Younger age at initiation of subcutaneous treprostinil is associated with better response in pediatric Group 1 pulmonary arterial hypertension
Abstract Children with severe Group 1 pulmonary arterial hypertension (PAH) have an unpredictable response to subcutaneous treprostinil (TRE) therapy, which may be influenced by age, disease severity, or other unknown variables at time of initiation. In this retrospective single‐center cohort study,...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2024-01-01
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| Series: | Pulmonary Circulation |
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| Online Access: | https://doi.org/10.1002/pul2.12328 |
| _version_ | 1797236729910919168 |
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| author | Justin J. Kochanski Jeffrey A. Feinstein Michelle Ogawa Victor Ritter Rachel K. Hopper Gregory T. Adamson |
| author_facet | Justin J. Kochanski Jeffrey A. Feinstein Michelle Ogawa Victor Ritter Rachel K. Hopper Gregory T. Adamson |
| author_sort | Justin J. Kochanski |
| collection | DOAJ |
| description | Abstract Children with severe Group 1 pulmonary arterial hypertension (PAH) have an unpredictable response to subcutaneous treprostinil (TRE) therapy, which may be influenced by age, disease severity, or other unknown variables at time of initiation. In this retrospective single‐center cohort study, we hypothesized that younger age at TRE initiation, early hemodynamic response (a decrease in pulmonary vascular resistance by ≥30% at follow‐up catheterization), and less severe baseline hemodynamics (Rp:Rs < 1.1) would each be associated with better clinical outcomes. In 40 pediatric patients with Group I PAH aged 17 days−18 years treated with subcutaneous TRE, younger age (cut‐off of 6‐years of age, AUC 0.824) at TRE initiation was associated with superior 5‐year freedom from adverse events (94% vs. 39%, p = 0.002), better WHO functional class (I or II: 88% vs. 39% p = 0.003), and better echocardiographic indices of right ventricular function at most recent follow‐up. Neither early hemodynamic response nor less severe baseline hemodynamics were associated with better outcomes. Patients who did not have a significant early hemodynamic response to TRE by first follow‐up catheterization were unlikely to show subsequent improvement in PVRi (1/8, 13%). These findings may help clinicians counsel families and guide clinical decision making regarding the timing of advanced therapies. |
| first_indexed | 2024-04-24T17:08:29Z |
| format | Article |
| id | doaj.art-86ca26691929454ca984becaacd82963 |
| institution | Directory Open Access Journal |
| issn | 2045-8940 |
| language | English |
| last_indexed | 2024-04-24T17:08:29Z |
| publishDate | 2024-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Pulmonary Circulation |
| spelling | doaj.art-86ca26691929454ca984becaacd829632024-03-28T19:28:30ZengWileyPulmonary Circulation2045-89402024-01-01141n/an/a10.1002/pul2.12328Younger age at initiation of subcutaneous treprostinil is associated with better response in pediatric Group 1 pulmonary arterial hypertensionJustin J. Kochanski0Jeffrey A. Feinstein1Michelle Ogawa2Victor Ritter3Rachel K. Hopper4Gregory T. Adamson5Department of Pediatrics (Cardiology) Stanford University School of Medicine Palo Alto California USADepartment of Pediatrics (Cardiology) Stanford University School of Medicine Palo Alto California USADepartment of Pediatrics (Cardiology) Stanford University School of Medicine Palo Alto California USAStanford University School of Medicine Quantitative Sciences Unit Palo Alto California USADepartment of Pediatrics (Cardiology) Stanford University School of Medicine Palo Alto California USADepartment of Pediatrics (Cardiology) Stanford University School of Medicine Palo Alto California USAAbstract Children with severe Group 1 pulmonary arterial hypertension (PAH) have an unpredictable response to subcutaneous treprostinil (TRE) therapy, which may be influenced by age, disease severity, or other unknown variables at time of initiation. In this retrospective single‐center cohort study, we hypothesized that younger age at TRE initiation, early hemodynamic response (a decrease in pulmonary vascular resistance by ≥30% at follow‐up catheterization), and less severe baseline hemodynamics (Rp:Rs < 1.1) would each be associated with better clinical outcomes. In 40 pediatric patients with Group I PAH aged 17 days−18 years treated with subcutaneous TRE, younger age (cut‐off of 6‐years of age, AUC 0.824) at TRE initiation was associated with superior 5‐year freedom from adverse events (94% vs. 39%, p = 0.002), better WHO functional class (I or II: 88% vs. 39% p = 0.003), and better echocardiographic indices of right ventricular function at most recent follow‐up. Neither early hemodynamic response nor less severe baseline hemodynamics were associated with better outcomes. Patients who did not have a significant early hemodynamic response to TRE by first follow‐up catheterization were unlikely to show subsequent improvement in PVRi (1/8, 13%). These findings may help clinicians counsel families and guide clinical decision making regarding the timing of advanced therapies.https://doi.org/10.1002/pul2.12328outcomesprostacyclinpulmonary hypertensionpulmonary vascular disease |
| spellingShingle | Justin J. Kochanski Jeffrey A. Feinstein Michelle Ogawa Victor Ritter Rachel K. Hopper Gregory T. Adamson Younger age at initiation of subcutaneous treprostinil is associated with better response in pediatric Group 1 pulmonary arterial hypertension Pulmonary Circulation outcomes prostacyclin pulmonary hypertension pulmonary vascular disease |
| title | Younger age at initiation of subcutaneous treprostinil is associated with better response in pediatric Group 1 pulmonary arterial hypertension |
| title_full | Younger age at initiation of subcutaneous treprostinil is associated with better response in pediatric Group 1 pulmonary arterial hypertension |
| title_fullStr | Younger age at initiation of subcutaneous treprostinil is associated with better response in pediatric Group 1 pulmonary arterial hypertension |
| title_full_unstemmed | Younger age at initiation of subcutaneous treprostinil is associated with better response in pediatric Group 1 pulmonary arterial hypertension |
| title_short | Younger age at initiation of subcutaneous treprostinil is associated with better response in pediatric Group 1 pulmonary arterial hypertension |
| title_sort | younger age at initiation of subcutaneous treprostinil is associated with better response in pediatric group 1 pulmonary arterial hypertension |
| topic | outcomes prostacyclin pulmonary hypertension pulmonary vascular disease |
| url | https://doi.org/10.1002/pul2.12328 |
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