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2)Cerro MJ, Abman S, Diaz G, et al. A consensus approach to the classification of pediatric pulmonary hypertensive vascular disease: Report from the PVRI Pediatric Taskforce, Panama 2011. Pulm Circ. 2011; 1: 286-98
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3)Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013; 62(25 Suppl): D34-41
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4)Barst RJ, Ivy DD, Foreman AJ, et al. Four- and seven-year outcomes of patients with congenital heart disease-associated pulmonary arterial hypertension (from the REVEAL Registry). Am J Cardiol. 2014; 113: 147-55
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5)Zijlstra WM, Douwes JM, Rosenzweig EB, et al. Survival differences in pediatric pulmonary arterial hypertension: clues to a better understanding of outcome and optimal treatment strategies. J Am Coll Cardiol. 2014; 63: 2159-69
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6)Deng Z, Morse JH, Slager SL, et al. Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet. 2000; 67: 737-44
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7)Lane KB, Machado RD, Pauciulo MW, et al. Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension. The International PPH Consortium. Nat Genet. 2000; 26: 81-4
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8)Austin ED, Loyd JE. Genetics and mediators in pulmonary arterial hypertension. Clin Chest Med. 2007; 28: 43-57
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9)Chida A, Shintani M, Yagi H, et al. Outcomes of childhood pulmonary arterial hypertension in BMPR2 and ALK1 mutation carriers. Am J Cardiol. 2012; 110: 586-93
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10)Rosenzweig EB, Morse JH, Knowles JA, et al. Clinical implications of determining BMPR2 mutation status in a large cohort of children and adults with pulmonary arterial hypertension. J Heart Lung Transplant. 2008; 27: 668-74
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11)Shimodaira K, Okubo Y, Ochiai E, et al. Gene expression analysis of a murine model with pulmonary vascular remodeling compared to end-stage IPAH lungs. Respir Res. 2012; 13: 103
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12)Lammers AE, Adatia I, Cerro MJ, et al. Functional classification of pulmonary hypertension in children: Report from the PVRI pediatric taskforce, Panama 2011. Pulm Circ. 2011; 1: 280-5
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13)Barst RJ, Beghetti M, Ivy DD, et al. Pulmonary arterial hypertension: a comparison between children and adults. Eur Respir J. 2011; 37: 665-77
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14)Barst RJ, Maislin G, Fishman AP. Vasodilator therapy for primary pulmonary hypertension in children. Circulation. 1999; 99: 1197-208
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15)Barst RJ, McGoon MD, Ivy DD, et al. Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management. Circulation. 2012; 125: 113-22
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16)Apitz C, Zimmermann R, Kreuder J, et al. Assessment of pulmonary endothelial function during invasive testing in children and adolescents with idiopathic pulmonary arterial hypertension. J Am Coll Cardiol. 2012; 60: 157-64
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17)Douwes JM, Roofthooft MT, Bartelds B, et al. Pulsatile haemodynamic parameters are predictors of survival in paediatric pulmonary arterial hypertension. Int J Cardiol. 2013; 168: 1370-7
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18)Wagner BD, Takatsuki S, Accurso FJ, et al. Evaluation of circulating proteins and hemodynamics towards predicting mortality in children with pulmonary arterial hypertension. PLoS One. 2013; 8: e80235
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19)Duncan M, Wagner BD, Murray K, et al. Circulating cytokines and growth factors in pediatric pulmonary hypertension. Mediators Inflamm. 2012; 2012: 143428
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20)Chida A, Sato H, Shintani M, et al. Soluble ST2 and N-terminal pro-brain natriuretic peptide combination. Useful biomarker for predicting outcome of childhoodpulmonary arterial hypertension. Circ J. 2014; 78: 436-42
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21)Takatsuki S, Nakayama T, Jone PN, et al. Tissue Doppler imaging predicts adverse outcome in children with idiopathic pulmonary arterial hypertension. J Pediatr. 2012; 161: 1126-31
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22)Jone PN, Hinzman J, Wagner BD, et al. Right ventricular to left ventricular diameter ratio at end-systole in evaluating outcomes in children with pulmonary hypertension. J Am Soc Echocardiogr. 2014; 27: 172-8
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23)Maxey DM, Ivy DD, Ogawa MT, et al. Food and Drug Administration (FDA) postmarket reported side effects and adverse events associated with pulmonary hypertension therapy in pediatric patients. Pediatr Cardiol. 2013; 34: 1628-36
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24)Yokoyama Y, Tomatsuri M, Hayashi H, et al. Simultaneous microdetermination of bosentan, ambrisentan, sildenafil, and tadalafil in plasma using liquid chromatography/tandem mass spectrometry for pediatric patients with pulmonary arterial hypertension. J Pharm Biomed Anal. 2014; 89: 227-32
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25)Siehr SL, Ivy DD, Miller-Reed K, et al. Children with pulmonary arterial hypertension and prostanoid therapy: long-term hemodynamics. J Heart Lung Transplant. 2013; 32: 546-52
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26)Krishnan U, Takatsuki S, Ivy DD, et al. Effectiveness and safety of inhaled treprostinil for the treatment of pulmonary arterial hypertension in children. Am J Cardiol. 2012; 110: 1704-9
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27)Barst RJ, Ivy DD, Gaitan G, et al. A randomized, double-blind, placebo-controlled, dose-ranging study of oral sildenafil citrate in treatment-naive children with pulmonary arterial hypertension. Circulation. 2012; 125: 324-34
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28)Barst RJ, Beghetti M, Pulido T, et al; STARTS-2 Investigators. STARTS-2: long-term survival with oral sildenafil monotherapy in treatment-naive pediatric pulmonary arterial hypertension. Circulation. 2014; 129: 1914-23
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29)Takatsuki S, Calderbank M, Ivy DD. Initial experience with tadalafil in pediatric pulmonary arterial hypertension. Pediatr Cardiol. 2012; 33: 683-8
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30)Kohno H, Ichida F, Hirono K, et al. Plasma concentrations of tadalafil in children with pulmonary arterial hypertension. Ther Drug Monit. 2014; 36: 576-83
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31)Sabri MR, Beheshtian E. Comparison of the therapeutic and side effects of tadalafil and sildenafil in children and adolescents with pulmonary arterial hypertension. Pediatr Cardiol. 2014; 35: 699-704
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32)Takatsuki S, Rosenzweig EB, Zuckerman W, et al. Clinical safety, pharmacokinetics, and efficacy of ambrisentan therapy in children with pulmonary arterial hypertension. Pediatr Pulmonol. 2013; 48: 27-34
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33)Blanc J, Vouhé P, Bonnet D. Potts shunt in patients with pulmonary hypertension. N Engl J Med. 2004; 350: 623
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34)Baruteau AE, Serraf A, Lévy M, et al. Potts shunt in children with idiopathic pulmonary arterial hypertension: long-term results. Ann Thorac Surg. 2012; 94: 817-24
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35)Esch JJ, Shah PB, Cockrill BA, et al. Transcatheter Potts shunt creation in patients with severe pulmonary arterial hypertension: initial clinical experience. J Heart Lung Transplant. 2013; 32: 381-7
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36)Saji T. Update on pediatric pulmonary arterial hypertension. Differences and similarities to adult disease. Circ J. 2013; 77: 2639-50
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