Pediatric Pulmonary Vascular Disease

Assessment of Quality of Life in Children With Pulmonary Hypertension Using Parent and Self-report Questionnaires

László Ablonczy, Zita Mayer, Orsolya Somoskövi, Andrea Berkes, Orsolya Csenteri, Eva Kis, György S. ReuszGottsegen National Cardiovascular Center. University of Debrecen Clinical Center. Semmelweis University.Hungary Transplantation ProceedingsTransplant Proc 2022; 54: 2598-2602DOI: 10.1016/j.transproceed.2022.10.051 AbstractPulmonary arterial hypertension (PAH) is a progressive disease characterized by elevation of pulmonary vascular resistance and right ventricular failure. By using advanced therapies to reduce mortality, clinicians focus on improving functional status and quality of life (QOL). […]

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PATET ratio by Doppler echocardiography: noninvasive detection of pediatric pulmonary arterial hypertension

Jennifer K. Trittmann, Hanadi Almazroue, Leif D. Nelin, Terri A. Shaffer, Charanda R. Celestine, Henry W. Green, Raphael A. MalbrueOhio State University and Nationwide Children’s Hospital.United States Pediatric ResearchPediatr Res 2022; 92: 631-636DOI: 10.1038/s41390-021-01840-9 AbstractPulmonary artery acceleration time (PAT) and PAT: ejection time (PATET) ratio are echocardiographic measurements of pulmonary arterial hypertension (PAH). These noninvasive quantitative

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Patent Ductus Arteriosus and Development of Bronchopulmonary Dysplasia with Pulmonary Hypertension

Samuel J. Gentle, Colm P. Travers, Matthew Clark, Waldemar A. Carlo, Namasivayam AmbalavanamUniversity of Alabama at Birmingham.United States American Journal of Respiratory and Critical Care MedicineAm J Respir Crit Care Med 2022; DOI: 10.1164/rccm.202203-0570OC AbstractBackground: Extremely preterm infants with evolving bronchopulmonary dysplasia (BPD) are at risk for development of BPD-associated pulmonary hypertension (BPD-PH). The presence and duration

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Parenteral Prostanoids in Pediatric Pulmonary Arterial Hypertension: Start Early, Dose High, Combine

Johannes M. Douwes, Willemijn M. H. Zijlstra, Erika B. Rosenzweig, Mark-Jan Ploegstra, Usha S. Krishnan, Meindina G. Haarman, Marcus T. R. Roofthooft, Douwe Postmus, Hans L. Hillege, D. Dunbar Ivy, Rolf M. F. BergerUniversity of Groningen, University Medical Center Groningen and Beatrix Children’s Hospital. Columbia University. University of Colorado, Children’s Hospital Colorado.Netherlands and United States

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Vasoreactive phenotype in children with pulmonary arterial hypertension and syncope

Alexandra N. Linder, Jill Hsia, Sheila V. Krishnan, Erika B. Rosenzweig, Usha S. KrishnanColumbia University Irving Medical Center and New York Presbyterian Hospital. Children’s Hospital of Philadelphia, Philadelphia. Stonybrook University School of Medicine.United States European Respiratory Journal Open ResearchERJ Open Res 2022; 8: DOI: 10.1183/23120541.00223-2022 AbstractBackground: Syncope in Group 1 pulmonary arterial hypertension (PAH) is an independent

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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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