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1. Introduction
2. Disclosures
3. Outline
4. Mechanisms involved in pulmonary arterial hypertension (PAH)
5. Lung explant histology: PAH, grade 4 (Heath and Edwards)
6. Severe RV failure in IPAH
7. World Symposium on PH (2024): PH classification (5 Dx groups)
8. WSPH 2018, 2024: New hemodynamic definitions of pediatric PH
9. Quote from Lewis Rubin
10. PH in childhood: frequently asked questions by parents
11. Published review of PH in infants, children and young adults
12. Common differences in the etiologies of pediatric and adult pulmonary hypertension
13. Most common etiologies (causes) of PH in childhood
14. Heritable PAH: relative contribution of known risk genes and de novo mutations
15. Algorithm on genetic testing and counseling
16. Consensus treatment on how to diagnose and treat pediatric PH
17. Diagnostic algorithm (pediatric PH)
18. Pulmonary hypertension in infancy (groups 1+3 PH)
19. Management of PPHN
20. Pulmonary hypertension in infancy (groups 1+3 PH)
21. PH risk stratification (adults, children)
22. Risk stratification tools have been lacking for pediatric PH
23. The 2022 adult PAH 3-strata risk assessment model
24. The 2022 adult PAH simplified 4-strata risk assessment model
25. LuTx-free survival in adult PAH according to risk (4-strata risk assessment model)
26. Adult PAH treatment algorithm (WSPH 2024) (according to 4-strata risk assessment model)
27. Sotatercept: likely a game changer for PAH therapy
28. Sotatercept is an investigational, potential first-in-class activin signaling inhibitor
29. MOONBEAM: Study design overview (pediatric PAH)
30. MOONBEAM: key inclusion and exclusion criteria
31. Determinants of risk in PH
32. Risk stratification for pediatric PAH (2-strata; WSPH 2024)
33. The 2019 EPPVDN pediatric PH risk score
34. Cardiac MR predicts clinical worsening and mortality in PAH
35. Validation of risk score by CMR and echo in pediatric PAH
36. Strong correlations of the risk scores with outcome-relevant imaging variables
37. Strong correlations of the EPPVDN risk score with outcome relevant CMR imaging variables
38. The EPPVDN PH online risk score calculator
39. Advanced treatment of PAH in childhood
40. VISION of reverse-remodeling therapies in PAH / PHVD
41. PAH pharmacotherapy
42. Targeted PAH medications and their approval by regulatory agencies
43. Treatment algorithm (EPPVDN 2019)
44. Macitentan: a modified, non-selective endothelin-1 receptor antagonist
45. Safety and efficacy of add-on macitentan in pediatric PH
46. Oral and parenteral (inhal., s.c./i.v.) prostacyclin (analogue) therapy of PAH
47. Selexipag
48. Selexipag in pediatric PH: improvement in 50%, stabil. 20-25%, detoriation in 20-25%
49. PAH associated with congenital heart diseases (PAH-CHD)
50. Hemodynamics with large VSD
51. Management of PAH-CHD
52. Conclusions: PH therapy - recent developments
53. Vasodilator response in PAH - good/poor, sustained/transient
54. Oral selexipag vs. continuous parenteral PCA therapy (S.C./I.V.)
55. OMT Lenus pro subcutaneously implanted, intravenous treprostinil pump
56. 2019 updated consensus statement
57. New emerging therapies
58. Off-label, compassionate use study
59. Mechanical circulatory support in acute cardipulmonary failure and lung transplantation for End-stage PAH
60. VA-ECMO in infants with cardiopulmonary failure
61. Endstage IPAH patient
62. Pediatric lung transplantation: Hannover (GER) - survival 2
63. LuTx for pediatric PAH at Hannover Medical School
64. LuTx for pediatric PAH: demographic data
65. 9 of 15 patients were higher risk (non-invasive) at the time of LuTx
66. Peri-op management of PAH patients undergoing lung transplantation (LuTx)
67. Full recovery of RV systolic function and RV volumes 2 months after LuTx (echocardiography)
68. Recovery of RV function after pressure unloading s/p LuTx (cardiac MRI)
69. Full recovery of RV systolic function and RV volumes 2 months after LuTx (cardiac MRI) (1)
70. Full recovery of RV systolic function and RV volumes 2 months after LuTx (cardiac MRI) (1)
71. Conclusions: LuTx for pediatric PAH
72. Outcome data (international Potts shunt registry)
73. Changes and future directions of clinical research in pediatric PH
74. EPPVDN consensus statements in development 2025-2026

Topics Covered

• Pulmonary arterial hypertension (PAH)
• PH in childhood
• Etiologies of pediatric and adult pulmonary hypertension
• Diagnostic algorithm
• Risk stratification
• Determinants of risk in PH
• Pediatric PH risk score
• Pharmacotherapy
• Management of PAH

Links

Categories:

• Physiology & Anatomy

Therapeutic Areas:

• Cardiovascular & Metabolic
• Immunology & Inflammation
• Respiratory Diseases

External Links

• Slide 30- Study to Evaluate Sotatercept (MK-7962) in Children With Pulmonary Arterial Hypertension (PAH) (MK-7962-008) (MOONBEAM)
• Slide 38- PH Risk Score

Talk Citation

Hansmann, G. (2025, July 31). Pulmonary arterial hypertension in infants, children and young adults [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved August 15, 2025, from https://doi.org/10.69645/EZRF6582.
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Publication History

• Published on July 31, 2025

Financial Disclosures

• Dr. Hansmann is the Chair, Leadership Board Member and Writing Group Chair of the European Pediatric Pulmonary Disease Network. He is the Founding Chair and subsequent Councillor (2015-2021) of the Working Group “Pulmonary Hypertension, Heart Failure, and Transplantation“ (PHHFTX). He is a writing group member of the Consensus Statement on PH-LHD, AHA and Consensus Statement on Periop. PH, ISHLT. He was a Co-Chair, AHA/ATS Guidelines on Pediatric Pulmonary Hypertension. He is a Task Force Member, ESC/ERS Guideline on Pulmonary Hypertension. He has received educational grants from Janssen/J&J, Bayer, Merck/MSD, Optimed and OrphaCare. He has a a European + International Patent Application related to HUCMSC-CM treatment of chronic heart-lung and vascular diseases (EP22177263.5; PCT/EP2023/064627).