Embracing the challenges of neonatal and paediatric pulmonary hypertension.

Autor:	Ivy D; Pediatric Cardiology, University of Colorado School of Medicine, and Children's Hospital Colorado, Aurora, CO, USA., Rosenzweig EB; Department of Pediatrics, Maria Fareri Children's Hospital at WMC Health and New York Medical College of Touro University, Valhalla, NY, USA Erika.BermanRosenzweig@wmchealth.org., Abman SH; Department of Pediatrics, University of Colorado School of Medicine, and Children's Hospital Colorado, Aurora, CO, USA., Beghetti M; Paediatric Cardiology Unit, Department of Paediatrics, Gynecology and Obstetrics, Geneva University Hospital, University of Geneva, Geneva, Switzerland., Bonnet D; Centre de Référence Malformations Cardiaques Congénitales Complexes, M3C, Necker Hospital for Sick Children, Assistance Publique des Hôpitaux de Paris, Paediatric Cardiology, Paris, France., Douwes JM; Center for Congenital Heart Diseases, Paediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands., Manes A; Cardiology Unit IRCCS, S. Orsola University Hospital, Bologna, Italy., Berger RMF; Center for Congenital Heart Diseases, Paediatric and Congenital Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Jazyk:	angličtina
Zdroj:	The European respiratory journal [Eur Respir J] 2024 Oct 31; Vol. 64 (4). Date of Electronic Publication: 2024 Oct 31 (Print Publication: 2024).
DOI:	10.1183/13993003.01345-2024
Abstrakt:	Paediatric pulmonary arterial hypertension (PAH) shares common features with adult disease, but is associated with several additional disorders and challenges that require unique approaches. This article discusses recent advances, ongoing challenges and distinct approaches for caring for infants and children with PAH, as presented by the paediatric task force of the 7th World Symposium on Pulmonary Hypertension. We provide updates on diagnosing, classifying, risk-stratifying and treating paediatric pulmonary hypertension (PH) and identify critical knowledge gaps. An updated risk stratification tool and treatment algorithm is provided, now also including strategies for patients with associated cardiopulmonary conditions. Treatment of paediatric PH continues to be hindered by the lack of randomised controlled clinical trials. The challenging management of children failing targeted PAH therapy is discussed, including balloon atrial septostomy, lung transplantation and pulmonary-to-systemic shunt (Potts). A novel strategy using a multimodal approach for the management of PAH associated with congenital heart diseases with borderline pulmonary vascular resistance is included. Advances in diagnosing neonatal PH, especially signs and interpretation of PH by echocardiography, are highlighted. A team approach to the rapidly changing physiology of neonatal PH is emphasised. Challenges in drug approval are discussed, particularly the challenges of designing accurate paediatric clinical trials with age-appropriate end-points and adequate enrolment. Competing Interests: Conflict of interest: D. Ivy reports grants from the National Institutes of Health, GSK and Janssen, consultancy fees to the University of Colorado and support for attending meetings from Bayer, Merck and Janssen, and a leadership role with the Association of Pediatric Pulmonary Hypertension. E.B. Rosenzweig reports grants from National Institutes of Health, Bayer, Janssen, Insmed and SonVie, and is Director, PPHNet and Board Member, Team Phenomenal Hope. S.H. Abman reports consultancy fees from Chiasi and Oak Hills Bio, and leadership roles with BPD Collaborative and Pediatric Pulmonary Hypertension Network. M. Beghetti reports consultancy fees from Actelion/Janssen, MSD, Merck, Gossamer, GSK and Orpha, payment or honoraria for lectures, presentations, manuscript writing or educational events and support for attending meetings from Actelion/Janssen and MSD, and participation on a data safety monitoring board or advisory board with GSK, Actelion/Janssen, Gossamer, Altavant and MSD. D. Bonnet reports consultancy fees from Janssen, MSD and Novartis, and participation on a data safety monitoring board or advisory board with Lupin. J.M. Douwes has no potential conflicts of interest to disclose. A. Manes reports grants from Janssen/Actelion and Merck, payment or honoraria for lectures, presentations, manuscript writing or educational events from Janssen/Actelion, support for attending meetings from Dompè, and participation on a data safety monitoring board or advisory board with AOP Health Italy. R.M.F. Berger reports grants from Johnson & Johnson, consultancy fees from Johnson & Johnson, GSK and Ferrer, payment for educational events from Johnson & Johnson, Ferrer, AOP, MSD, Heart Medical, Occlutech Salveo, Bayer and Gossamerbio, participation on a data safety monitoring board or advisory board with MSD, and leadership roles with TOPP-registry, PPHNet and the ESC/ERS 2022 guidelines for diagnosis and treatment of pulmonary hypertension task force. (Copyright ©The authors 2024.)
Databáze:	MEDLINE
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