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Medical and Invasive Management of Congenital and Acquired Pulmonary Vein Stenosis

  • Cardiology/CT Surgery (K Gist, Section Editor)
  • Published:
Current Treatment Options in Pediatrics Aims and scope Submit manuscript

Abstract

Purpose of review

Pulmonary vein stenosis (PVS) is an increasingly recognized disease in infants and young children. Controversy exists regarding the best management of this disease. The hallmarks of PVS include recurrence, upstream progression, and spread to previously unaffected pulmonary veins. This review highlights invasive and medical therapies for pediatric patients with primary and secondary PVS.

Recent findings

Catheter-based therapies for PVS are the mainstay of anatomic, large-vessel therapy. Angioplasty and stenting both have a role in the anatomic management of PVS. Repeated transcatheter interventions have been shown to improve survival. Surgical PVS intervention is an important element to PVS therapy. Primary (medical) therapy is increasingly recognized as an important element to PVS treatment. New anti-proliferative therapies have proven to be a useful adjunct to invasive therapies.

Summary

Congenital and acquired PVS are progressive diseases that pose a growing challenge for cardiologists. Surgical and transcatheter interventions in most cases have early success but are only temporizing solutions in the face of myofibroblastic proliferation. More recent studies focusing on benefits of immunomodulatory therapy have paved the way for the use of primary treatment alongside the anatomic therapy.

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Authors and Affiliations

  1. Department of Pediatrics, Division of Cardiology, Emory University and Children’s Healthcare of Atlanta, 2835 Brandywine Rd Suite 300, Atlanta, GA, 30341, USA

    Jay D. Patel MD, Michael Briones DO, Shannon Jones MD, Divya Suthar MD, Rosemary Gray RN & Christopher J. Petit MD

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  1. Jay D. Patel MD
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  2. Michael Briones DO
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  3. Shannon Jones MD
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  4. Divya Suthar MD
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  6. Christopher J. Petit MD
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Correspondence to Christopher J. Petit MD.

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Patel, J.D., Briones, M., Jones, S. et al. Medical and Invasive Management of Congenital and Acquired Pulmonary Vein Stenosis. Curr Treat Options Peds 6, 170–181 (2020). https://doi.org/10.1007/s40746-020-00198-0

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  • DOI: https://doi.org/10.1007/s40746-020-00198-0

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