Clinical Worsening as Composite Study End Point in Pediatric Pulmonary Arterial Hypertension

Chest
Mark-Jan PloegstraRolf M F Berger

Abstract

Clinical worsening (CW), an increasingly used composite end point in adult pulmonary arterial hypertension (PAH), has not yet been evaluated in pediatric PAH. This study aims to evaluate the usefulness of CW in pediatric PAH by assessing the event incidence and prognostic value of each separate component of CW and of the composite CW end point. Seventy pediatric patients with PAH from the Dutch National Network for Pediatric Pulmonary Hypertension, who started PAH-targeted therapy between January 2000 and January 2014, were included in the study and underwent standardized follow-up. The following CW components were prospectively registered: death, lung transplantation (LTx), PAH-related hospitalizations, initiation of IV prostanoids, and functional deterioration (World Health Organization functional-class deterioration, ≥ 15% decrease in 6-min walk distance, or both). The longitudinal event incidence and prognostic value were assessed for each separate component and their combination. The end-point components of death, LTx, hospitalizations, initiation of IV prostanoids, and functional deterioration occurred with a longitudinal event rate of 10.1, 2.5, 21.4, 9.4 and 48.1 events per 100 person-years, respectively. The composite ...Continue Reading

References

Jan 30, 1992·Statistics in Medicine·L S FreedmanA Schatzkin
Aug 1, 1987·Annals of Internal Medicine·S RichS K Koerner
Oct 1, 1996·Annals of Internal Medicine·T R Fleming, D L DeMets
Mar 22, 2002·The New England Journal of Medicine·Lewis J RubinGerald Simonneau
Nov 25, 2003·American Journal of Respiratory and Critical Care Medicine·Robyn J BarstUNKNOWN STRIDE-1 Study Group
Jun 15, 2004·Journal of the American College of Cardiology·Marius M HoeperAdam Torbicki
Oct 2, 2004·American Heart Journal·Steven M Kawut, Harold I Palevsky
Nov 18, 2005·The New England Journal of Medicine·Nazzareno GalièUNKNOWN Sildenafil Use in Pulmonary Arterial Hypertension (SUPER) Study Group
Feb 4, 2006·American Journal of Respiratory and Critical Care Medicine·Marc HumbertGérald Simonneau
May 16, 2006·Journal of the American College of Cardiology·Robyn J BarstUNKNOWN STRIDE-2 Study Group
Sep 2, 2006·American Journal of Respiratory and Critical Care Medicine·Vallerie V McLaughlinLewis J Rubin
May 29, 2008·Circulation·Nazzareno GalièUNKNOWN Ambrisentan in Pulmonary Arterial Hypertension, Randomized, Double-Blind, Placebo-Controlled, Multicenter, Efficacy Studies
Jun 16, 2009·The Journal of Pediatrics·Rosa Laura E van LoonRolf M F Berger
Jul 9, 2009·Journal of the American College of Cardiology·Vallerie V McLaughlinWerner Seeger
Apr 14, 2010·Current Opinion in Pulmonary Medicine·Andrew PeacockMarc Humbert
Apr 14, 2010·Current Opinion in Pulmonary Medicine·Nazzareno GalièLewis Rubin
Apr 14, 2010·Current Opinion in Pulmonary Medicine·Sheila G Haworth, Maurice Beghetti
Jul 9, 2010·The American Journal of Cardiology·Rosa Laura E van LoonRolf M F Berger
Jun 22, 2011·Nature Reviews. Cardiology·Ralph T SchermulyFriedrich Grimminger
Nov 8, 2011·The Journal of Heart and Lung Transplantation : the Official Publication of the International Society for Heart Transplantation·Raymond L BenzaLewis J Rubin
Nov 15, 2011·Pulmonary Pharmacology & Therapeutics·Julio SandovalUNKNOWN STRIDE-4 investigators
Jun 20, 2012·Statistics in Medicine·Thomas R Fleming, John H Powers
Jul 24, 2012·Journal of the American College of Cardiology·Ronald J OudizUNKNOWN PHIRST Study Group
Sep 22, 2012·Journal of the American College of Cardiology·Gianluigi SavaresePasquale Perrone-Filardi
Oct 27, 2012·The European Respiratory Journal·Gianluigi SavaresePasquale Perrone-Filardi
Jul 26, 2013·The New England Journal of Medicine·Hossein-Ardeschir GhofraniUNKNOWN PATENT-1 Study Group
Aug 30, 2013·The New England Journal of Medicine·Tomás PulidoUNKNOWN SERAPHIN Investigators
Dec 3, 2013·European Respiratory Review : an Official Journal of the European Respiratory Society·Sean Gaine, Gérald Simonneau
Dec 21, 2013·Journal of the American College of Cardiology·Mardi Gomberg-MaitlandLewis J Rubin
Apr 1, 2014·Journal of the American College of Cardiology·Willemijn M H ZijlstraRolf M F Berger
May 13, 2014·The Lancet. Respiratory Medicine·Rolf M F Berger
Jun 22, 2014·Circulation·Corey E VentetuoloSteven M Kawut
Jul 19, 2014·The European Respiratory Journal·Mark-Jan PloegstraRolf M F Berger

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Citations

Oct 1, 2016·American Journal of Respiratory and Critical Care Medicine·Steven H AbmanUNKNOWN AHA/ATS Joint Guidelines for Pediatric Pulmonary Hypertension Committee
Jul 15, 2017·American Journal of Respiratory and Critical Care Medicine·Eric D Austin, Jeffrey A Feinstein
Apr 5, 2017·American Journal of Respiratory and Critical Care Medicine·Hong-Da ZhangZhi-Cheng Jing
Nov 6, 2018·Current Opinion in Cardiology·Carlos SisniegaTomas Pulido
Jan 8, 2019·Expert Review of Respiratory Medicine·Julie WackerMaurice Beghetti
May 23, 2018·European Heart Journal Cardiovascular Imaging·Richard M FriesenUyen Truong
Dec 14, 2018·The European Respiratory Journal·Erika B RosenzweigRolf M F Berger
Jun 8, 2017·Current Opinion in Pulmonary Medicine·Johannes M Douwes, Rolf M F Berger
Jun 10, 2017·Pulmonary Circulation·Jordan D AwerbachKevin D Hill
Mar 2, 2019·American Journal of Physiology. Heart and Circulatory Physiology·Michal SchäferUyen Truong
Aug 11, 2020·Pediatric Cardiology·Anna MigdałGrażyna Brzezińska-Rajszys
Feb 10, 2021·Pediatric Pulmonology·Ryan D ColemanPeter M Mourani
Jun 5, 2021·American Journal of Physiology. Heart and Circulatory Physiology·Michal SchäferMichael E Yeager

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