title: Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension.
abstract: OBJECTIVE
  The aim of this study was to evaluate the relationships between echocardiographic findings and clinical outcomes in patients with severe primary pulmonary hypertension (PPH).
  
  
  BACKGROUND
  Primary pulmonary hypertension is associated with abnormalities of right heart structure and function that contribute to the poor prognosis of the disease. Echocardiographic abnormalities associated with PPH have been described, but the prognostic significance of these findings remains poorly characterized.
  
  
  METHODS
  Echocardiographic studies, invasive hemodynamic measurements and 6-min walk tests were performed and outcomes prospectively followed in 81 patients with severe PPH. Subjects were participants in a 12-week randomized trial examining the effects of prostacyclin plus conventional therapy compared with conventional therapy alone.
  
  
  RESULTS
  During the mean follow-up period of 36.9 +/- 15.4 months, 20 patients died and 21 patients underwent transplantation. Pericardial effusion (p = 0.003) and indexed right atrial area (p = 0.005) were predictors of mortality. Pericardial effusion (p = 0.017), indexed right atrial area (p = 0.012) and the degree of septal shift in diastole (p = 0.004) were predictors of a composite end point of death or transplantation. In multivariable analyses incorporating clinical, hemodynamic and echocardiographic variables, pericardial effusion and an enlarged right atrium remained predictors of adverse outcomes. Six-minute walk results, mixed venous oxygen saturation and initial treatment randomization were also independently associated with a poor prognosis.
  
  
  CONCLUSIONS
  Pericardial effusion, right atrial enlargement and septal displacement are echocardiographic abnormalities that reflect the severity of right heart failure and predict adverse outcomes in patients with severe PPH. These characteristics may help identify patients appropriate for more intensive medical therapy or earlier transplantation.
authors.affiliation: University of North Carolina, Chapel Hill, North Carolina 27599-7075, USA.
authors.forename: Ronald J
authors.initials: RJ
authors.lastname: Raymond
authors.forename: Alan L
authors.initials: AL
authors.lastname: Hinderliter
authors.forename: Park W
authors.initials: PW
authors.lastname: Willis
authors.forename: David
authors.initials: D
authors.lastname: Ralph
authors.forename: Edgar J
authors.initials: EJ
authors.lastname: Caldwell
authors.forename: William
authors.initials: W
authors.lastname: Williams
authors.forename: Neil A
authors.initials: NA
authors.lastname: Ettinger
authors.forename: Nicholas S
authors.initials: NS
authors.lastname: Hill
authors.forename: Warren R
authors.initials: WR
authors.lastname: Summer
authors.forename: Bennett
authors.initials: B
authors.lastname: de Boisblanc
authors.forename: Todd
authors.initials: T
authors.lastname: Schwartz
authors.forename: Gary
authors.initials: G
authors.lastname: Koch
authors.forename: Linda M
authors.initials: LM
authors.lastname: Clayton
authors.forename: Maria M
authors.initials: MM
authors.lastname: Jöbsis
authors.forename: James W
authors.initials: JW
authors.lastname: Crow
authors.forename: Walker
authors.initials: W
authors.lastname: Long
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use; D016280:Atrial Function, Right / Q000502:physiology; D004452:Echocardiography; D011464:Epoprostenol / Q000627:therapeutic use; D017079:Exercise Tolerance; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000401:mortality; D008297:Male; D010490:Pericardial Effusion / Q000453:epidemiology; D011379:Prognosis; D016016:Proportional Hazards Models; D013997:Time Factors; D016278:Ventricular Function, Right / Q000502:physiology
chemical_list: D000959:Antihypertensive Agents; D011464:Epoprostenol