Pediatric Pulmonary Vascular Disease

Long-term prognostic value of cardiac catheterization and acute vasodilator testing with inhaled iloprost in pediatric idiopathic pulmonary arterial hypertension

Chen Zhang, Konstantinos Dimopoulos, Qiangqiang Li, Hong GuBeijing Anzhen Hospital, Capital Medical University. Royal Brompton Hospital.China and United Kingdom Pulmonary Circulation Pulm Circ 2022; 12: DOI: 10.1002/pul2.12169 AbstractTo assess the long-term prognostic value of cardiac catheterization and acute vasodilator testing (AVT) with inhaled iloprost in children with idiopathic pulmonary arterial hypertension (IPAH). Data on 81 consecutive […]

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Abnormal platelet aggregation in pediatric pulmonary hypertension

Stephanie S. Handler, Jing Jin, Michelle T. Ogawa, Jeffrey A. Feinstein, Clara LoMedical College of Wisconsin. Stanford University.United States Pulmonary CirculationPulm Circ 2022; 12: DOI: 10.1002/pul2.12104 AbstractEndogenous prostacyclin stimulates pulmonary vasodilation and inhibits platelet aggregation. For the synthetic analog treprostinil, used in the treatment of pulmonary hypertension (PH), conflicting, anecdotal evidence exists regarding its effects on

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Surgical repair of peripheral pulmonary artery stenosis: A 2-decade experience with 145 patients

L. Mac Felmly, Richard D. Mainwaring, R. Thomas Collins, Kirstie Lechich, Elisabeth Martin, Michael Ma, Frank L. HanleyStanford University and Lucile Packard Children’s Hospital.United States Journal of Thoracic and Cardiovascular SurgeryJ Thorac Cardiovasc Surg 2022; DOI: 10.1016/j.jtcvs.2022.07.037 AbstractBackground: Peripheral pulmonary artery stenosis (PPAS) is a relatively rare form of congenital heart disease often associated with Williams syndrome,

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Transcatheter closure of congenital portosystemic shunts – A multicenter experience

Nageswara Rao Koneti, Shweta Bakhru, Mahimarangaiah Jayranganath, Mahesh Kappanayil, Prashant Bobhate, Lakshmivenkateshiah Srinivas, Snehal Kulkarni, Usha Mandikal Kodandarama Sastry, Raman Krishna KumarRainbow Children’s Heart Institute. Sri Jayadeva Institute of Cardiovascular Sciences and Research. Amrita Institute of Medical Sciences. Kokilaben Ambani Hospital. Jupiter Hospital.India Annals of Pediatric CardiologyAnn Pediatr Cardiol 2022; 15: 114-120DOI: 10.4103/apc.apc_5_22 AbstractBackground: Congenital portosystemic shunts

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A rare homozygous missense GDF2 (BMP9) mutation causing PAH in siblings: Does BMP10 status contribute?

Paul Upton, Susan Richards, Angela Bates, Karen Y. Niederhoffer, Nicolas W. Morrell, Susan ChristianUniversity of Cambridge. Stollery Children’s Hospital. University of Alberta.United Kingdom and Canada American Journal of Medical Genetics Part AAm J Med Genet A 2023; 191: 228-233DOI: 10.1002/ajmg.a.62996 AbstractPulmonary arterial hypertension (PAH) is a disease characterized by pathological remodeling of the pulmonary vasculature causing

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Inpatient Transition From Intravenous to Inhaled Treprostinil in a Pediatric Patient

David Procaccini, Dennis Delany, Abigail Self, Patricia Lawrence Kane, John D. CoulsonJohns Hopkins HospitalUnited States Journal of Pediatric Pharmacology and TherapeuticsJ Pediatr Pharmacol Ther 2023; 28: 102-107DOI: 10.5863/1551-6776-28.1.102 AbstractWe report a case of a 7-year old male with idiopathic pulmonary arterial hypertension, successfully transitioned from an intravenous infusion to inhaled treprostinil during inpatient admission, after his

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Innovations in Pediatric Therapeutics Development: Principles for the Use of Bridging Biomarkers in Pediatric Extrapolation

Thomas R. Fleming, Christine E. Garnett, Laurie S. Conklin, Solange Corriol‑Rohou, Sudharshan Hariharan, Daphne Hsu, Guenther Mueller‑Velten, Yeruk Mulugeta, Ronald Portman, Mark D. Rothmann, Norman L. Stockbridge, Simon Wandel, Jialu Zhang, Lynne YaoUniversity of Washington. U.S. Food and Drug Administration. Janssen Research and Development. AstraZeneca. Children’s Hospital at Montefiore and Albert Einstein College of Medicine. Novartis Pharma AG and Novartis Pharmaceuticals.United States Therapeutic Innovation and Regulatory ScienceTher Innov Regul Sci 2023; 57: 109-120DOI: 10.1007/s43441-022-00445-6 AbstractEven with recent substantive improvements in health care in pediatric populations, considerable need remains

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Pulmonary Vein Atresia: Feasibility of Initial Recanalization Attempts with Subsequent Follow-up

Varun Aggarwal, Gary E. Stapleton, Lindsay F. Eilers, Srinath Gowda, Manish Bansal, Athar M. Qureshi, Melissa K. Webb, Asra Khan, Henri JustinoBaylor College of Medicine and Texas Children’s HospitalUnited States Current Problems in CardiologyCurr Probl Cardiol 2023; 48: DOI: 10.1016/j.cpcardiol.2022.101463 AbstractPulmonary vein atresia (PVA) may lead to pulmonary hypertension, cardiac failure, and death. Transcatheter or surgical treatments have rarely been offered to this population because of perceived poor outcomes. We describe single center

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NT-proBNP and Zlog-transformed NT-proBNP values predict extubation failure in critically ill neonates with pulmonary hypertension and ventricular dysfunction

Lukas Schroeder, Manuel Kuelshammer, Ramona Dolsheid-Pommerich, Stefan Holdenrieder, Andreas Mueller, Florian KipfmuellerUniversity Children’s Hospital Bonn. University Hospital Bonn. Technical University Munich.Germany Pediatric PulmonologyPediatr Pulmonol 2023; 58: 253-261DOI: 10.1002/ppul.26193 AbstractObjectives: Critically ill neonates with a history of pulmonary hypertension (PH) or ventricular dysfunction are at risk to experience an extubation failure (EF) after liberation from mechanical ventilation (MV).

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Ambrisentan for in pediatric pulmonary arterial hypertension: a cost-utility analysis

Jefferson Antonio Buendia, Diana Guerrero Patino, Erika Fernanda LindarteUniversity of Antioquia.Columbia Pediatric PulmonologyPediatr Pulmonol 2023; DOI: 10.1002/ppul.26361 AbstractIntroduction: Despite the growing evidence of efficacy, little is known regarding the efficiency of ambrisentan to decrease cost and improve the functional classes of pediatric patients with Pulmonary arterial hypertension. This study aims to determine the cost-utility of ambrisentan regarding

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