Abstract | BACKGROUND: METHODS: RESULTS: Mean age at baseline was 7.7 ± 5.2 years, with follow-up of 4.3 ± 3.4 years. Thirty-seven patients were treated with epoprostenol, 20 with treprostinil, and 20 were transitioned from epoprostenol to treprostinil. Mean pulmonary-to-systemic vascular resistance ratio (Rp/Rs) for epoprostenol was 1.0 ± 0.4, 0.8 ± 0.4, 0.8 ± 0.4, 1.0 ± 0.4, and 1.2 ± 0.4, respectively, at baseline, 1, 2, 3, and 4 years. For treprostinil, Rp/Rs was 0.9 ± 0.3, 0.7 ± 0.3, 0.5 ± 0.2, (p < 0.01 vs baseline), and 1.1 ± 0.2, respectively, at baseline, 1, 2, and 3 to 4 years, respectively. There were similar changes in mean pulmonary artery pressure and pulmonary vascular resistance index. The Rp/Rs 1 year after epoprostenol to treprostinil transition increased from 0.6 to 0.8 (n = 7). Changes not statistically significant unless noted. Eight patients died or received a transplant within 2 years of baseline; compared with the rest of the cohort, mean baseline Rp/Rs, right atrial pressure, and pulmonary vascular resistance index were significantly worse in this group. Thirty-nine patients remain on prostanoids, 17 are off, 16 died, and 5 received heart-lung transplant. Kaplan-Meier 5-year transplant-free survival was 70% (95% confidence interval, 56%-80%). CONCLUSION: There was improvement in Rp/Rs on both therapies at 1 to 2 years that was not sustained. The 5-year transplant-free survival was better than in similar adult studies.
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Authors | Stephanie L Siehr, D Dunbar Ivy, Kathleen Miller-Reed, Michelle Ogawa, David N Rosenthal, Jeffrey A Feinstein |
Journal | The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation
(J Heart Lung Transplant)
Vol. 32
Issue 5
Pg. 546-52
(May 2013)
ISSN: 1557-3117 [Electronic] United States |
PMID | 23453572
(Publication Type: Journal Article, Multicenter Study, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
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Copyright | Copyright © 2013 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved. |
Chemical References |
- Antihypertensive Agents
- Prostaglandins
- Epoprostenol
- treprostinil
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Topics |
- Administration, Intravenous
- Adolescent
- Antihypertensive Agents
(administration & dosage, pharmacology, therapeutic use)
- Blood Pressure
(drug effects, physiology)
- Child
- Child, Preschool
- Epoprostenol
(administration & dosage, analogs & derivatives, pharmacology, therapeutic use)
- Familial Primary Pulmonary Hypertension
- Female
- Follow-Up Studies
- Hemodynamics
(drug effects, physiology)
- Humans
- Hypertension, Pulmonary
(drug therapy, mortality, physiopathology)
- Infusions, Subcutaneous
- Longitudinal Studies
- Male
- Prostaglandins
(administration & dosage, pharmacology, therapeutic use)
- Retrospective Studies
- Survival Rate
- Treatment Outcome
- Vascular Resistance
(drug effects, physiology)
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