Summary for Clinicians: Clinical Practice Guideline on Interventional Strategies for Children with Progressive Pulmonary Hypertension Despite Optimal Therapy

This guideline provides evidence-based recommendations for managing pediatric patients with progressive pulmonary hypertension despite optimal medical therapy. Key interventions systematically reviewed include atrial septal defect (ASD) intervention/atrial septostomy (creation and/or enlargement), pulmonary-to-systemic shunt (reverse Pott’s shunt) creation, lung transplantation, and the use of extracorporeal membrane oxygenation (ECMO) support as bridge to other treatments (Table 1). These recommendations aim to guide clinicians managing this fragile patient population, with the strength of recommendation and evidence quality specifically annotated (Table 2). Also of note, these recommendations are focused primarily on patients with progressive pulmonary arterial hypertension despite the use of multi-pathway pharmacotherapy; under certain circumstances, however, these recommendations may also apply to children with other types of progressive pulmonary hypertension, as discussed. As the literature discussing these interventions is only continuing to grow, clinicians are encouraged to consider individual patient circumstances. This summary is intended to provide practicing physicians with key points from the guideline.

This content is free for users with an active ATS Membership.

Target Audience

Pulmonologists, critical care specialists, translational researchers, and clinicians

Learning Objectives

At the conclusion of this activity, learners should be able to:

  • Describe new strategies to manage pediatric patients with progressive pulmonary hypertension
  • Identify patients with progressive right ventricular dysfunction who require early referral for surgical or catheter-based interventions and/or lung transplantation
  • Describe the most recent studies and recommendations regarding atrial septal defect (ASD) intervention, pulmonary-to-systemic shunt creation, and the roles of ECMO and lung transplantation for pediatric pulmonary hypertension
Course summary
Available credit: 
  • 1.00 AMA PRA Category 1 Credit(s)™
    The American Thoracic Society designates this for a maximum of 1.00 AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.
  • 1.00 Participation
Publication Date: 
05/30/2025
Credit Expires: 
05/01/2027
Cost:
$25.00
Rating: 
0

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Accreditation Statement

The American Thoracic Society is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.

Disclosure Declaration

Article Authorship Disclosures (as submitted to the ATS prior to article publication date)

Ryan D. Coleman, M.D. (Division of Critical Care and Division of Pulmonary Medicine, Baylor College of Medicine, Houston, TX) reported payments for testimony by Hassard Bonnington LLP, made to Baylor College of Medicine. 

David L.S. Morales, M.D. (Division of Cardiothoracic Surgery, Department of Surgery, University of Cincinnati College of Medicine, Cincinnati, OH) reported consulting fees from Berlin Inc and Abbott Inc. 

Sarah P. Cohen, M.D. (Division of Pulmonary, Sleep Medicine and Cystic Fibrosis, Department of Pediatrics, Ohio State University Wexner Medical Center, Columbus, Ohio), reported no relevant financial relationships with ineligible companies.  

Pirooz Eghtesady, M.D., Ph.D. (Section of Pediatric Cardiothoracic Surgery, Mary Culver Department of Surgery, Washington University School of Medicine, St Louis, MO) reported no relevant financial relationships with ineligible companies.  

R. Mark Grady, M.D. (Division of Pediatric Cardiology, Department of Pediatrics, Washington University School of Medicine, St Louis, MO) reported no relevant financial relationships with ineligible companies.  

Don Hayes, Jr, M.D. (Division of Pulmonary Medicine, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH) reported no relevant financial relationships with ineligible companies.  

Joseph K. Ruminjo, M.D. (American Thoracic Society, New York, New York) reported no relevant financial relationships with ineligible companies.  

W. Graham Carlos, M.D. (Division of Pulmonary, Critical Care, Sleep and Occupational Medicine, Indiana University School of Medicine, Indianapolis, IN) reported no relevant financial relationships with ineligible companies. 

Off-Label Usage Disclosure

None

Disclosures of AnnalsATS CME Planners

The Annals of the American Thoracic Society (AnnalsATS) publishes original research, commentaries, reviews, and educational content of interest to clinicians and clinical investigators in pediatric and adult pulmonary and sleep medicine and medical critical care. The scope of the journal encompasses content that is applicable to clinical practice, the formative and continuing education of clinical specialists, and the advancement of public health.

The publication of articles that meet these goals by itself is only one step in a multi-step process for the translation of evidence-based improvements in are to clinical practice. Testing for CME credit is designed to function as a next step in the process. This is accomplished through a series of questions written by the author(s) to test that readers have the tools needed to translate recommendations for diagnostic and therapeutic clinical care into clinical practice. Members of the AnnalsATS editorial board review these questions and edit these questions for clarity, educational content, and the quality of the evidence supporting the response to the question.

AnnalsATS CME Planners

Margaret M. Hayes, M.D.
Harvard Medical School, Boston MA, USA
Dr. Hayes reported receiving payments as an author for a chapter on heliox for UpToDate. 

Caroline Okorie, M.D., M.P.H.
Stanford University School of Medicine, Stanford CA, USA
Dr. Okorie reported no financial relationships with ineligible companies.

All relevant financial relationships have been reviewed and mitigated.

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Available Credit

  • 1.00 AMA PRA Category 1 Credit(s)™
    The American Thoracic Society designates this for a maximum of 1.00 AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.
  • 1.00 Participation

Price

Cost:
$25.00
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