title: Right ventricular diastolic performance in children with pulmonary arterial hypertension associated with congenital heart disease: correlation of echocardiographic parameters with invasive reference standards by high-fidelity micromanometer catheter.
abstract: BACKGROUND
  Right ventricular diastolic dysfunction influences outcomes in pulmonary arterial hypertension (PAH), but echocardiographic parameters have not been investigated in relation to invasive reference standards in pediatric PAH. We investigated echocardiographic parameters of right ventricular diastolic function in children with PAH in relation to simultaneously measured invasive reference measures.
  
  
  RESULTS
  We prospectively recruited children undergoing a clinically indicated cardiac catheterization for evaluation of PAH and pulmonary vasoreactivity testing. Echocardiography was performed simultaneously with invasive reference measurements by high-fidelity micromanometer catheter. For analysis, patients were divided into shunt and nonshunt groups. Sixteen children were studied. In the group as a whole, significant correlations were found among τ and tricuspid deceleration time, E', E/E', TimeE-E', A wave velocity, and global early and late diastolic strain rate. dp/dt minimum correlated significantly with late diastolic tricuspid annular velocity (A'), tissue Doppler imaging-derived systolic:diastolic duration ratio, and global late diastolic strain rate. End-diastolic pressure correlated significantly with tissue Doppler imaging-derived systolic:diastolic duration ratio. On multivariate analysis, tricuspid deceleration time, TimeE-E', and global early diastolic strain rate were independent predictors of τ, whereas tissue Doppler imaging-derived systolic:diastolic duration ratio was an independent predictor of dp/dt minimum. In general, correlations between echocardiographic and invasive parameters were better in the shunt group than in the nonshunt group.
  
  
  CONCLUSIONS
  Echocardiography correlates with invasive reference measures of right ventricular diastolic function in children with PAH, although it does not differentiate between early versus late diastolic abnormalities. Newer echocardiographic techniques may have added value to assess right ventricular diastolic dysfunction in this population.
authors.affiliation: From the Division of Cardiology, The Labatt Family Heart Center, Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Kenichi
authors.initials: K
authors.lastname: Okumura
authors.affiliation: From the Division of Cardiology, The Labatt Family Heart Center, Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Cameron
authors.initials: C
authors.lastname: Slorach
authors.affiliation: From the Division of Cardiology, The Labatt Family Heart Center, Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Dariusz
authors.initials: D
authors.lastname: Mroczek
authors.affiliation: From the Division of Cardiology, The Labatt Family Heart Center, Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Andreea
authors.initials: A
authors.lastname: Dragulescu
authors.affiliation: From the Division of Cardiology, The Labatt Family Heart Center, Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Luc
authors.initials: L
authors.lastname: Mertens
authors.affiliation: From the Division of Cardiology, The Labatt Family Heart Center, Hospital for Sick Children, University of Toronto, Toronto, Canada.
authors.forename: Andrew N
authors.initials: AN
authors.lastname: Redington
authors.affiliation: From the Division of Cardiology, The Labatt Family Heart Center, Hospital for Sick Children, University of Toronto, Toronto, Canada. mark.friedberg@sickkids.ca.
authors.forename: Mark K
authors.initials: MK
authors.lastname: Friedberg
mesh_terms: D006328:Cardiac Catheterization / Q000295:instrumentation / Q000379:methods; D002648:Child; D003971:Diastole; D015150:Echocardiography, Doppler / Q000379:methods; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D008365:Manometry / Q000295:instrumentation* / Q000379:methods; D015588:Observer Variation; D011446:Prospective Studies; D012015:Reference Standards; D015203:Reproducibility of Results; D018497:Ventricular Dysfunction, Right / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D016278:Ventricular Function, Right
keywords: echocardiography; pulmonary arterial hypertension; ventricular function, right