FOR IMMEDIATE RELEASE
Treatment for Children with Progressive Pulmonary Hypertension Addressed in New Clinical Practice Guideline
The American Thoracic Society Guideline is believed to be the first to tackle patient management after first line treatments fail.
NEW YORK, NY – Feb. 04, 2025 – Children with progressive pulmonary hypertension (PPH) face a significant risk of mortality and morbidity. Until now, there has been little direction for clinicians whose patients fail to improve from first-line treatments.
“Although the true incidence of PPH is unknown, more patients are being diagnosed with it today with a larger proportion of these patients having more severe disease that requires a significant amount of health care resources,” said Don Hayes, Jr. MD, ATSF, co-author of the latest Clinical Practice Guideline and professor of pediatrics and medical director of lung transplant at Cincinnati Children’s Hospital Medical Center.
In the latest issue of the American Journal of Respiratory and Critical Care Medicine, guideline authors pick up where the 2015 guideline left off addressing what clinicians should consider when initial therapy fails in this complex population.
“As the parent of a child who had end-stage pulmonary hypertension who is now beyond 10 years post double lung transplant, I am very proud to have played a role in the development of the first clinical practice guidelines for surgical options,” said Michelle F. Liu, MD, MPH, co-author of the new guidelines and a member of the Pulmonary Hypertension Association.
“We were told by one specialist that there was nothing other than medical therapy as we watched my daughter’s progressive 18-month decline after her miraculous survival of a cardiopulmonary arrest. We then (through social media) found a PH specialist who knew she was a candidate for lung transplant. This saved her life. My hope is that many young lives will be extended because of these guidelines.”
Using the thorough, evidence-based Grading of Recommendations, Assessment, Development and Evaluation or GRADE framework, the multidisciplinary panel made the following recommendations:
Recommendations (Q & A):
1. Should children with progressive PH despite optimal therapy undergo atrial septal defect (ASD) intervention (creation/enlargement)?
A. We suggest ASD-intervention for children with progressive PH and RV failure despite optimal therapy (conditional recommendation, very low certainty of evidence).
2. Should children with progressive PH despite optimal therapy undergo creation of a pulmonary-to-systemic shunt?
A. We suggest creation of a pulmonary-to-systemic shunt in children with progressive PH and suprasystemic RV pressure despite optimal therapy (conditional recommendation, very low certainty of evidence).
3. Should children with progressive PH despite optimal therapy undergo lung transplant?
A. We suggest lung transplantation in children with progressive PH and RV failure despite optimal therapy (conditional recommendation, very low certainty of evidence).
4. Should children with progressive PH despite optimal therapy on ECMO undergo bridge to lung transplant?
A. We suggest lung transplantation in children with progressive PH and RV failure despite optimal therapy who are on ECMO support without reversible cause (conditional recommendation, very low certainty of evidence).
5. Should children with progressive PH unresponsive to optimal therapy on ECMO undergo creation of a pulmonary-to-systemic shunt?
A. We suggest against pulmonary-to-systemic shunt creation for children with progressive PH and suprasystemic RV pressure despite optimal therapy who are on ECMO support without reversible cause (conditional recommendation, very low certainty of evidence).
Visit our website to read “Interventional Strategies for Children with Progressive Pulmonary Hypertension Despite Optimal Therapy: An Official American Thoracic Society Clinical Practice Guideline” in full.
About the American Thoracic Society: Founded in 1905, the American Thoracic Society is the world's leading medical society dedicated to accelerating the advancement of global respiratory health through multidisciplinary collaboration, education, and advocacy. Core activities of the Society’s more than 16,000 members are focused on leading scientific discoveries, advancing professional development, impacting global health, and transforming patient care. Key areas of member focus include developing clinical practice guidelines, hosting the annual International Conference, publishing four peer-reviewed journals, advocating for improved respiratory health globally, and developing an array of patient education and career development resources. Please visit our website to learn more.
About the American Journal of Respiratory and Critical Care Medicine (AJRCCM): The AJRCCM is a peer-reviewed journal published by the American Thoracic Society. The Journal takes pride in publishing the most innovative science and the highest quality reviews, practice guidelines and statements in pulmonary, critical care and sleep medicine. With an impact factor of 30.528, it is one of the highest ranked journals in pulmonology.