Genetic Factors Influencing the Onset, Severity or Outcome of Disease

First locus for primary pulmonary vein stenosis maps to chromosome 2q

Ingrid van de Laar, Marja Wessels, Ingrid Frohn-Mulder, Michiel Dalinghaus, Bianca de Graaf, Marianne van Tienhoven, Paul van der Moer, Margreet Husen-Ebbinge, Maarten Lequin, Dennis Dooijes, Ronald de Krijger, Ben A. Oostra, Aida M. Bertoli-Avella Erasmus Medical Center. Medical Center Rijnmond-Zuid.Netherlands European Heart JournalEur Heart J 2009; 30: 2485–2492DOI: 10.1093/eurheartj/ehp271 AbstractAims: Primary pulmonary vein stenosis (PVS) is […]

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Preoperative pulmonary venous obstruction affects long-term outcome for survivors of total anomalous pulmonary venous connection repair

Paul M. Kirshbom, Richard J. Myung, J. William Gaynor, Richard F. Ittenbach, Stephen M. Paridon, William M. DeCampli, Tom R. Karl, Thomas L. Spray Children’s Hospital of Philadelphia.United States Annals of Thoracic SurgeryAnn thorac Surg 2002; 74: 1616-1620DOI: 10.1016/s0003-4975(02)03935-8 AbstractBackground: Early outcomes after repair of total anomalous pulmonary venous connection continue to improve; however, little information is

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Pulmonary vein stenosis in patients with Smith-Lemli-Opitz syndrome

Aaron R. Prosnitz, Jane Leopold, Mira Irons, Kathy Jenkins, Amy E. Roberts Boston Children’s Hospital. Brigham and Women’s Hospital. American Board of Medical Specialties.United States Congenital Heart DiseaseCongenit Heart Dis 2017; 12: 475-483DOI: 10.1111/chd.12471 AbstractObjective: To describe a group of children with co-incident pulmonary vein stenosis and Smith-Lemli-Opitz syndrome and to generate hypotheses as to the shared

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Pulmonary vein stenosis: Treatment and challenges

Rachel D. Vanderlaan, Jonathan Rome, Russel Hirsch, Dunbar Ivy, Christopher A. CaldaroneHospital for Sick Children. Children’s Hospital of Philadelphia. Cincinnati Children’s Hospital. Children’s Hospital Colorado. Texas Children’s Hospital and Baylor College of Medicine.Canada and United States Journal of Thoracic and Cardiovascular SurgeryJ Thorac Cardiovasc Surg 2021; 161: 2169-2176DOI: 10.1016/j.jtcvs.2020.05.117 AbstractNo Abstract CategoryStenosis or Obstruction of Normal

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Pulmonary Vein Stenosis: Incremental Knowledge Gains to Improve Outcomes

Rachel D. Vanderlaan, Christopher A. Caldarone Hospital for Sick Children. Texas Children’s Hospital.Canada and United States ChildrenChildren 2021; 8DOI: 10.3390/children8060481 AbstractPulmonary vein stenosis remains a considerable clinical challenge, with high mortality still present in children with progressive disease. In this review, we discuss the clinical spectrum of pulmonary vein stenosis and what is known about the

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Clinical Syndromic Phenotypes and the Potential Role of Genetics in Pulmonary Vein Stenosis

Abbas H. Zaidi, Jessica M. Yamada, David T. Miller, Kerry McEnaney, Christina Ireland, Amy E. Roberts, Kimberlee Gauvreau, Kathy J. Jenkins, Ming Hui Chen Boston Children’s Hospital and Harvard Medical School.United States ChildrenChildren 2021; 8DOI: 10.3390/children8020128 AbstractPulmonary vein stenosis (PVS) is a rare, frequently lethal disease with heterogeneous phenotypes and an unclear etiology. Limited studies have

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Longer Exposure to Left-to-Right Shunts Is a Risk Factor for Pulmonary Vein Stenosis in Patients with Trisomy 21

Connie Choi, Kimberlee Gauvreau, Philip Levy, Ryan Callahan, Kathy J. Jenkins, Minghui Chen Harvard Medical School and Boston Children’s Hospital.United States ChildrenChildren 2021; 8DOI: 10.3390/children8010019 AbstractWe conducted a study to determine whether patients born with Trisomy 21 and left-to-right shunts who develop pulmonary vein stenosis (PVS) have a longer exposure to shunt physiology compared to those

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Left hypoplastic lung and hemoptysis-rare familial unilateral pulmonary vein atresia

Ronly Har-Even Cohn, Matthew Hicks, Atilano Lacson, Anne Hicks University of Alberta.Canada Clinical Case ReportsClin Case Rep 2020; 8: 1698-1703DOI: 10.1002/ccr3.2982 AbstractUnilateral pulmonary vein atresia (UPVA) is a rare congenital vascular malformation with obliteration of the pulmonary vein. We present a case series of three siblings with variable presentation of UPVA. We suggest a dominant genetic cause based on different

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Repair of Total Anomalous Pulmonary Venous Connection: Risk Factors for Postoperative Obstruction

Brian R. White, Deborah Y. Ho, Jennifer A. Faerber, Hannah Katcoff, Andrew C. Glatz, Christopher E. Mascio, Paul Stephens Jr., Meryl S. Cohen Children’s Hospital of Philadelphia.United States Annals of Thoracic SurgeryAnn Thorac Surg 2019; 108: 122-129DOI: 10.1016/j.athoracsur.2019.02.017 AbstractBackground: Pulmonary venous obstruction after repair of total anomalous pulmonary venous connection (TAPVC) results in substantial morbidity and mortality.

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Progressive pulmonary vein stenosis in Down syndrome infant: a rare cause of pulmonary hypertension

Norazah Zahari, Joyce Darshinee Dom Sirisani University of Malaya.Malaysia Medical Journal of MalaysiaMed J Malaysia 2018; 73: 119-120DOI: Not available AbstractPulmonary veins stenosis in a Down Syndrome infant with normal connection pulmonary vein is rare and precise incidence of this disease is unknown. We report a case of progressive multiple pulmonary vein stenosis in a

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