Medical Therapy. Efficacy or Lack of Efficacy

Never Say Never: The Use of Nitric Oxide in Patients With Obstructed Pulmonary Veins: A Case Report

Victoria Sokoliuk, James A. DiNardo, Morgan L. BrownBoston Children’s Hospital.United States A and A PracticeA A Pract 2019; 12: 205-207DOI: 10.1213/XAA.0000000000000885 AbstractPulmonary vein stenosis (PVS) is a progressive disease with pulmonary hypertension (PH) as a major cause of morbidity and mortality. Traditional management of PH with inhaled nitric oxide (iNO) is typically avoided in PVS patients […]

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Use of Metformin in Pulmonary Vein Stenosis after TAPVR Repair

Edward C. Kirkpatrick, Michael E. Mitchell, MD, William G. Thilly, Joseph Cava, Aoy Tomita-Mitchell, Elena V. GostjevaChildren’s Hospital of Wisconsin and Medical College of Wisconsin. Massachusetts Institute of Technology. United States Global Pediatric HealthGlob Pediatr Health 2020;DOI: 10.1177/2333794X20958924 AbstractAbstract Not Available CategoryClass II. Pulmonary Hypertension Associated with Pulmonary Vein StenosisMedical Therapy. Efficacy or Lack of EfficacyMedical Therapy. Adverse Effects or Lack of Adverse Effects Age Focus: Pediatric Pulmonary

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Bilateral disease and early age at presentation are associated with shorter survival in patients with congenital heart disease and intraluminal pulmonary vein stenosis

Sowmya Balasubramanian, Maliha Rehman, Kimberlee Gauvreau, Kathy J. JenkinsChildren’s Hospital Boston.United States Congenital Heart DiseaseCongenit Heart Dis 2012; 7: 378-386DOI: 10.1111/j.1747-0803.2012.00647.x AbstractPurpose: Pulmonary vein stenosis (PVS) is a progressive disease that is frequently lethal. We have previously identified neoproliferation of myofibroblasts as the mechanism for progressive intraluminal PVS. PVS occurs in association with other congenital heart diseases

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A prospective phase II trial of vinblastine and methotrexate in multivessel intraluminal pulmonary vein stenosis in infants and children

Maliha Rehman, Kathy J. Jenkins, Amy L. Juraszek, Jean A. Connor, Kimberlee Gauvreau, Muhammad Muneeb, Laureen M. Sena, Steven D. Colan, Terry Saia, Mark W. KieranChildren’s Hospital, Boston.United States Congenital Heart DiseaseCongenit Heart Dis 2011; 6: 608-623DOI: 10.1111/j.1747-0803.2011.00574.x AbstractObjective: To determine the safety and efficacy of the chemotherapeutic agents vinblastine and methotrexate in the treatment of children

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Simvastatin restores pulmonary endothelial function in the setting of pulmonary over-circulation

Jason T. Boehme, Xutong Sun, Qing Lu, Jubilee Barton, Xiaomin Wu, Wenhui Gong, Gary W. Raff, Sanjeev A. Datar, Ting Wang, Jeffrey R. Fineman, Stephen M. BlackUniversity of California San Francisco. Howard Wertheim College of Medicine and Florida International University. University of Arizona Health Sciences. University of California Davis. United States Nitric OxideNitric Oxide 2023;

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Comparison of treprostinil and oral sildenafil for the treatment of persistent pulmonary hypertension of the newborn: a retrospective cohort study

Enhuan Wei, Xiu-hua Chen, Si-Jia ZhouAffiliated Sanming First Hospital and Fujian Children’s Hospital (Fujian Branch of Shanghai Children’s Medical Center) of Fujian Medical University.China Frontiers in PediatricsFront Pediatr 2023; 11: DOI: 10.3389/fped.2023.1270712 AbstractBackground: This study aims to evaluate the effectiveness of treprostinil and oral sildenafil in managing persistent pulmonary hypertension of newborns (PPHN).Methods: We conducted a retrospective cohort

Comparison of treprostinil and oral sildenafil for the treatment of persistent pulmonary hypertension of the newborn: a retrospective cohort study Read More »

Effective long-term sirolimus treatment in hypoxemia mainly due to intrapulmonary right-to-left shunt in a patient with multiple vascular anomalies

Jinrong Liu, Xiaomin Duan, Jie Yin, Haiming Yang, Ruxuan He, Shunying ZhaoBeijing Children’s Hospital, National Center for Children’s Health and Capital Medical University.China Orphanet Journal of Rare DiseasesOrphanet J Rare Dis 2023; 18:DOI: 10.1186/s13023-023-02732-3 AbstractPulmonary arteriovenous malformations (PAVMs), particularly where feeding artery/arteries to PAVMs ≥ 3 mm can be treated with embolization. The treatment for hypoxemia

Effective long-term sirolimus treatment in hypoxemia mainly due to intrapulmonary right-to-left shunt in a patient with multiple vascular anomalies Read More »

Reassessing the role of milrinone in the treatment of heart failure and pulmonary hypertension in neonates and children: a systematic review and meta-analysis

Felipe Yu Matsushita, Vera Lúcia Jornada Krebs, Carolina Vieira de Campos, Paula Vieira de Vincenzi Gaiolla, Werther Brunow de CarvalhoUniversity of São Paulo. Hospital Samaritano. Brazil European Journal of PediatricsEur J Pediatr 2023; DOI: 10.1007/s00431-023-05342-0 AbstractTo evaluate milrinone’s impact on pediatric cardiac function, focusing on its specific role as an inotrope and lusitrope, while considering its

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Prostaglandin-E1 infusion in persistent pulmonary hypertension of the newborn

Stephanie M. Tsoi, Hythem Nawaytou, Hassan Almeneisi, Martina Steurer, Yili Zhao, Jeffrey R. Fineman, Roberta L. KellerUniversity of California San Francisco.United States Pediatric PulmonologyPediatr Pulmonol 2023; DOI: 10.1002/ppul.26759 AbstractBackground: Neonates with persistent pulmonary hypertension of the newborn (PPHN) can present with hypoxia and right ventricular dysfunction with resultant inadequate oxygen delivery and end-organ damage. This study describes

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Progression of vascular remodeling in pulmonary vein obstruction

Naoki Masaki, Osamu Adachi, Shintaro Katahira, Yuriko Saiki, Akira Horii, Shunsuke Kawamoto, Yoshikatsu SaikiTohoku University Graduate School of Medicine. Japan Journal of Thoracic and Cardiovascular SurgeryJ Thorac Cardiovasc Surg 2020; 160: 777-790.e5DOI: 10.1016/j.jtcvs.2020.01.098 AbstractObjectives: Pulmonary vein obstruction (PVO) frequently occurs after repair of total anomalous pulmonary vein connection with progression of intimal hyperplasia from the anastomotic site

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