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Current and advancing treatments for pulmonary arterial hypertension in childhood

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References

  • Galie N, Hoeper MM, Humbert M, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 2009;30(20):2493-537
  • Humbert M, Morrell NW, Archer SL, et al. Cellular and molecular pathobiology of pulmonary arterial hypertension. J Am Coll Cardiol 2004;43(12 Suppl S):13S-24S
  • D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991;115(5):343-9
  • Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 2006;173(9):1023-30
  • Peacock AJ, Murphy NF, McMurray JJ, et al. An epidemiological study of pulmonary arterial hypertension. Eur Respir J 2007;30(1):104-9
  • van Loon RL, Roofthooft MT, Hillege HL, et al. Pediatric pulmonary hypertension in the Netherlands: epidemiology and characterization during the period 1991 to 2005. Circulation 2011;124(16):1755-64
  • Moledina S, Hislop AA, Foster H, et al. Childhood idiopathic pulmonary arterial hypertension: a national cohort study. Heart 2010;96(17):1401-6
  • Rabinovitch M. Molecular pathogenesis of pulmonary arterial hypertension. J Clin Invest 2008;118(7):2372-9
  • Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med 2004;351(14):1425-36
  • Ghofrani HA, Galie N, Grimminger F, et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med 2013;369(4):330-40
  • Simonneau G, Torbicki A, Hoeper MM, et al. Selexipag: an oral, selective prostacyclin receptor agonist for the treatment of pulmonary arterial hypertension. Eur Respir J 2012;40(4):874-80
  • McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 2002;106(12):1477-82
  • McLaughlin VV, Sitbon O, Badesch DB, et al. Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J 2005;25(2):244-9
  • Rosenzweig EB, Widlitz AC, Barst RJ. Pulmonary arterial hypertension in children. Pediatr Pulmonol 2004;38(1):2-22
  • Barst RJ, Ertel SI, Beghetti M, Ivy DD. Pulmonary arterial hypertension: a comparison between children and adults. Eur Respir J 2011;37(3):665-77
  • Berger RM, Bonnet D. Treatment options for paediatric pulmonary arterial hypertension. Eur Respir Rev 2010;19(118):321-30
  • Berger RM, Beghetti M, Humpl T, et al. Clinical features of paediatric pulmonary hypertension: a registry study. Lancet 2012;379(9815):537-46
  • Haworth SG, Hislop AA. Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006. Heart 2009;95(4):312-17
  • Fraisse A, Jais X, Schleich JM, et al. Characteristics and prospective 2-year follow-up of children with pulmonary arterial hypertension in France. Arch Cardiovasc Dis 2010;103(2):66-74
  • van Loon RL, Roofthooft MT, van Osch-Gevers M, et al. Clinical characterization of pediatric pulmonary hypertension: complex presentation and diagnosis. J Pediatr 2009;155(2):176-82. e1
  • Rosenzweig EB, Ivy DD, Widlitz A, et al. Effects of long-term bosentan in children with pulmonary arterial hypertension. J Am Coll Cardiol 2005;46(4):697-704
  • Ivy DD, Rosenzweig EB, Lemarie JC, et al. Long-term outcomes in children with pulmonary arterial hypertension treated with bosentan in real-world clinical settings. Am J Cardiol 2010;106(9):1332-8
  • van Loon RL, Roofthooft MT, Delhaas T, et al. Outcome of pediatric patients with pulmonary arterial hypertension in the era of new medical therapies. Am J Cardiol 2010;106(1):117-24
  • Barst RJ, McGoon MD, Elliott CG, 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(1):113-22
  • 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(20):2159-69
  • Sitbon O, Humbert M, Jais X, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005;111(23):3105-11
  • Barst RJ, Maislin G, Fishman AP. Vasodilator therapy for primary pulmonary hypertension in children. Circulation 1999;99(9):1197-208
  • Douwes JM, van Loon RL, Hoendermis ES, et al. Acute pulmonary vasodilator response in paediatric and adult pulmonary arterial hypertension: occurrence and prognostic value when comparing three response criteria. Eur Heart J 2011;32(24):3137-46
  • Barst RJ. Pharmacologically induced pulmonary vasodilatation in children and young adults with primary pulmonary hypertension. Chest 1986;89(4):497-503
  • Ivy DD, Abman SH, Barst RJ, et al. Pediatric pulmonary hypertension. J Am Coll Cardiol 2013;62(25 Suppl):D117-26
  • Galie N, Corris PA, Frost A, et al. Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Cardiol 2013;62(25 Suppl):D60-72
  • Barst RJ, Rubin LJ, Long WA, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 1996;334(5):296-301
  • Badesch DB, Tapson VF, McGoon MD, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med 2000;132(6):425-34
  • McLaughlin VV, Genthner DE, Panella MM, Rich S. Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension. N Engl J Med 1998;338(5):273-7
  • McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 2002;106(12):1477-82
  • Nakayama T, Shimada H, Takatsuki S, et al. Efficacy and limitations of continuous intravenous epoprostenol therapy for idiopathic pulmonary arterial hypertension in Japanese children. Circ J 2007;71(11):1785-90
  • Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999;99(14):1858-65
  • Rubin LJ, Mendoza J, Hood M, et al. Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial. Ann Intern Med 1990;112(7):485-91
  • Barst R. How has epoprostenol changed the outcome for patients with pulmonary arterial hypertension? Int J Clin Pract Suppl 2010(168):23-32
  • Simonneau G, Barst RJ, Galie N, et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med 2002;165(6):800-4
  • Barst RJ, Galie N, Naeije R, et al. Long-term outcome in pulmonary arterial hypertension patients treated with subcutaneous treprostinil. Eur Respir J 2006;28(6):1195-203
  • Lang I, Gomez-Sanchez M, Kneussl M, et al. Efficacy of long-term subcutaneous treprostinil sodium therapy in pulmonary hypertension. Chest 2006;129(6):1636-43
  • Hiremath J, Thanikachalam S, Parikh K, et al. Exercise improvement and plasma biomarker changes with intravenous treprostinil therapy for pulmonary arterial hypertension: a placebo-controlled trial. J Heart Lung Transplant 2010;29(2):137-49
  • McLaughlin VV, Benza RL, Rubin LJ, et al. Addition of inhaled treprostinil to oral therapy for pulmonary arterial hypertension: a randomized controlled clinical trial. J Am Coll Cardiol 2010;55(18):1915-22
  • Benza RL, Seeger W, McLaughlin VV, et al. Long-term effects of inhaled treprostinil in patients with pulmonary arterial hypertension: the Treprostinil sodium inhalation used in the management of pulmonary arterial hypertension (TRIUMPH) study open-label extension. J Heart Lung Transplant 2011;30(12):1327-33
  • Jing ZC, Parikh K, Pulido T, et al. Efficacy and safety of oral treprostinil monotherapy for the treatment of pulmonary arterial hypertension: a randomized, controlled trial. Circulation 2013;127(5):624-33
  • Tapson VF, Torres F, Kermeen F, et al. Oral treprostinil for the treatment of pulmonary arterial hypertension in patients on background endothelin receptor antagonist and/or phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C study): a randomized controlled trial. Chest 2012;142(6):1383-90
  • Tapson VF, Jing ZC, Xu KF, et al. Oral treprostinil for the treatment of pulmonary arterial hypertension in patients receiving background endothelin receptor antagonist and phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C2 study): a randomized controlled trial. Chest 2013;144(3):952-8
  • Levy M, Celermajer DS, Bourges-Petit E, et al. Add-on therapy with subcutaneous treprostinil for refractory pediatric pulmonary hypertension. J Pediatr 2011;158(4):584-8
  • 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(11):1704-9
  • Barst RJ, McGoon M, McLaughlin V, et al. Beraprost therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2003;41(12):2119-25
  • Galie N, Humbert M, Vachiery JL, et al. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled trial. J Am Coll Cardiol 2002;39(9):1496-502
  • Nagaya N, Uematsu M, Okano Y, et al. Effect of orally active prostacyclin analogue on survival of outpatients with primary pulmonary hypertension. J Am Coll Cardiol 1999;34(4):1188-92
  • Olschewski H, Simonneau G, Galie N, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002;347(5):322-9
  • McLaughlin VV, Oudiz RJ, Frost A, et al. Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension. Am J Respir Crit Care Med 2006;174(11):1257-63
  • Hoeper MM, Schwarze M, Ehlerding S, et al. Long-term treatment of primary pulmonary hypertension with aerosolized iloprost, a prostacyclin analogue. N Engl J Med 2000;342(25):1866-70
  • Alehan D, Yildirim I, Sahin M, et al. Long-term inhaled iloprost use in children with pulmonary arterial hypertension. Cardiol Young 2012;22(4):396-403
  • Ivy DD, Doran AK, Smith KJ, et al. Short- and long-term effects of inhaled iloprost therapy in children with pulmonary arterial hypertension. J Am Coll Cardiol 2008;51(2):161-9
  • Hoeper MM, Spiekerkoetter E, Westerkamp V, et al. Intravenous iloprost for treatment failure of aerosolised iloprost in pulmonary arterial hypertension. Eur Respir J 2002;20(2):339-43
  • Knudsen L, Schurawlew A, Nickel N, et al. Long-term effects of intravenous iloprost in patients with idiopathic pulmonary arterial hypertension deteriorating on non-parenteral therapy. BMC Pulm Med 2011;11:56
  • Ewert R, Opitz CF, Wensel R, et al. Continuous intravenous iloprost to revert treatment failure of first-line inhaled iloprost therapy in patients with idiopathic pulmonary arterial hypertension. Clin Res Cardiol 2007;96(4):211-17
  • Yoshibayashi M, Nishioka K, Nakao K, et al. Plasma endothelin concentrations in patients with pulmonary hypertension associated with congenital heart defects. Evidence for increased production of endothelin in pulmonary circulation. Circulation 1991;84(6):2280-5
  • Galie N, Manes A, Branzi A. The endothelin system in pulmonary arterial hypertension. Cardiovasc Res 2004;61(2):227-37
  • Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002;346(12):896-903
  • Maiya S, Hislop AA, Flynn Y, Haworth SG. Response to bosentan in children with pulmonary hypertension. Heart 2006;92(5):664-70
  • Hislop AA, Moledina S, Foster H, et al. Long-term efficacy of bosentan in treatment of pulmonary arterial hypertension in children. Eur Respir J 2011;38(1):70-7
  • Barst RJ, Ivy D, Dingemanse J, et al. Pharmacokinetics, safety, and efficacy of bosentan in pediatric patients with pulmonary arterial hypertension. Clin Pharmacol Ther 2003;73(4):372-82
  • Beghetti M, Haworth SG, Bonnet D, et al. Pharmacokinetic and clinical profile of a novel formulation of bosentan in children with pulmonary arterial hypertension: the FUTURE-1 study. Br J Clin Pharmacol 2009;68(6):948-55
  • Beghetti M, Hoeper MM, Kiely DG, et al. Safety experience with bosentan in 146 children 2-11 years old with pulmonary arterial hypertension: results from the European Postmarketing Surveillance program. Pediatr Res 2008;64(2):200-4
  • Beghetti M. Bosentan in pediatric patients with pulmonary arterial hypertension. Curr Vasc Pharmacol 2009;7(2):225-33
  • Galie N, Badesch D, Oudiz R, et al. Ambrisentan therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2005;46(3):529-35
  • Galie N, Olschewski H, Oudiz RJ, et al. Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation 2008;117(23):3010-19
  • Oudiz RJ, Galie N, Olschewski H, et al. Long-term ambrisentan therapy for the treatment of pulmonary arterial hypertension. J Am Coll Cardiol 2009;54(21):1971-81
  • Klinger JR, Oudiz RJ, Spence R, et al. Long-term pulmonary hemodynamic effects of ambrisentan in pulmonary arterial hypertension. Am J Cardiol 2011;108(2):302-7
  • 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(1):27-34
  • Gatfield J, Mueller Grandjean C, Sasse T, et al. Slow receptor dissociation kinetics differentiate macitentan from other endothelin receptor antagonists in pulmonary arterial smooth muscle cells. PLoS One 2012;7(10):e47662
  • Pulido T, Adzerikho I, Channick RN, et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013;369(9):809-18
  • Galie N, Ghofrani HA, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005;353(20):2148-57
  • Rubin LJ, Badesch DB, Fleming TR, et al. Long-term treatment with sildenafil citrate in pulmonary arterial hypertension: the SUPER-2 study. Chest 2011;140(5):1274-83
  • 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(2):324-34
  • Barst RJ, Beghetti M, Pulido T, et al. STARTS-2: long-term survival with oral sildenafil monotherapy in treatment-naive pediatric pulmonary arterial hypertension. Circulation 2014;129(19):1914-23
  • Abman SH, Kinsella JP, Rosenzweig EB, et al. Implications of the U.S. Food and Drug Administration warning against the use of sildenafil for the treatment of pediatric pulmonary hypertension. Am J Respir Crit Care Med 2013;187(6):572-5
  • Galie N, Brundage BH, Ghofrani HA, et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009;119(22):2894-903
  • Oudiz RJ, Brundage BH, Galie N, et al. Tadalafil for the treatment of pulmonary arterial hypertension: a double-blind 52-week uncontrolled extension study. J Am Coll Cardiol 2012;60(8):768-74
  • Takatsuki S, Calderbank M, Ivy DD. Initial experience with tadalafil in pediatric pulmonary arterial hypertension. Pediatr Cardiol 2012;33(5):683-8
  • Jing ZC, Jiang X, Wu BX, et al. Vardenafil treatment for patients with pulmonary arterial hypertension: a multicentre, open-label study. Heart 2009;95(18):1531-6
  • Jing ZC, Yu ZX, Shen JY, et al. Vardenafil in pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled study. Am J Respir Crit Care Med 2011;183(12):1723-9
  • Barst RJ. PDGF signaling in pulmonary arterial hypertension. J Clin Invest 2005;115(10):2691-4
  • Ghofrani HA, Morrell NW, Hoeper MM, et al. Imatinib in pulmonary arterial hypertension patients with inadequate response to established therapy. Am J Respir Crit Care Med 2010;182(9):1171-7
  • Hoeper MM, Barst RJ, Bourge RC, et al. Imatinib mesylate as add-on therapy for pulmonary arterial hypertension: results of the randomized IMPRES study. Circulation 2013;127(10):1128-38
  • Fukumoto Y, Yamada N, Matsubara H, et al. Double-blind, placebo-controlled clinical trial with a rho-kinase inhibitor in pulmonary arterial hypertension. Circ J 2013;77(10):2619-25
  • Zhu JH, Wang XX, Zhang FR, et al. Safety and efficacy of autologous endothelial progenitor cells transplantation in children with idiopathic pulmonary arterial hypertension: open-label pilot study. Pediatr Transplant 2008;12(6):650-5
  • Smadja DM, Gaussem P, Mauge L, et al. Circulating endothelial cells: a new candidate biomarker of irreversible pulmonary hypertension secondary to congenital heart disease. Circulation 2009;119(3):374-81
  • Hoeper MM, Faulenbach C, Golpon H, et al. Combination therapy with bosentan and sildenafil in idiopathic pulmonary arterial hypertension. Eur Respir J 2004;24(6):1007-10
  • D’Alto M, Romeo E, Argiento P, et al. Bosentan-sildenafil association in patients with congenital heart disease-related pulmonary arterial hypertension and Eisenmenger physiology. Int J Cardiol 2012;155(3):378-82
  • Lunze K, Gilbert N, Mebus S, et al. First experience with an oral combination therapy using bosentan and sildenafil for pulmonary arterial hypertension. Eur J Clin Invest 2006;36(Suppl 3):32-8
  • Porhownik NR, Al-Sharif H, Bshouty Z. Addition of sildenafil in patients with pulmonary arterial hypertension with inadequate response to bosentan monotherapy. Can Respir J 2008;15(8):427-30
  • Mathai SC, Girgis RE, Fisher MR, et al. Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J 2007;29(3):469-75
  • Simonneau G, Rubin LJ, Galie N, et al. Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med 2008;149(8):521-30
  • Channick RN, Olschewski H, Seeger W, et al. Safety and efficacy of inhaled treprostinil as add-on therapy to bosentan in pulmonary arterial hypertension. J Am Coll Cardiol 2006;48(7):1433-7
  • Benza RL, Rayburn BK, Tallaj JA, et al. Treprostinil-based therapy in the treatment of moderate-to-severe pulmonary arterial hypertension: long-term efficacy and combination with bosentan. Chest 2008;134(1):139-45
  • Douwes JM, Roofthooft MT, Van Loon RL, et al. Sildenafil add-on therapy in paediatric pulmonary arterial hypertension, experiences of a national referral centre. Heart 2014;100(3):224-30
  • Kerstein D, Levy PS, Hsu DT, et al. Blade balloon atrial septostomy in patients with severe primary pulmonary hypertension. Circulation 1995;91(7):2028-35
  • Thanopoulos BD, Georgakopoulos D, Tsaousis GS, Simeunovic S. Percutaneous balloon dilatation of the atrial septum: immediate and midterm results. Heart 1996;76(6):502-6
  • Micheletti A, Hislop AA, Lammers A, et al. Role of atrial septostomy in the treatment of children with pulmonary arterial hypertension. Heart 2006;92(7):969-72
  • Law MA, Grifka RG, Mullins CE, Nihill MR. Atrial septostomy improves survival in select patients with pulmonary hypertension. Am Heart J 2007;153(5):779-84
  • Sandoval J, Gaspar J, Pulido T, et al. Graded balloon dilation atrial septostomy in severe primary pulmonary hypertension. A therapeutic alternative for patients nonresponsive to vasodilator treatment. J Am Coll Cardiol 1998;32(2):297-304
  • Blanc J, Vouhe P, Bonnet D. Potts shunt in patients with pulmonary hypertension. N Engl J Med 2004;350(6):623
  • Labombarda F, Maragnes P, Dupont-Chauvet P, Serraf A. Potts anastomosis for children with idiopathic pulmonary hypertension. Pediatr Cardiol 2009;30(8):1143-5
  • Baruteau AE, Serraf A, Levy M, et al. Potts shunt in children with idiopathic pulmonary arterial hypertension: long-term results. Ann Thorac Surg 2012;94(3):817-24
  • Apitz C, Honjo O, Humpl T, et al. Biventricular structural and functional responses to aortic constriction in a rabbit model of chronic right ventricular pressure overload. J Thorac Cardiovasc Surg 2012;144(6):1494-501
  • Benden C, Edwards LB, Kucheryavaya AY, et al. The Registry of the International Society for Heart and Lung Transplantation: sixteenth official pediatric lung and heart-lung transplantation report – 2013; focus theme: age. J Heart Lung Transplant 2013;32(10):989-97
  • Lammers AE, Burch M, Benden C, et al. Lung transplantation in children with idiopathic pulmonary arterial hypertension. Pediatr Pulmonol 2010;45(3):263-9
  • Schaellibaum G, Lammers AE, Faro A, et al. Bilateral lung transplantation for pediatric idiopathic pulmonary arterial hypertension: a multi-center experience. Pediatr Pulmonol 2011;46(11):1121-7
  • Goldstein BS, Sweet SC, Mao J, et al. Lung transplantation in children with idiopathic pulmonary arterial hypertension: an 18-year experience. J Heart Lung Transplant 2011;30(10):1148-52
  • Hoeper MM, Markevych I, Spiekerkoetter E, et al. Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J 2005;26(5):858-63
  • McLaughlin VV, Gaine SP, Howard LS, et al. Treatment goals of pulmonary hypertension. J Am Coll Cardiol 2013;62(25 Suppl):D73-81
  • McLaughlin VV, McGoon MD. Pulmonary arterial hypertension. Circulation 2006;114(13):1417-31
  • Boissel JP, Collet JP, Moleur P, Haugh M. Surrogate endpoints: a basis for a rational approach. Eur J Clin Pharmacol 1992;43(3):235-44
  • Fleming TR, Powers JH. Biomarkers and surrogate endpoints in clinical trials. Stat Med 2012;31(25):2973-84
  • Nickel N, Golpon H, Greer M, et al. The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 2012;39(3):589-96
  • Barst RJ, Chung L, Zamanian RT, et al. Functional class improvement and 3-year survival outcomes in patients with pulmonary arterial hypertension in the REVEAL Registry. Chest 2013;144(1):160-8
  • Sitbon O, Humbert M, Nunes H, et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol 2002;40(4):780-8
  • Henkens IR, Van Wolferen SA, Gan CT, et al. Relation of resting heart rate to prognosis in patients with idiopathic pulmonary arterial hypertension. Am J Cardiol 2009;103(10):1451-6
  • van Loon RL, Hoendermis ES, Duffels MG, et al. Long-term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-to-pulmonary shunt: does the beneficial effect persist? Am Heart J 2007;154(4):776-82
  • Lammers AE, Munnery E, Hislop AA, Haworth SG. Heart rate variability predicts outcome in children with pulmonary arterial hypertension. Int J Cardiol 2010;142(2):159-65
  • 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(2):1370-7
  • 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(2):280-5
  • Savarese G, Paolillo S, Costanzo P, et al. Do changes of 6-minute walk distance predict clinical events in patients with pulmonary arterial hypertension? A meta-analysis of 22 randomized trials. J Am Coll Cardiol 2012;60(13):1192-201
  • Li AM, Yin J, Au JT, et al. Standard reference for the six-minute-walk test in healthy children aged 7 to 16 years. Am J Respir Crit Care Med 2007;176(2):174-80
  • Sajan I, Manlhiot C, Reyes J, et al. Pulmonary arterial capacitance in children with idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with congenital heart disease: relation to pulmonary vascular resistance, exercise capacity, and survival. Am Heart J 2011;162(3):562-8
  • Wensel R, Opitz CF, Anker SD, et al. Assessment of survival in patients with primary pulmonary hypertension: importance of cardiopulmonary exercise testing. Circulation 2002;106(3):319-24
  • Oudiz RJ, Midde R, Hovenesyan A, et al. Usefulness of right-to-left shunting and poor exercise gas exchange for predicting prognosis in patients with pulmonary arterial hypertension. Am J Cardiol 2010;105(8):1186-91
  • Cooper DM, Weiler-Ravell D, Whipp BJ, Wasserman K. Aerobic parameters of exercise as a function of body size during growth in children. J Appl Physiol Respir Environ Exerc Physiol 1984;56(3):628-34
  • Ten Harkel AD, Takken T, Van Osch-Gevers M, Helbing WA. Normal values for cardiopulmonary exercise testing in children. Eur J Cardiovasc Prev Rehabil 2011;18(1):48-54
  • Yetman AT, Taylor AL, Doran A, Ivy DD. Utility of cardiopulmonary stress testing in assessing disease severity in children with pulmonary arterial hypertension. Am J Cardiol 2005;95(5):697-9
  • Lammers AE, Diller GP, Odendaal D, et al. Comparison of 6-min walk test distance and cardiopulmonary exercise test performance in children with pulmonary hypertension. Arch Dis Child 2011;96(2):141-7
  • Nagaya N, Nishikimi T, Uematsu M, et al. Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension. Circulation 2000;102(8):865-70
  • Benza RL, Miller DP, Gomberg-Maitland M, et al. Predicting survival in pulmonary arterial hypertension: insights from the Registry to evaluate early and long-term pulmonary arterial hypertension disease management (REVEAL). Circulation 2010;122(2):164-72
  • Fijalkowska A, Kurzyna M, Torbicki A, et al. Serum N-terminal brain natriuretic peptide as a prognostic parameter in patients with pulmonary hypertension. Chest 2006;129(5):1313-21
  • Bernus A, Wagner BD, Accurso F, et al. Brain natriuretic peptide levels in managing pediatric patients with pulmonary arterial hypertension. Chest 2009;135(3):745-51
  • Van Albada ME, Loot FG, Fokkema R, et al. Biological serum markers in the management of pediatric pulmonary arterial hypertension. Pediatr Res 2008;63(3):321-7
  • Lammers AE, Hislop AA, Haworth SG. Prognostic value of B-type natriuretic peptide in children with pulmonary hypertension. Int J Cardiol 2009;135(1):21-6
  • Takatsuki S, Wagner BD, Ivy DD. B-type natriuretic peptide and amino-terminal pro-B-type natriuretic peptide in pediatric patients with pulmonary arterial hypertension. Congenit Heart Dis 2012;7(3):259-67
  • Forfia PR, Fisher MR, Mathai SC, et al. Tricuspid annular displacement predicts survival in pulmonary hypertension. Am J Respir Crit Care Med 2006;174(9):1034-41
  • Alkon J, Humpl T, Manlhiot C, et al. Usefulness of the right ventricular systolic to diastolic duration ratio to predict functional capacity and survival in children with pulmonary arterial hypertension. Am J Cardiol 2010;106(3):430-6
  • Kassem E, Humpl T, Friedberg MK. Prognostic significance of 2-dimensional, M-mode, and Doppler echo indices of right ventricular function in children with pulmonary arterial hypertension. Am Heart J 2013;165(6):1024-31
  • Moledina S, Pandya B, Bartsota M, et al. Prognostic significance of cardiac magnetic resonance imaging in children with pulmonary hypertension. Circ Cardiovasc Imaging 2013;6(3):407-14
  • van Wolferen SA, Marcus JT, Boonstra A, et al. Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension. Eur Heart J 2007;28(10):1250-7
  • Eysmann SB, Palevsky HI, Reichek N, et al. Two-dimensional and Doppler-echocardiographic and cardiac catheterization correlates of survival in primary pulmonary hypertension. Circulation 1989;80(2):353-60
  • Humbert M, Sitbon O, Yaici A, et al. Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J 2010;36(3):549-55
  • Humbert M, Sitbon O, Chaouat A, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 2010;122(2):156-63
  • Thenappan T, Shah SJ, Rich S, et al. Survival in pulmonary arterial hypertension: a reappraisal of the NIH risk stratification equation. Eur Respir J 2010;35(5):1079-87
  • Yung D, Widlitz AC, Rosenzweig EB, et al. Outcomes in children with idiopathic pulmonary arterial hypertension. Circulation 2004;110(6):660-5
  • Humpl T, Reyes JT, Holtby H, et al. Beneficial effect of oral sildenafil therapy on childhood pulmonary arterial hypertension: twelve-month clinical trial of a single-drug, open-label, pilot study. Circulation 2005;111(24):3274-80
  • Mahapatra S, Nishimura RA, Sorajja P, et al. Relationship of pulmonary arterial capacitance and mortality in idiopathic pulmonary arterial hypertension. J Am Coll Cardiol 2006;47(4):799-803
  • Carmosino MJ, Friesen RH, Doran A, Ivy DD. Perioperative complications in children with pulmonary hypertension undergoing noncardiac surgery or cardiac catheterization. Anesth Analg 2007;104(3):521-7
  • Hill KD, Lim DS, Everett AD, et al. Assessment of pulmonary hypertension in the pediatric catheterization laboratory: current insights from the Magic registry. Catheter Cardiovasc Interv 2010;76(6):865-73
  • Taylor CJ, Derrick G, McEwan A, et al. Risk of cardiac catheterization under anaesthesia in children with pulmonary hypertension. Br J Anaesth 2007;98(5):657-61
  • Ploegstra M, Douwes JM, Roofthooft MT, et al. Identification of treatment goals in paediatric pulmonary arterial hypertension. Eur Respir J. In press
  • Ploegstra M, Roofthooft M, Douwes M, et al. The value of echocardiography in pediatric pulmonary arterial hypertension: assessing disease severity and outcome. Am J Respir Crit Care Med 2014;189:A3256

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