Echocardiography. 2018;35:841–848. wileyonlinelibrary.com/journal/echo
© 2018 Wiley Periodicals, Inc.
1 | INTRODUCTION
Comprehensive echocardiographic assessment of children and
adults with pulmonary hypertension (PH) includes several variables
of right ventricular (RV) and right atrial (RA) size, morphology, and
However, data regarding the effects of increased right
ventricular pressure afterload on RV outflow tract (RVOT) size and
systolic function are lacking in children with PH, including those
subgroups with pulmonary arterial hypertension (PAH) and PH as-
sociated with bronchopulmonary dysplasia (PH- BPD). Guidelines
The right ventricular outflow tract in pediatric pulmonary
hypertension—Data from the European Pediatric Pulmonary
Vascular Disease Network
Martin Koestenberger MD
| Alexander Avian PhD
| Hannes Sallmon MD
Andreas Gamillscheg MD
| Gernot Grangl MD
| Stefan Kurath-Koller MD
Sabrina Schweintzger MD
| Ante Burmas MD
| Georg Hansmann MD, PhD
Division of Pediatric
Cardiology, Department of
Pediatrics, Medical University Graz, Graz,
Institute for Medical Informatics, Statistics
and Documentation, Medical University
Graz, Graz, Austria
Department of Neonatology, Charité —
Universitätsmedizin Berlin, Berlin, Germany
Department of Pediatric Cardiology and
Critical Care, Hannover Medical School,
Martin Koestenberger, Division of Pediatric
Cardiology, Department of Pediatrics,
Medical University Graz, Graz, Austria.
GH currently receives grant support from
the German Research Foundation (DFG; HA
4348/6- 1, KFO311).
Objective: The right ventricular outflow tract (RVOT) is pivotal for adequate RV
function and known to be adversely affected by elevated pulmonary arterial pres-
sure (PAP) in adults with pulmonary hypertension (PH). Aim of this study was to de-
termine the effects of increased RV pressure afterload in children with PH on RVOT
size, function, and flow parameters.
Methods: We conducted a transthoracic echocardiographic study in 51 children with
PH (median age: 5.3 years; range 1.5 months to 18 years) and determined the follow-
ing RVOT variables: RVOT diameter, RVOT velocity time integral (VTI), ratio of tricus-
pid regurgitation velocity (TRV)/RVOT VTI, and RVOT systolic excursion (SE).
Results: In our pediatric PH cohort, the age- specific RVOT diameter z- score was
higher compared to normal values. Deviation from normal RVOT diameter values in-
creased with age, disease severity, and New York Heart Association functional class.
Significant correlations were found between RVOT diameter and the RV end- diastolic
area and right atrial area. The age- specific RVOT VTI z- score values were significantly
lower in children with PH vs healthy controls. The TRV/RVOT VTI ratio increased
with rising systolic RV pressure, while the RVOT SE was similar between PH children
and control subjects.
Conclusions: In pediatric PH cohort, the RVOT VTI is decreased, and the TRV/RVOT
VTI ratio and the RVOT diameter increased compared to healthy subjects. Assessment
of RVOT variables, together with established RV parameters, allows for a compre-
hensive assessment of global right heart size and performance in children with PH.
pediatric, pulmonary hypertension, ratio of tricuspid regurgitation/RVOT velocity time
integral, right ventricular outflow tract diameter, RVOT velocity time integral