Children with pulmonary arterial hypertension and prostanoid therapy: Long-term hemodynamics

被引:52
|
作者
Siehr, Stephanie L. [1 ]
Ivy, D. Dunbar [2 ]
Miller-Reed, Kathleen [2 ]
Ogawa, Michelle [1 ]
Rosenthal, David N. [1 ]
Feinstein, Jeffrey A. [1 ]
机构
[1] Stanford Univ, Dept Pediat, Div Pediat Cardiol, Palo Alto, CA 94304 USA
[2] Univ Colorado, Dept Pediat, Div Pediat Cardiol, Aurora, CO USA
来源
基金
美国国家卫生研究院;
关键词
pediatric patients; pulmonary arterial hypertension; epoprostenol; treprostinil; hemodynamics; INTRAVENOUS EPOPROSTENOL; SUBCUTANEOUS TREPROSTINIL; PEDIATRIC-PATIENTS; PROSTACYCLIN; SURVIVAL; EFFICACY; BOSENTAN; SAFETY;
D O I
10.1016/j.healun.2013.01.1055
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
BACKGROUND: Pediatric patients with severe pulmonary arterial hypertension (PAH) are treated with intravenous epoprostenol or intravenous or subcutaneous treprostinil. Little is known about longitudinal hemodynamics and outcomes of epoprostenol, treprostinil, and transitions from epoprostenol to treprostinil. METHODS: This was retrospective study of 77 pediatric patients (47 idiopathic PAH, 24 congenital heart disease-PAH) receiving epoprostenol or treprostinil from 1992 to 2010 at 2 centers. Outcomes were defined as living vs dead/transplant. 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. J Heart Lung Transplant 2013;32:546-552 (C) 2013 International Society for Heart and Lung Transplantation. All rights reserved.
引用
收藏
页码:546 / 552
页数:7
相关论文
共 50 条
  • [41] Long-term outcome in pulmonary arterial hypertension: a plea for earlier parenteral prostacyclin therapy
    Delcroix, M.
    Spaas, K.
    Quarck, R.
    [J]. EUROPEAN RESPIRATORY REVIEW, 2009, 18 (114): : 253 - 259
  • [42] Choice of Initial Oral Therapy for Pulmonary Arterial Hypertension: Age and Long-Term Survival
    Heresi, Gustavo A.
    Love, Thomas E.
    Tonelli, Adriano R.
    Highland, Kristin B.
    Dweik, Raed A.
    [J]. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 2018, 198 (08) : 1090 - 1093
  • [43] Long-term ambrisentan therapy provides sustained benefit in patients with pulmonary arterial hypertension
    Oudiz, Ronald J.
    [J]. CHEST, 2007, 132 (04) : 474S - 474S
  • [44] MINOXIDIL - LONG-TERM EFFECT ON PULMONARY HEMODYNAMICS IN PATIENTS WITH SYSTEMIC HYPERTENSION
    ALPERT, MA
    BAUER, JH
    PARKER, BM
    BROOKS, CS
    FREEMAN, JA
    [J]. CLINICAL RESEARCH, 1978, 26 (01): : A1 - A1
  • [45] Long-Term Mechanical Ventilation as Adjunctive Therapy for Children With Severe Pulmonary Hypertension
    Sammut, Paul
    [J]. CHEST, 2016, 149 (04) : 515A - 515A
  • [46] Long-term treatment with oral sildenafil is safe and improves functional capacity and hemodynamics in patients with pulmonary arterial hypertension
    Michelakis, ED
    Tymchak, W
    Noga, M
    Webster, L
    Wu, XC
    Lien, D
    Wang, SH
    Modry, D
    Archer, SL
    [J]. CIRCULATION, 2003, 108 (17) : 2066 - 2069
  • [47] Long-term results of the DelIVery for Pulmonary Arterial Hypertension trial
    Gomberg-Maitland, Mardi
    Bourge, Robert C.
    Shapiro, Shelley M.
    Tarver, James H., III
    Zwicke, Dianne L.
    Feldman, Jeremy P.
    Chakinala, Murali M.
    Frantz, Robert P.
    Torres, Fernando
    Bag, Remzi
    Murphy, Jeffrey A.
    Lautenbach, Amy A.
    Morris, Marty
    Peterson, Leigh
    Waxman, Aaron B.
    [J]. PULMONARY CIRCULATION, 2019, 9 (04)
  • [48] Long-term safety and efficacy of imatinib in pulmonary arterial hypertension
    Frost, Adaani E.
    Barst, Robyn J.
    Hoeper, Marius M.
    Chang, Hyuk-Jae
    Frantz, Robert P.
    Fukumoto, Yoshihiro
    Galie, Nazzareno
    Hassoun, Paul M.
    Klose, Hans
    Matsubara, Hiromi
    Morrell, Nicholas W.
    Peacock, Andrew J.
    Pfeifer, Michael
    Simonneau, Gerald
    Tapson, Victor F.
    Torres, Fernando
    Vizza, Carmine Dario
    Lawrence, David
    Yang, Wei
    Felser, James M.
    Quinn, Deborah A.
    Ghofrani, Hossein-Ardeschir
    [J]. JOURNAL OF HEART AND LUNG TRANSPLANTATION, 2015, 34 (11): : 1366 - 1375
  • [49] Long-term safety and efficacy of imatinib in pulmonary arterial hypertension
    Hoeper, Marius
    Barst, Robyn
    Galie, Nazzareno
    Hassoun, Paul
    Morrell, Nicholas
    Peacock, Andrew
    Simonneau, Gerald
    Tapson, Victor
    Torres, Fernando
    Lawrence, David
    Quinn, Deborah
    Ghofrani, Hossein Ardeschir
    [J]. EUROPEAN RESPIRATORY JOURNAL, 2012, 40
  • [50] Pulmonary arterial hypertension: new challenges in long-term survivors
    Guimaraes, Joana
    Castro, Graca
    Goncalves, Lino
    [J]. EUROPEAN HEART JOURNAL-CARDIOVASCULAR IMAGING, 2024,