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Prognostic factors, treatment goals and clinical endpoints in pediatric pulmonary
arterial hypertension
Ploegstra, Mark-Jan
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2017
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Ploegstra, M-J. (2017). Prognostic factors, treatment goals and clinical endpoints in pediatric pulmonary
arterial hypertension [Groningen]: Rijksuniversiteit Groningen
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Download date: 24-04-2017
Prognostic factors, treatment goals
and clinical endpoints in pediatric
pulmonary arterial hypertension
Mark-Jan Ploegstra
Mark-Jan Ploegstra
Prognostic factors, treatment goals and clinical endpoints in pediatric pulmonary arterial hypertension
Financial support for the publication of this thesis by the following companies / institutes is gratefully
acknowledged:
Abbvie
Actelion Pharmaceuticals
Chipsoft
Graduate School of Medical Sciences
Heart Medical
Longfonds
Pfizer
Roche Diagnostics
Rijksuniversiteit Groningen
Salveo Medical
Scovas Medical
St. Jude Medical
Stichting PHA
Therabel Pharma
Universitair Medisch Centrum Groningen
Copyright © 2016, Mark-Jan Ploegstra
All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or
transmitted in any form or by an means, without the written permission of the author.
ISBN: 978-94-6169-996-1
e-pub ISBN: 978-90-8559-243-3
Cover design, layout and printing: Optima Grafische Communicatie, Rotterdam, The Netherlands
Prognostic factors, treatment goals and
clinical endpoints in pediatric pulmonary
arterial hypertension
Proefschrift
ter verkrijging van de graad van doctor aan de
Rijksuniversiteit Groningen
op gezag van de
rector magnificus prof. dr. E. Sterken
en volgens besluit van het College voor Promoties.
De openbare verdediging zal plaatsvinden op
woensdag 1 maart 2017 om 14.30 uur
door
Mark-Jan Ploegstra
geboren op 12 mei 1987
te Dongeradeel
Promotores
Prof. dr. R.M.F. Berger
Prof. dr. H.L. Hillege
Beoordelingscommissie
Prof. dr. S.H. Abman
Prof. dr. R. Naeije
Prof. mr. dr. A.A.E. Verhagen
Financial support by the Dutch Heart Foundation for the publication of this thesis is
gratefully acknowledged.
Paranimfen:
Mw. drs. W.M. Ploegstra
Mr. J. Veninga
Van alles waarover u waakt, waak vooral over uw hart,
het is de bron van uw leven. (Spreuken 4:23)
Contents
Chapter 1
Chapter 2
General introduction and aims of this thesis
9
I. Introduction to pediatric pulmonary arterial hypertension
11
II. Current challenges in pediatric pulmonary arterial
hypertension
18
Current and advancing treatments for pulmonary arterial
hypertension in childhood
31
Expert Review of Respiratory Medicine. 2014; 8: 615-628
Chapter 3
Prognostic factors in pediatric pulmonary arterial hypertension:
A systematic review and meta-analysis
59
International Journal of Cardiology. 2015; 184: 198-207
Chapter 4
Echocardiography in pediatric pulmonary arterial hypertension:
early study on assessing disease severity and predicting outcome
95
Circulation Cardiovascular Imaging. 2014; 8: e000878
Chapter 5
Growth in children with pulmonary arterial hypertension: a
longitudinal retrospective multi-registry study
119
The Lancet Respiratory Medicine. 2016; 4: 281-290
Chapter 6
Serially measured uric acid levels predict disease severity and
outcome in pediatric pulmonary arterial hypertension
143
American Journal of Respiratory and Critical Care Medicine. 2017; 195
Chapter 7
Pulmonary arterial stiffness indices assessed by intravascular
ultrasound in children with early pulmonary vascular disease:
prediction of disease progression and mortality during 20-year
follow-up
163
European Heart Journal - Cardiovascular Imaging. 2017; 18
Chapter 8
Identification of treatment goals in pediatric pulmonary arterial
hypertension
European Respiratory Journal. 2014; 44: 1616-1626
183
Chapter 9
Clinical worsening as composite study end point in pediatric
pulmonary arterial hypertension
207
Chest. 2015; 148: 655-666
Chapter 10
General discussion and future prospects
Appendices English summary
233
255
Nederlandse samenvatting
257
Dankwoord
263
About the author
267
List of publications
269