) died and 102 (24%) did not undergo a follow-up RHC within the first year.
  
  
  RESULTS
  After the follow-up RHC, 63 patients died over a median follow-up period of 39months. At multivariate analysis, age>50years old, male gender, etiology associated with systemic sclerosis, persistence of WHO class III/IV, and reduced PCa at follow-up RHC were the independent parameters significantly associated with poor prognosis. At ROC analysis, the optimal cut-off point of PCa to predict survival was 1.4mL/mmHg (AUC 0.73, sensitivity 81.8%, specificity 58.8%).
  
  
  CONCLUSIONS
  In PAH patients hospitalized to initiate or to escalate PAH-specific therapy, failure to improve PCa after therapy is a strong hemodynamic predictor of poor prognosis.
authors.affiliation: Dept. of Cardiology, Fondazione IRCCS Policlinico S.Matteo, Pavia, Italy. Electronic address: s.ghio@smatteo.pv.it.
authors.forename: Stefano
authors.initials: S
authors.lastname: Ghio
authors.affiliation: Dept. of Cardiology, Second University of Naples - Monaldi Hospital, Naples, Italy.
authors.forename: Michele
authors.initials: M
authors.lastname: D'Alto
authors.affiliation: Pulmonary Hypertension Unit, Dept. of Cardiovascular and Respiratory Science, Sapienza University of Rome, Rome, Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: Badagliacca
authors.affiliation: Pulmonology Unit, Dept. for the Treatment and Study of Cardiothoracic Diseases and Cardiothoracic Transplantation, IRCCS - ISMETT, Palermo, Italy.
authors.forename: Patrizio
authors.initials: P
authors.lastname: Vitulo
authors.affiliation: Dept. of Cardiology, Second University of Naples - Monaldi Hospital, Naples, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Argiento
authors.affiliation: Ferrarotto Hospital, Catania, Italy.
authors.forename: Massimiliano
authors.initials: M
authors.lastname: Mulè
authors.affiliation: Pulmonology Unit, Dept. for the Treatment and Study of Cardiothoracic Diseases and Cardiothoracic Transplantation, IRCCS - ISMETT, Palermo, Italy.
authors.forename: Fabio
authors.initials: F
authors.lastname: Tuzzolino
authors.affiliation: Dept. of Cardiology, Fondazione IRCCS Policlinico S.Matteo, Pavia, Italy.
authors.forename: Laura
authors.initials: L
authors.lastname: Scelsi
authors.affiliation: Dept. of Cardiology, Second University of Naples - Monaldi Hospital, Naples, Italy.
authors.forename: Emanuele
authors.initials: E
authors.lastname: Romeo
authors.affiliation: Dept. of Cardiology, Fondazione IRCCS Policlinico S.Matteo, Pavia, Italy.
authors.forename: Claudia
authors.initials: C
authors.lastname: Raineri
authors.affiliation: Pulmonology Unit, Dept. for the Treatment and Study of Cardiothoracic Diseases and Cardiothoracic Transplantation, IRCCS - ISMETT, Palermo, Italy.
authors.forename: Lavinia
authors.initials: L
authors.lastname: Martino
authors.affiliation: Ferrarotto Hospital, Catania, Italy.
authors.forename: Corrado
authors.initials: C
authors.lastname: Tamburino
authors.affiliation: Pulmonary Hypertension Unit, Dept. of Cardiovascular and Respiratory Science, Sapienza University of Rome, Rome, Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: Poscia
authors.affiliation: Pulmonary Hypertension Unit, Dept. of Cardiovascular and Respiratory Science, Sapienza University of Rome, Rome, Italy.
authors.forename: Carmine Dario
authors.initials: CD
authors.lastname: Vizza
mesh_terms: D000368:Aged; D006328:Cardiac Catheterization; D015331:Cohort Studies; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011651:Pulmonary Artery / Q000503:physiopathology*; D012372:ROC Curve; D014655:Vascular Resistance / Q000502:physiology
keywords: Prognosis; Pulmonary arterial hypertension; Right heart hemodynamics