title: Tissue Doppler imaging predicts adverse outcome in children with idiopathic pulmonary arterial hypertension.
abstract: OBJECTIVE
  To evaluate the clinical utility of tissue Doppler imaging (TDI) in assessment of disease severity and prognostic value in children with idiopathic pulmonary arterial hypertension (PAH).
  
  
  METHODS
  A prospective study was performed to evaluate TDI velocities (systolic myocardial velocity, early diastolic myocardial relaxation velocity [Em], late diastolic myocardial velocity associated with atrial contraction), brain natriuretic peptide, New York Heart Association (NYHA) functional class, and hemodynamics in 51 children (mean age; 11.6 years) with idiopathic PAH. Fifty-one healthy children with comparable demographics served as controls.
  
  
  RESULTS
  Em, Em/late diastolic myocardial velocity associated with atrial contraction ratio, and systolic myocardial velocity at mitral annulus, septum, and tricuspid annulus in PAH were significantly reduced compared with controls. Tricuspid Em had significant inverse correlations with plasma brain natriuretic peptide levels (r = -0.60, P < .001), right ventricular end-diastolic pressure (r = -0.79, P < .001), and mean pulmonary arterial pressure (r = -0.67, P < .001). Statistically significant differences were observed in tricuspid Em between NYHA functional class II vs combined III and IV (mean and SD; 11.9 ± 4.2 cm/s vs 8.2 ± 3.6 cm/s, respectively, P = .002). Cumulative event-free survival rate was significantly lower when tricuspid Em was ≤8 cm/s (log-rank test, P < .001)
  
  
  CONCLUSIONS
  Tricuspid Em velocity correlated with NYHA functional class as disease severity and may serve as a useful prognostic marker in children with idiopathic PAH. The present study is the initial report to evaluate TDI velocities against midterm outcome variables in a relatively large pediatric PAH population.
authors: Department of Pediatrics, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO, USA.
authors: Shinichi
authors: S
authors: Takatsuki
authors: Tomotaka
authors: T
authors: Nakayama
authors: Pei-Ni
authors: PN
authors: Jone
authors: Brandie D
authors: BD
authors: Wagner
authors: Kazuyuki
authors: K
authors: Naoi
authors: D Dunbar
authors: DD
authors: Ivy
authors: Tsutomu
authors: T
authors: Saji
mesh_terms: D000293:Adolescent; D015415:Biomarkers / Q000097:blood; D001783:Blood Flow Velocity; D001794:Blood Pressure; D016022:Case-Control Studies; D002648:Child; D018572:Disease-Free Survival; D018618:Echocardiography, Doppler, Color; D018619:Echocardiography, Doppler, Pulsed; D005080:Exercise Test; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000000981:diagnostic imaging* / Q000503:physiopathology; D053208:Kaplan-Meier Estimate; D016015:Logistic Models; D008297:Male; D020097:Natriuretic Peptide, Brain / Q000097:blood; D011379:Prognosis; D011446:Prospective Studies; D012372:ROC Curve; D012720:Severity of Illness Index
chemical_list: D015415:Biomarkers; D020097:Natriuretic Peptide, Brain