Catherine M Avitabile1,2,3, Xuemei Zhang4, Steve B Ampah4, Yan Wang5,6, Devon Ash5,6, Kathleen Nilan7, Laura Mercer-Rosa5,6,8, Julie L Fierro8,9, David B Frank5,8, Kathleen A Gibbs8,7. 1. Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA. avitabilec@chop.edu. 2. Echocardiography Laboratory Research Unit, The Children's Hospital of Philadelphia, Philadelphia, PA, USA. avitabilec@chop.edu. 3. Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA. avitabilec@chop.edu. 4. Data Science and Biostatistics Unit, Department of Biomedical and Health Informatics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA. 5. Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA. 6. Echocardiography Laboratory Research Unit, The Children's Hospital of Philadelphia, Philadelphia, PA, USA. 7. Division of Neonatology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA. 8. Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA. 9. Division of Pulmonary and Sleep Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Abstract
OBJECTIVE: To evaluate factors associated with discontinuation of pulmonary vasodilator therapy in bronchopulmonary dysplasia-related pulmonary hypertension (BPD-PH). STUDY DESIGN: Retrospective study of neonatal, echocardiographic, and cardiac catheterization data in 121 infants with BPD-PH discharged on pulmonary vasodilator therapy from 2009-2020 and followed into childhood. RESULT: After median 4.4 years, medications were discontinued in 58%. Those in whom medications were discontinued had fewer days of invasive support, less severe BPD, lower incidence of PDA closure or cardiac catheterization, and higher incidence of fundoplication or tracheostomy decannulation (p < 0.05). On multivariable analysis, likelihood of medication discontinuation was lower with longer period of invasive respiratory support [HR 0.95 (CI:0.91-0.99), p = 0.01] and worse RV dilation on pre-discharge echocardiogram [HR 0.13 (CI:0.03-0.70), p = 0.017]. In those with tracheostomy, likelihood of medication discontinuation was higher with decannulation [HR 10.78 (CI:1.98-58.59), p < 0.001]. CONCLUSION: In BPD-PH, childhood discontinuation of pulmonary vasodilator therapy is associated with markers of disease severity.
OBJECTIVE: To evaluate factors associated with discontinuation of pulmonary vasodilator therapy in bronchopulmonary dysplasia-related pulmonary hypertension (BPD-PH). STUDY DESIGN: Retrospective study of neonatal, echocardiographic, and cardiac catheterization data in 121 infants with BPD-PH discharged on pulmonary vasodilator therapy from 2009-2020 and followed into childhood. RESULT: After median 4.4 years, medications were discontinued in 58%. Those in whom medications were discontinued had fewer days of invasive support, less severe BPD, lower incidence of PDA closure or cardiac catheterization, and higher incidence of fundoplication or tracheostomy decannulation (p < 0.05). On multivariable analysis, likelihood of medication discontinuation was lower with longer period of invasive respiratory support [HR 0.95 (CI:0.91-0.99), p = 0.01] and worse RV dilation on pre-discharge echocardiogram [HR 0.13 (CI:0.03-0.70), p = 0.017]. In those with tracheostomy, likelihood of medication discontinuation was higher with decannulation [HR 10.78 (CI:1.98-58.59), p < 0.001]. CONCLUSION: In BPD-PH, childhood discontinuation of pulmonary vasodilator therapy is associated with markers of disease severity.
Authors: Bernard Thébaud; Kara N Goss; Matthew Laughon; Jeffrey A Whitsett; Steven H Abman; Robin H Steinhorn; Judy L Aschner; Peter G Davis; Sharon A McGrath-Morrow; Roger F Soll; Alan H Jobe Journal: Nat Rev Dis Primers Date: 2019-11-14 Impact factor: 52.329
Authors: Shannon E G Hamrick; Hannes Sallmon; Allison T Rose; Diego Porras; Elaine L Shelton; Jeff Reese; Georg Hansmann Journal: Pediatrics Date: 2020-11 Impact factor: 7.124
Authors: Tim Hundscheid; Wes Onland; Bart van Overmeire; Peter Dijk; Anton H L C van Kaam; Koen P Dijkman; Elisabeth M W Kooi; Eduardo Villamor; André A Kroon; Remco Visser; Daniel C Vijlbrief; Susanne M de Tollenaer; Filip Cools; David van Laere; Anne-Britt Johansson; Catheline Hocq; Alexandra Zecic; Eddy Adang; Rogier Donders; Willem de Vries; Arno F J van Heijst; Willem P de Boode Journal: BMC Pediatr Date: 2018-08-04 Impact factor: 2.125