Eur Respir J

. 2024 Aug 15:2400639. https://pubmed.ncbi.nlm.nih.gov/39147412/

 doi: 10.1183/13993003.00639-2024. Online ahead of print.

An Interdisciplinary Consensus Approach to Pulmonary Hypertension in Developmental Lung Disorders

Nidhy P Varghese ¹, Eric D Austin ², Csaba Galambos ³, Mary P Mullen ⁴, Delphine Yung ⁵, R Paul Guillerman ⁶, Sara O Vargas ⁷, Catherine M Avitabile ⁸, Corey A Chartan ⁹, Nahir Cortes-Santiago ¹⁰, Michaela Ibach ¹¹, Emma O Jackson ¹², Jill Ann Jarrell ¹³, Roberta L Keller ¹⁴, Usha S Krishnan ¹⁵, Kalyani R Patel ¹⁰, Jennifer Pogoriler ¹⁶, Elise C Whalen ¹⁷, Kathryn Wikenheiser-Brokamp ¹⁸, Natalie M Villafranco ¹⁹, Steven H Abman ²⁰; Pediatric Pulmonary Hypertension Network (PPHNet)

Affiliations Expand

• PMID: 39147412
• DOI: 10.1183/13993003.00639-2024

Abstract

It is increasingly recognised that diverse genetic respiratory disorders present as severe pulmonary hypertension (PH) in the neonate and young infant, but many controversies and uncertainties persist regarding optimal strategies for diagnosis and management to maximize long-term outcomes. To better define the nature of PH in the setting of developmental lung disorders (DEVLD), in addition to the common diagnoses of bronchopulmonary dysplasia and congenital diaphragmatic hernia, we established a multidisciplinary group of expert clinicians from stakeholder paediatric specialties to highlight current challenges and recommendations for clinical approaches, as well as counselling and support of families. In this review, we characterise clinical features of infants with DEVLD/DEVLD-PH and identify decision-making challenges including genetic evaluations, the role of lung biopsies, the use of imaging modalities, and treatment approaches. The importance of working with team members from multiple disciplines, enhancing communication and providing sufficient counselling services for families is emphasized to create an interdisciplinary consensus.

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