Steven H Abman1, D Dunbar Ivy. 1. Pediatric Heart Lung Center, Department of Pediatrics, University of Colorado School of Medicine and Children's Hospital, Aurora, Colorado, USA.
Abstract
PURPOSE OF REVIEW: Pulmonary artery hypertension (PAH) in children contributes significantly to morbidity and mortality in diverse pediatric cardiac, lung, hematologic and other diseases. Advances in pulmonary vascular biology over the past few decades have significantly expanded therapeutic strategies; however, many unique issues persist regarding our understanding of pediatric PAH. RECENT FINDINGS: Recent studies of pediatric PAH include those that highlight gaps in our understanding of pediatric diseases associated with PAH from those of adult onset, emphasizing the strong need for specific studies regarding unique aspects of the pathogenesis and treatment of children with PAH. Registries have begun to provide new data showing differences in physiology, course, and genetics between adult and pediatric forms of PAH. Unfortunately, therapeutic strategies in pediatric pulmonary hypertension are often limited to small observational studies in children and are dependent on results from larger adult studies. In addition, clinical endpoints for studies and care remain poorly defined in infants and children. SUMMARY: Despite many advances, long-term outcomes for children with PAH remain guarded and substantial challenges persist, especially with regard to understanding mechanisms and approach to severe PAH. Future studies are needed to develop novel biomarkers, clinical endpoints and interventions for young children with diverse causes of PAH.
PURPOSE OF REVIEW: Pulmonary artery hypertension (PAH) in children contributes significantly to morbidity and mortality in diverse pediatric cardiac, lung, hematologic and other diseases. Advances in pulmonary vascular biology over the past few decades have significantly expanded therapeutic strategies; however, many unique issues persist regarding our understanding of pediatric PAH. RECENT FINDINGS: Recent studies of pediatric PAH include those that highlight gaps in our understanding of pediatric diseases associated with PAH from those of adult onset, emphasizing the strong need for specific studies regarding unique aspects of the pathogenesis and treatment of children with PAH. Registries have begun to provide new data showing differences in physiology, course, and genetics between adult and pediatric forms of PAH. Unfortunately, therapeutic strategies in pediatric pulmonary hypertension are often limited to small observational studies in children and are dependent on results from larger adult studies. In addition, clinical endpoints for studies and care remain poorly defined in infants and children. SUMMARY: Despite many advances, long-term outcomes for children with PAH remain guarded and substantial challenges persist, especially with regard to understanding mechanisms and approach to severe PAH. Future studies are needed to develop novel biomarkers, clinical endpoints and interventions for young children with diverse causes of PAH.
Authors: Robyn J Barst; Kamal K Mubarak; Roberto F Machado; Kenneth I Ataga; Raymond L Benza; Oswaldo Castro; Robert Naeije; Namita Sood; Paul S Swerdlow; Mariana Hildesheim; Mark T Gladwin Journal: Br J Haematol Date: 2010-02-17 Impact factor: 6.998
Authors: Gérald Simonneau; Ivan M Robbins; Maurice Beghetti; Richard N Channick; Marion Delcroix; Christopher P Denton; C Gregory Elliott; Sean P Gaine; Mark T Gladwin; Zhi-Cheng Jing; Michael J Krowka; David Langleben; Norifumi Nakanishi; Rogério Souza Journal: J Am Coll Cardiol Date: 2009-06-30 Impact factor: 24.094
Authors: M Jakkula; T D Le Cras; S Gebb; K P Hirth; R M Tuder; N F Voelkel; S H Abman Journal: Am J Physiol Lung Cell Mol Physiol Date: 2000-09 Impact factor: 5.464
Authors: Robin H Steinhorn; John P Kinsella; Christine Pierce; Ghazwan Butrous; Maria Dilleen; Michael Oakes; David L Wessel Journal: J Pediatr Date: 2009-12 Impact factor: 4.406
Authors: Benjamin Sztrymf; Florence Coulet; Barbara Girerd; Azzedine Yaici; Xavier Jais; Olivier Sitbon; David Montani; Rogério Souza; Gerald Simonneau; Florent Soubrier; Marc Humbert Journal: Am J Respir Crit Care Med Date: 2008-03-20 Impact factor: 21.405
Authors: D Dunbar Ivy; Aimee K Doran; Kelly J Smith; George B Mallory; Maurice Beghetti; Robyn J Barst; Daniela Brady; Yuk Law; Donna Parker; Lori Claussen; Steven H Abman Journal: J Am Coll Cardiol Date: 2008-01-15 Impact factor: 24.094
Authors: Ekkehard Grünig; Rolf Koehler; Gabriel Miltenberger-Miltenyi; Rainer Zimmermann; Matthias Gorenflo; Derliz Mereles; Karlin Arnold; Barbara Naust; Heinrike Wilkens; Andreas Benz; Albrecht von Hippel; Herbert E Ulmer; Wolfgang Kübler; Hugo A Katus; Claus R Bartram; Dietmar Schranz; Bart Janssen Journal: Pediatr Res Date: 2004-08-04 Impact factor: 3.756
Authors: Joseph M Collaco; Lewis H Romer; Bridget D Stuart; John D Coulson; Allen D Everett; Edward E Lawson; Joel I Brenner; Anna T Brown; Melanie K Nies; Priya Sekar; Lawrence M Nogee; Sharon A McGrath-Morrow Journal: Pediatr Pulmonol Date: 2012-07-06
Authors: David B Frank; Matthew A Crystal; David L S Morales; Ken Gerald; Brian D Hanna; George B Mallory; Joseph W Rossano Journal: Pulm Circ Date: 2015-06 Impact factor: 3.017
Authors: Antoinette T Burns; Shana L Hansen; Zachary S Turner; James K Aden; Alexander B Black; Daniel P Hsu Journal: J Clin Sleep Med Date: 2019-08-15 Impact factor: 4.062