Sildenafil for the treatment of pulmonary hypertension in children

References

• Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med 2004;351:1425-36
   PubMed Web of Science ®Google Scholar
• 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;30:2493-537
   PubMed Web of Science ®Google Scholar
• Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2013;62:D34-41
   PubMed Web of Science ®Google Scholar
• 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
   Google Scholar
• Barst RJ, Ertel SI, Beghetti M, Ivy DD. Pulmonary arterial hypertension: a comparison between children and adults. Eur Respir J 2011;37:665-77
   PubMed Web of Science ®Google Scholar
• Abman SH, Ivy DD. Recent progress in understanding pediatric pulmonary hypertension. Curr Opin Pediatr 2011;23:298-304
   PubMed Web of Science ®Google Scholar
• Ivy DD, Abman SH, Barst RJ, et al. Pediatric pulmonary hypertension. J Am Coll Cardiol 2013;62:D117-26
   PubMed Web of Science ®Google Scholar
• Haworth SG, Beghetti M. Assessment of endpoints in the pediatric population: congenital heart disease and idiopathic pulmonary arterial hypertension. Curr Opin Pulm Med 2010;16(Suppl 1):S35-41
   Google Scholar
• Berger RM, Beghetti M, Humpl T, et al. Clinical features of paediatric pulmonary hypertension: a registry study. Lancet 2012;379:537-46
   PubMed Web of Science ®Google Scholar
• Fraisse A, Butrous G, Taylor MB, et al. Intravenous sildenafil for postoperative pulmonary hypertension in children with congenital heart disease. Intensive Care Med 2011;37:502-9
   PubMed Web of Science ®Google Scholar
• Haworth SG, Hislop AA. Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006. Heart 2009;95:312-17
   PubMed Web of Science ®Google Scholar
• 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;124:1755-64
   PubMed Web of Science ®Google Scholar
• Moledina S, Hislop AA, Foster H, et al. Childhood idiopathic pulmonary arterial hypertension: a national cohort study. Heart 2010;96:1401-6
   PubMed Web of Science ®Google Scholar
• Hui-li G. The management of acute pulmonary arterial hypertension. Cardiovasc Ther 2011;29:153-75
   Google Scholar
• McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol 2009;53:1573-619
   PubMed Web of Science ®Google Scholar
• Barst RJ, Rubin LJ, Long WA, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N Engl J Med 1996;334:296-302
   PubMed Web of Science ®Google Scholar
• McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 2002;106:1477-82
   PubMed Web of Science ®Google Scholar
• 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;40:780-8
   PubMed Web of Science ®Google Scholar
• Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002;346:896-903
   PubMed Web of Science ®Google Scholar
• Channick RN, Simonneau G, Sitbon O, et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet 2001;358:1119-23
   PubMed Web of Science ®Google Scholar
• Tracleer (bosentan). Full prescribing information. Actelion Pharmaceuticals US, Inc., South San Francisco, CA, USA. 2011
   Google Scholar
• 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;117:3010-19
   PubMed Web of Science ®Google Scholar
• Opsumit (macitentan). Full prescribing information. Actelion Pharmaceuticals US, Inc., South San Francisco, CA, USA. 2013
   Google Scholar
• Pulido T, Adzerikho I, Channick RN, et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013;369:809-18
   PubMed Web of Science ®Google Scholar
• Letairis (ambrisentan). Full prescribing information. Gilead Sciences, Inc., Foster City, CA, USA. 2012
   Google Scholar
• Giaid A, Saleh D. Reduced expression of endothelial nitric oxide synthase in the lungs of patients with pulmonary hypertension. N Engl J Med 1995;333:214-21
   PubMed Web of Science ®Google Scholar
• Pepke-Zaba J, Higenbottam TW, Dinh-Xuan AT, et al. Inhaled nitric oxide as a cause of selective pulmonary vasodilatation in pulmonary hypertension. Lancet 1991;338:1173-4
   PubMed Web of Science ®Google Scholar
• Adempas (riociguat). Full prescribing information. Bayer HealthCare Pharmaceuticals Inc, Whippany, NJ, USA. 2013
   Google Scholar
• Ghofrani HA, Galie N, Grimminger F, et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med 2013;369:330-40
   PubMed Web of Science ®Google Scholar
• Galie N, Ghofrani HA, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005;353:2148-57
   PubMed Web of Science ®Google Scholar
• 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;149:521-30
   PubMed Web of Science ®Google Scholar
• Galie N, Brundage BH, Ghofrani HA, et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009;119:2894-903
   PubMed Web of Science ®Google Scholar
• Revatio® (sildenafil citrate). Full prescribing information. Pfizer Inc, New York, NY, USA. 2014
   Google Scholar
• Nichols DJ, Muirhead GJ, Harness JA. Pharmacokinetics of sildenafil after single oral doses in healthy male subjects: absolute bioavailability, food effects and dose proportionality. Br J Clin Pharmacol 2002;53:5S-12S
   PubMed Web of Science ®Google Scholar
• Vachiery JL, Huez S, Gillies H, et al. Safety, tolerability and pharmacokinetics of an intravenous bolus of sildenafil in patients with pulmonary arterial hypertension. Br J Clin Pharmacol 2011;71:289-92
   PubMed Web of Science ®Google Scholar
• Burgess G, Hoogkamer H, Collings L, Dingemanse J. Mutual pharmacokinetic interactions between steady-state bosentan and sildenafil. Eur J Clin Pharmacol 2008;64:43-50
   PubMed Web of Science ®Google Scholar
• Paul GA, Gibbs JS, Boobis AR, et al. Bosentan decreases the plasma concentration of sildenafil when coprescribed in pulmonary hypertension. Br J Clin Pharmacol 2005;60:107-12
   PubMed Web of Science ®Google Scholar
• Hatano M, Yao A, Kinugawa K, et al. Acute effect of sildenafil is maintained in pulmonary arterial hypertension patients chronically treated with bosentan. Int Heart J 2011;52:233-9
   Google Scholar
• Spence R, Mandagere A, Dufton C, Venitz J. Pharmacokinetics and safety of ambrisentan in combination with sildenafil in healthy volunteers. J Clin Pharmacol 2008;48:1451-9
   PubMed Web of Science ®Google Scholar
• Rubin LJ, Badesch DB, Fleming TR, et al. Long-term treatment with sildenafil citrate in pulmonary arterial hypertension: the SUPER-2 study. Chest 2011;140:1274-83
   PubMed Web of Science ®Google Scholar
• Simonneau G, Rubin LJ, Galie N, et al. Long-term sildenafil added to intravenous epoprostenol in patients with pulmonary arterial hypertension. J Heart Lung Transplant 2014;33:689-97
   Google Scholar
• 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
   PubMedGoogle Scholar
• Abrams D, Schulze-Neick I, Magee AG. Sildenafil as a selective pulmonary vasodilator in childhood primary pulmonary hypertension. Heart 2000;84:E4
   PubMedGoogle Scholar
• Karatza AA, Narang I, Rosenthal M, et al. Treatment of primary pulmonary hypertension with oral sildenafil. Respiration 2004;71:192-4
   PubMedGoogle Scholar
• Karatza AA, Bush A, Magee AG. Safety and efficacy of sildenafil therapy in children with pulmonary hypertension. Int J Cardiol 2005;100:267-73
   PubMed Web of Science ®Google Scholar
• Carroll WD, Dhillon R. Sildenafil as a treatment for pulmonary hypertension. Arch Dis Child 2003;88:827-8
   Web of Science ®Google Scholar
• 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
   PubMed Web of Science ®Google Scholar
• 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;129:1914-23
   PubMed Web of Science ®Google Scholar
• US FDA. Revatio (sildenafil): Drug Safety Communication - Recommendation Against Use in Children [8-30-12]. Available from: www.fda.gov/Safety/MedWatch/SafetyInformation/SafetyAlertsforHumanMedicalProducts/ucm317743.htm [Last accessed 14 April 2014]
   Google Scholar
• Scientific Leadership Council. Revatio (Sildenafil) for Pediatric Use. Available from: www.phassociation.org/document.doc?id=1903 [Last accessed 14 April 2014]
   Google Scholar
• 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;187:572-5
   PubMedGoogle Scholar
• McElhinney DB. A new START for sildenafil in pediatric pulmonary hypertension: reframing the dose-survival relationship in the STARTS-2 trial. Circulation 2014;129:1905-8
   Google Scholar
• US FDA. FDA clarifies warning about pediatric use of revatio (sildenafil) for pulmonary arterial hypertension [3-31-14]. Available from: www.fda.gov/Drugs/DrugSafety/ucm390876.htm [Last accessed 14 April 2014]
   Google Scholar
• Kothari SS, Duggal B. Chronic oral sildenafil therapy in severe pulmonary artery hypertension. Indian Heart J 2002;54:404-9
   PubMedGoogle Scholar
• 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;111:3274-80
   PubMed Web of Science ®Google Scholar
• Sastry BK, Narasimhan C, Reddy NK, et al. A study of clinical efficacy of sildenafil in patients with primary pulmonary hypertension. Indian Heart J 2002;54:410-14
   PubMedGoogle Scholar
• Kim HW, Kim GB, Je HG, et al. Pulmonary arterial hypertension in children: a single center experience Korean Circ J. 2008;38:644-50
   Google Scholar
• Roldan T, Deiros L, Romero JA, et al. Safety and tolerability of targeted therapies for pulmonary hypertension in children. Pediatr Cardiol 2014;35:490-8
   Google Scholar
• 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;100:224-30
   PubMed Web of Science ®Google Scholar
• Watt S, Hayashi N, Harnish L, Gao X. Population pharmacokinetics (PK) of sildenafil in paediatric and adult patients with pulmonary arterial hypertension (PAH). Presented at European Society of Cardiology Annual Meeting; Stockholm, Sweden; 2010
   Google Scholar
• Mukherjee A, Dombi T, Wittke B, Lalonde R. Population pharmacokinetics of sildenafil in term neonates: evidence of rapid maturation of metabolic clearance in the early postnatal period. Clin Pharmacol Ther 2009;85:56-63
   PubMed Web of Science ®Google Scholar
• Harnisch L, Hayashi N. Exercise tolerability in children with pulmonary arterial hypertension (PAH): a population PK/PD assessment of the effects of sildenafil. Presented at European Respiratory Society Annual Congress; 12–16 September 2009; Vienna, Austria
   Google Scholar
• Harnisch L, Gao X, Chanu P. Dose recommendation and risk/benefit assessment of Revatio in pediatric pulmonary arterial hypertension using a modelling and simulation approach [abstract]. Eur Heart J 2012;33(Suppl 1)
   Google Scholar
• Taguchi M, Ichida F, Hirono K, et al. Pharmacokinetics of bosentan in routinely treated Japanese pediatric patients with pulmonary arterial hypertension. Drug Metab Pharmacokinet 2011;26:280-7
   PubMed Web of Science ®Google Scholar
• Baquero H, Soliz A, Neira F, et al. Oral sildenafil in infants with persistent pulmonary hypertension of the newborn: a pilot randomized blinded study. Pediatrics 2006;117:1077-83
   PubMed Web of Science ®Google Scholar
• Khorana M, Yookaseam T, Layangool T, et al. Outcome of oral sildenafil therapy on persistent pulmonary hypertension of the newborn at Queen Sirikit National Institute of Child Health. J Med Assoc Thai 2011;94(Suppl 3):S64-73
   PubMedGoogle Scholar
• Steinhorn RH, Kinsella JP, Pierce C, et al. Intravenous sildenafil in the treatment of neonates with persistent pulmonary hypertension. J Pediatr 2009;155:841-7. e1
   PubMed Web of Science ®Google Scholar
• Limjoco J, Paquette L, Ramanathan R, et al. Changes in mean arterial blood pressure during sildenafil use in neonates with meconium aspiration syndrome or sepsis. Am J Ther 2013. [ Epub ahead of print]
   Google Scholar
• Uslu S, Kumtepe S, Bulbul A, et al. A comparison of magnesium sulphate and sildenafil in the treatment of the newborns with persistent pulmonary hypertension: a randomized controlled trial. J Trop Pediatr 2011;57:245-50
   PubMed Web of Science ®Google Scholar
• Herrera Torres R, Concha Gonzalez EP, Holberto Castillo J, et al. Sildenafil oral como alternativa en el tratamiento de recien nacidos con hipertension pulmonar persistente. Revista Mexicana de Pediatria 2006;73:159-63
   Google Scholar
• Vargas-Origel A, Gomez-Rodriguez G, Aldana-Valenzuela C, et al. The use of sildenafil in persistent pulmonary hypertension of the newborn. Am J Perinatol 2010;27:225-30
   PubMed Web of Science ®Google Scholar
• Juliana AE, Abbad FC. Severe persistent pulmonary hypertension of the newborn in a setting where limited resources exclude the use of inhaled nitric oxide: successful treatment with sildenafil. Eur J Pediatr 2005;164:626-9
   PubMed Web of Science ®Google Scholar
• Engelbrecht AL. Sildenafil in the management of neonates with PPHN: a rural regional hospital experience. S Afr J Child Health 2008;2:166-9
   Google Scholar
• A study to evaluate safety and efficacy of IV sildenafil in the treatment of neonates with persistent pulmonary hypertension of the newborn. Available from: http://clinicaltrials.gov/show/NCT01720524. [Last accessed 3 September 2014]
   Google Scholar
• Ahsman MJ, Witjes BC, Wildschut ED, et al. Sildenafil exposure in neonates with pulmonary hypertension after administration via a nasogastric tube. Arch Dis Child Fetal Neonatal Ed 2010;95:F109-14
   PubMed Web of Science ®Google Scholar
• Marsh CS, Marden B, Newsom R. Severe retinopathy of prematurity (ROP) in a premature baby treated with sildenafil acetate (Viagra) for pulmonary hypertension. Br J Ophthalmol 2004;88:306-7
   PubMed Web of Science ®Google Scholar
• Kehat R, Bonsall DJ, North R, Connors B. Ocular findings of oral sildenafil use in term and near-term neonates. J AAPOS 2010;14:159-62
   PubMed Web of Science ®Google Scholar
• Gaffuri M, Cristofaletti A, Mansoldo C, Biban P. Acute onset of bilateral visual loss during sildenafil therapy in a young infant with congenital heart disease. BMJ Case Rep 2014. [ Epub ahead of print]
   Google Scholar
• Atz AM, Wessel DL. Sildenafil ameliorates effects of inhaled nitric oxide withdrawal. Anesthesiology 1999;91:307-10
   PubMed Web of Science ®Google Scholar
• Kovacikova L, Zahorec M, Nosal M. Sildenafil as a pulmonary vasodilator after repair of congenital heart disease. Bratislavske Lekarske Listy 2007;108:453-4
   Google Scholar
• Maxted AP, Hill A, Davies P. Oral sildenafil as a rescue therapy in presumed acute pulmonary hypertensive crisis. Pediatrics 2013;131:e626-8
   PubMed Web of Science ®Google Scholar
• Atz AM, Lefler AK, Fairbrother DL, et al. Sildenafil augments the effect of inhaled nitric oxide for postoperative pulmonary hypertensive crises. J Thorac Cardiovasc Surg 2002;124:628-9
   PubMed Web of Science ®Google Scholar
• Stocker C, Penny DJ, Brizard CP, et al. Intravenous sildenafil and inhaled nitric oxide: a randomised trial in infants after cardiac surgery. Intensive Care Med 2003;29:1996-2003
   PubMed Web of Science ®Google Scholar
• Peiravian F, Amirghofran AA, Borzouee M, et al. Oral sildenafil to control pulmonary hypertension after congenital heart surgery. Asian Cardiovasc Thorac Ann 2007;15:113-17
   PubMedGoogle Scholar
• Namachivayam P, Theilen U, Butt WW, et al. Sildenafil prevents rebound pulmonary hypertension after withdrawal of nitric oxide in children. Am J Respir Crit Care Med 2006;174:1042-7
   PubMed Web of Science ®Google Scholar
• Humpl T, Reyes JT, Erickson S, et al. Sildenafil therapy for neonatal and childhood pulmonary hypertensive vascular disease. Cardiol Young 2011;21:187-93
   PubMed Web of Science ®Google Scholar
• Schulze-Neick I, Hartenstein P, Li J, et al. Intravenous sildenafil is a potent pulmonary vasodilator in children with congenital heart disease. Circulation 2003;108:167-73
   Google Scholar
• Ajami GH, Borzoee M, Radvar M, Amoozgar H. Comparison of the effectiveness of oral sildenafil versus oxygen administration as a test for feasibility of operation for patients with secondary pulmonary arterial hypertension. Pediatr Cardiol 2008;29:552-5
   Google Scholar
• Apitz C, Reyes JT, Holtby H, et al. Pharmacokinetic and hemodynamic responses to oral sildenafil during invasive testing in children with pulmonary hypertension. J Am Coll Cardiol 2010;55:1456-62
   PubMed Web of Science ®Google Scholar
• Raja SG, Danton MD, MacArthur KJ, Pollock JC. Effects of escalating doses of sildenafil on hemodynamics and gas exchange in children with pulmonary hypertension and congenital cardiac defects. J Cardiothorac Vasc Anesthesia 2007;21:203-7
   PubMed Web of Science ®Google Scholar
• Nemoto S, Sasaki T, Ozawa H, et al. Oral sildenafil for persistent pulmonary hypertension early after congenital cardiac surgery in children. Eur J Cardiothorac Surg 2010;38:71-7
   Google Scholar
• Uhm JY, Jhang WK, Park JJ, et al. Postoperative use of oral sildenafil in pediatric patients with congenital heart disease. Pediatr Cardiol 2010;31:515-20
   Google Scholar
• Lee JE, Hillier SC, Knoderer CA. Use of sildenafil to facilitate weaning from inhaled nitric oxide in children with pulmonary hypertension following surgery for congenital heart disease. J Intensive Care Med 2008;23:329-34
   PubMedGoogle Scholar
• Palma G, Giordano R, Russolillo V, et al. Sildenafil therapy for pulmonary hypertension before and after pediatric congenital heart surgery. Texas Heart Inst J 2011;38:238-42
   PubMed Web of Science ®Google Scholar
• Peiravian F, Amirghofran AA, Ghamsari H, Emaminia A. Additive effect of phosphodiesterase inhibitors in control of pulmonary hypertension after congenital cardiac surgery in children. Iran J Pediatr 2013;23:19-26
   Google Scholar
• Beghetti M. Fontan and the pulmonary circulation: a potential role for new pulmonary hypertension therapies. Heart 2010;96:911-16
   Google Scholar
• Swetman GL, Berk DR, Vasanawala SS, et al. Sildenafil for severe lymphatic malformations. N Engl J Med 2012;366:384-6
   PubMed Web of Science ®Google Scholar
• Mourani PM, Sontag MK, Ivy DD, Abman SH. Effects of long-term sildenafil treatment for pulmonary hypertension in infants with chronic lung disease. J Pediatr 2009;154:379-84. e1-2
   PubMed Web of Science ®Google Scholar
• del Cerro MJ, Sabate Rotes A, Carton A, et al. Pulmonary hypertension in bronchopulmonary dysplasia: clinical findings, cardiovascular anomalies and outcomes. Pediatr Pulmonol 2014;49:49-59
   PubMed Web of Science ®Google Scholar
• Nyp M, Sandritter T, Poppinga N, et al. Sildenafil citrate, bronchopulmonary dysplasia and disordered pulmonary gas exchange: any benefits? J Perinatol 2012;32:64-9
   PubMed Web of Science ®Google Scholar
• Caputo S, Furcolo G, Rabuano R, et al. Severe pulmonary arterial hypertension in a very premature baby with bronchopulmonary dysplasia: normalization with long-term sildenafil. J Cardiovasc Med (Hagerstown) 2010;11:704-6
   Google Scholar
• Gurakan B, Kayiran P, Ozturk N, et al. Therapeutic combination of sildenafil and iloprost in a preterm neonate with pulmonary hypertension. Pediatr Pulmonol 2011;46:617-20
   PubMed Web of Science ®Google Scholar
• Hwang SK, O YC, Kim NS, et al. Use of inhaled iloprost in an infant with bronchopulmonary dysplasia and pulmonary artery hypertension. Korean Circ J 2009;39:343-5
   Google Scholar
• Hon KL, Cheung KL, Siu KL, et al. Oral sildenafil for treatment of severe pulmonary hypertension in an infant. Biol Neonate 2005;88:109-12
   PubMedGoogle Scholar
• Krishnan U, Krishnan S, Gewitz M. Treatment of pulmonary hypertension in children with chronic lung disease with newer oral therapies. Pediatr Cardiol 2008;29:1082-6
   PubMed Web of Science ®Google Scholar
• Noori S, Friedlich P, Wong P, et al. Cardiovascular effects of sildenafil in neonates and infants with congenital diaphragmatic hernia and pulmonary hypertension. Neonatology 2007;91:92-100
   PubMed Web of Science ®Google Scholar
• De Luca D, Zecca E, Vento G, et al. Transient effect of epoprostenol and sildenafil combined with iNO for pulmonary hypertension in congenital diaphragmatic hernia. Paediatr Anaesth 2006;16:597-8
   Google Scholar
• Harris K. Extralobar sequestration with congenital diaphragmatic hernia: a complicated case study. Neonatal Netw 2004;23:7-24
   PubMedGoogle Scholar
• Filan PM, McDougall PN, Shekerdemian LS. Combination pharmacotherapy for severe neonatal pulmonary hypertension. J Paediatr Child Health 2006;42:219-20
   PubMed Web of Science ®Google Scholar
• Keller RL, Hamrick SE, Kitterman JA, et al. Treatment of rebound and chronic pulmonary hypertension with oral sildenafil in an infant with congenital diaphragmatic hernia. Pediatr Crit Care Med 2004;5:184-7
   Google Scholar
• Keller RL, Moore P, Teitel D, et al. Abnormal vascular tone in infants and children with lung hypoplasia: findings from cardiac catheterization and the response to chronic therapy. Pediatr Crit Care Med 2006;7:589-94
   Google Scholar
• Rocha GM, Bianchi RF, Severo M, et al. Congenital diaphragmatic hernia - the neonatal period (part I). Eur J Pediatr Surg 2008;18:219-23
   Google Scholar
• Hunter L, Richens T, Davis C, et al. Sildenafil use in congenital diaphragmatic hernia. Arch Dis Child Fetal Neonatal Ed 2009;94:F467
   Google Scholar
• Menon SC, Tani LY, Weng HY, et al. Clinical characteristics and outcomes of patients with cardiac defects and congenital diaphragmatic hernia. J Pediatr 2012;162:114-19
   Google Scholar
• Ciliberti P, Schulze-Neick I, Giardini A. Modulation of pulmonary vascular resistance as a target for therapeutic interventions in Fontan patients: focus on phosphodiesterase inhibitors. Future Cardiol 2012;8:271-84
   Google Scholar
• Gewillig M. The Fontan circulation. Heart 2005;91:839-46
   PubMed Web of Science ®Google Scholar
• Naranjo-Ugalde AM, Rivera-Ladino KM, Consuegra-Chuarey MT, et al. Sildenafil before total cavopulmonary connection. Rev Esp Cardiol 2013;66:71
   Google Scholar
• Bialkowski J, Szkutnik M, Fiszer R. Midterm results of sildenafil therapy in two complex patients with elevated pulmonary artery pressure after cavopulmonary connection. Postepy Kardiol Interwencyjnej 2013;9:262-4
   Google Scholar
• Goldberg DJ, French B, McBride MG, et al. Impact of oral sildenafil on exercise performance in children and young adults after the Fontan operation: a randomized, double-blind, placebo-controlled, crossover trial. Circulation 2011;123:1185-93
   PubMed Web of Science ®Google Scholar
• Goldberg DJ, French B, Szwast AL, et al. Impact of sildenafil on echocardiographic indices of myocardial performance after the Fontan operation. Pediatr Cardiol 2012;33:689-96
   Google Scholar
• Giardini A, Balducci A, Specchia S, et al. Effect of sildenafil on haemodynamic response to exercise and exercise capacity in Fontan patients. Eur Heart J 2008;29:1681-7
   Google Scholar
• Hill KD, Tunks RD, Barker PC, et al. Sildenafil exposure and hemodynamic effect after stage II single-ventricle surgery. Pediatr Crit Care Med 2013;14:593-600
   Google Scholar
• Tunks RD, Barker PC, Benjamin DK Jr, et al. Sildenafil exposure and hemodynamic effect after Fontan surgery. Pediatr Crit Care Med 2014;15:28-34
   Google Scholar
• Van De Bruaene A, La Gerche A, Claessen G, et al. Sildenafil improves exercise hemodynamics in Fontan patients. Circ Cardiovasc Imaging 2014;7:265-73
   Google Scholar
• Morchi GS, Ivy DD, Duster MC, et al. Sildenafil increases systemic saturation and reduces pulmonary artery pressure in patients with failing Fontan physiology. Congenital Heart Dis 2009;4:107-11
   PubMed Web of Science ®Google Scholar
• Celik L, Papakostas K, Lentschig M, et al. Lost unilateral capillary perfusion during nonpulsatile pulmonary circulation: successful recovery by oral sildenafil. Pediatr Cardiol 2012;33:370-4
   Google Scholar
• Haseyama K, Satomi G, Yasukochi S, et al. Pulmonary vasodilation therapy with sildenafil citrate in a patient with plastic bronchitis after the Fontan procedure for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 2006;132:1232-3
   Google Scholar
• Do P, Randhawa I, Chin T, et al. Successful management of plastic bronchitis in a child post Fontan: case report and literature review. Lung 2012;190:463-8
   Google Scholar
• Uzun O, Wong JK, Bhole V, Stumper O. Resolution of protein-losing enteropathy and normalization of mesenteric Doppler flow with sildenafil after Fontan. Ann Thorac Surg 2006;82:e39-40
   Google Scholar
• Reinhardt Z, Uzun O, Bhole V, et al. Sildenafil in the management of the failing Fontan circulation. Cardiol Young 2010;20:522-5
   Google Scholar
• Carls A, Winter J, Enderle Y, et al. Substantially increased sildenafil bioavailability after sublingual administration in children with congenital heart disease: two case reports. J Med Case Rep 2014;8:171
   Google Scholar
• Bhutta AT, Beam B, Prodhan P. Temporal trends in use of sildenafil among pediatric patients. Am J Respir Crit Care Med 2013;188:1269-71
   Google Scholar
• Samiee-Zafarghandy S, Smith PB, van den Anker JN. Safety of sildenafil in infants. Pediatr Crit Care Med 2014;15:362-8
   PubMed Web of Science ®Google Scholar
• Maxey DM, Ivy DD, Ogawa MT, Feinstein JA. 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
   PubMed Web of Science ®Google Scholar
• A study of tadalafil in pediatric participants with pulmonary arterial hypertension (PAH). Available from: http://clinicaltrials.gov/show/NCT01824290. [Last accessed 3 September 2014]
   Google Scholar
• Manes A, Palazzini M, Leci E, et al. Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups. Eur Heart J 2014;35:716-24
   PubMed Web of Science ®Google Scholar
• Shabanian R, Shahbaznejad L, Razaghian A, et al. Sildenafil and ventriculo-arterial coupling in Fontan-palliated patients: a noninvasive echocardiographic assessment. Pediatr Cardiol 2013;34:129-34
   Google Scholar
• Giordano R, Palma G, Poli V, et al. First experience with sildenafil after Fontan operation: short-term outcomes. J Cardiovasc Med (Hagerstown) 2013. [ Epub ahead of print]
   Google Scholar
• Jackson KW, Butts RJ, Svenson AJ, et al. Response to a single dose of sildenafil in single-ventricle patients: an echocardiographic evaluation. Pediatr Cardiol 2013;34:1739-42
   Google Scholar
• Hager A, Weber R, Muller J, Hess J. Predictors of sildenafil effects on exercise capacity in adolescents and adults with Fontan circulation. Clin Res Cardiol 2014;103:641-6
   Google Scholar