Medical Therapy. Adverse Effects or Lack of Adverse Effects

Pulmonary Vein Stenosis Associated with Germline PIK3CA Mutation

Delphine Yung, Kaitlyn Freeman, Ghayda Mirzaa University of Washington School of Medicine and Seattle Children’s Research Institute. Brotman Baty Institute for Precision Medicine.United States ChildrenChildren 2022; 9: DOI: 10.3390/children9050671 AbstractPulmonary vein stenosis is a rare and frequently lethal childhood disease. There are few known genetic associations, and the pathophysiology is not well known. Current treatments include […]

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Severe hypertriglyceridemia associated with everolimus drug-eluting stent placement in an infant

Kuan-Chi Lai, Julie A. Brothers, Jason Z. Stoller Children’s Hospital of Philadelphia.United States Cardiology in the YoungCardiol Young 2019; 29: 541-543DOI: 10.1017/S1047951119000167 AbstractWe report a case of severe hypertriglyceridemia associated with an everolimus drug-eluting stent in an infant with pulmonary vein stenosis. We review from current literature the mechanisms by which everolimus may cause dyslipidaemia, pharmacokinetics

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New Paradigms for Pulmonary Vein Stenosis Treatment: When Surgery and Transcatheter Therapy Aren’t Good Enough

Usama B. Kanaan, William T. Mahle Children’s Healthcare of Atlanta and Emory University School of Medicine.United States Journal of PediatricsJ Pediatr 2018; 198: 12-13DOI: 10.1016/j.jpeds.2018.01.082 AbstractAbstract Not Available CategoryStenosis or Obstruction of Normal Pulmonary Venous ConnectionsStenosis or Obstruction of Pulmonary Veins Following Surgical Repair of Anomalous Pulmonary Venous ConnectionsMultidisciplinary CareMedical Therapy to Prevent or Reverse the

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Systemic Sirolimus Monitoring in Pediatric Pulmonary Vein Stenosis

Heather Meluskey, Bridget Blowey, Anna B. O’Brien, Michael L. O’Byrne, Jonathan J. Rome, David B. Frank, Catherine M. Avitabile, Mudit Gupta, Jessica Tang, Kimberly L. Butler, Constantine D. Mavroudis, Stephanie Fuller, Ryan Callahan Children’s Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania. Dell School of Medicine at the University of

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Valsartan to Prevent Acquired Pulmonary Vein Stenosis in Pediatric Patients After Total Anomalous Pulmonary Venous Connection Surgery

Jin Shentu, Mingjie Zhang, Zhuoming Xu, Chen Wen, Hao Zhang, Zhongqun Zhu, Huiwen Chen, Guocheng Shi Shanghai Children’s Medical Center and Shanghai Jiao Tong University.China Journal of the American Heart AssociationJ Am Heart Assoc 2025; 14:DOI: 10.1161/JAHA.124.036911 AbstractBackground: Recurrent pulmonary vein stenosis (PVS) following surgical repair of total anomalous pulmonary venous connection is associated with poor prognosis. Preclinical studies have shown that use of an angiotensin receptor blocker can attenuate intimal hyperplasia;

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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. Kieran Children’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

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Systemic Sirolimus Therapy for Infants and Children With Pulmonary Vein Stenosis

Jay D. Patel, Michael Briones, Mansi Mandhani, Shannon Jones, Divya Suthar, Rosemary Gray, Joelle Pettus, Courtney McCraken, Amanda Thomas, Christopher J. Petit Emory University and Children’s Healthcare of AtlantaUnited States Journal of the American College of CardiologyJ Am Coll Cardiol 2021; 77: 2807-2818DOI: 10.1016/j.jacc.2021.04.013 AbstractBackground: Anatomic interventions for pulmonary vein stenosis (PVS) in infants and children have

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Acute Pulmonary Vasodilator Testing and Long-Term Clinical Course in Segmental Pulmonary Vascular Disease

Liezl Domingo, H. Sonali Magdo, Ronald W. Day University of Utah and Primary Children’s Hospital.United States Pediatric CardiologyPediatr Cardiol 2018; 39: 501-508DOI: 10.1007/s00246-017-1780-9 AbstractResults of acute pulmonary vasodilator testing (AVT) and the outcome of medical therapy have not been described in patients with segmental pulmonary vascular disease (SPVD). We sought to compare the pulmonary vasodilatory effects

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Systemic Sirolimus to Prevent In-Stent Stenosis in Pediatric Pulmonary Vein Stenosis

Ryan Callahan, Jesse J. Esch, Grace Wang, Christina M. Ireland, Kimberlee Gauvreau, Kathy J. Jenkins Boston Children’s Hospital and Harvard Medical School.United States Pediatric CardiologyPediatr Cardiol 2020; 41: 282-289DOI: 10.1007/s00246-019-02253-6 AbstractEvaluate the efficacy of systemic sirolimus (rapamycin) in preventing in-stent stenosis (ISS) in pediatric intraluminal pulmonary vein stenosis (PVS). Report the adverse events related to sirolimus

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Adjunct Targeted Biologic Inhibition Agents to Treat Aggressive Multivessel Intraluminal Pediatric Pulmonary Vein Stenosis

Ryan Callahan, Mark W. Kieran, Christopher W. Baird, Steven D. Colan, Kimberlee Gauvreau, Chistina M. Ireland, Audrey Marshall, Laureen M. Sena, Sara O. Vargas, Kathy J. Jenkins Boston Children’s Hospital and Harvard Medical School. Floating Hospital for Children at Tufts Medical Center. UMass Memorial Medical Center.United States Journal of PediatricsJ Pediatr 2018; 198: 29-35DOI: 10.1016/j.jpeds.2018.01.029 AbstractObjective: To

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