title: Tricuspid annular plane systolic excursion is a robust outcome measure in systemic sclerosis-associated pulmonary arterial hypertension.
abstract: OBJECTIVE
  The tricuspid annular plane systolic excursion (TAPSE) strongly reflects right ventricular (RV) function and predicts survival in idiopathic pulmonary arterial hypertension (PAH). But its role in systemic sclerosis (SSc)-associated PAH has not been established. Our objective was to validate the TAPSE in the assessment of RV function and prediction of survival in SSc-PAH.
  
  
  METHODS
  Fifty consecutive patients with SSc-PAH who underwent echocardiography with TAPSE measurement within 1 h of clinically indicated right heart catheterization were followed prospectively. The relationship between TAPSE and measures of RV function and measures of survival was assessed.
  
  
  RESULTS
  The majority of the cohort were women in New York Heart Association class III/IV with severe PAH (mean cardiac index 2.4 ± 0.8 l/min/m(2)). RV function was significantly impaired (mean cardiac index 2.1 ± 0.7 vs 2.9 ± 0.8 l/min/m(2); p < 0.01) and RV afterload was significantly greater (mean pulmonary vascular resistance 11.1 ± 5.1 vs 5.8 ± 2.5 Wood units; p < 0.01) in subjects with a TAPSE ≤ 1.7 cm. The proportion surviving in the low TAPSE group was significantly lower [0.56 (95% CI 0.37-0.71) and 0.46 (95% CI 0.28-0.62) vs 0.87 (95% CI 0.55-0.96) and 0.79 (95% CI 0.49-0.93), 1- and 2-year survival, respectively]. TAPSE ≤ 1.7 cm conferred a nearly 4-fold increased risk of death (HR 3.81, 95% CI 1.31-11.1, p < 0.01).
  
  
  CONCLUSIONS
  TAPSE is a robust measure of RV function and strongly predicts survival in patients with PAH-SSc. Future studies are needed to identify the responsiveness of TAPSE to PAH-specific therapy and to assess its diagnostic utility in PAH-SSc.
authors.affiliation: Divisions of Pulmonary and Critical Care Medicine, Cardiology, and Rheumatology, Department of Medicine, Johns Hopkins, University School of Medicine, Baltimore, Maryland, USA. smathai4@jhmi.edu
authors.forename: Stephen C
authors.initials: SC
authors.lastname: Mathai
authors.forename: Christopher T
authors.initials: CT
authors.lastname: Sibley
authors.forename: Paul R
authors.initials: PR
authors.lastname: Forfia
authors.forename: James O
authors.initials: JO
authors.lastname: Mudd
authors.forename: Micah R
authors.initials: MR
authors.lastname: Fisher
authors.forename: Ryan J
authors.initials: RJ
authors.lastname: Tedford
authors.forename: Noah
authors.initials: N
authors.lastname: Lechtzin
authors.forename: Danielle
authors.initials: D
authors.lastname: Boyce
authors.forename: Laura K
authors.initials: LK
authors.lastname: Hummers
authors.forename: Traci
authors.initials: T
authors.lastname: Housten
authors.forename: Ari L
authors.initials: AL
authors.lastname: Zaiman
authors.forename: Reda E
authors.initials: RE
authors.lastname: Girgis
authors.forename: Paul M
authors.initials: PM
authors.lastname: Hassoun
mesh_terms: D000368:Aged; D001794:Blood Pressure / Q000502:physiology; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006352:Heart Ventricles / Q000000981:diagnostic imaging*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000401:mortality / Q000503:physiopathology*; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D017063:Outcome Assessment, Health Care; D011237:Predictive Value of Tests; D011446:Prospective Studies; D012039:Regional Blood Flow / Q000502:physiology; D015203:Reproducibility of Results; D012595:Scleroderma, Systemic / Q000150:complications* / Q000401:mortality / Q000503:physiopathology*; D012680:Sensitivity and Specificity; D015996:Survival Rate; D013599:Systole / Q000502:physiology; D014261:Tricuspid Valve / Q000000981:diagnostic imaging* / Q000502:physiology; D014463:Ultrasonography; D014655:Vascular Resistance / Q000502:physiology; D016278:Ventricular Function, Right / Q000502:physiology*