Implications of the U.S. Food and Drug Administration warning against the use of sildenafil for the treatment of pediatric pulmonary hypertension

American Journal of Respiratory and Critical Care Medicine

Volumn 187, Issue 6, 2013, Pages 572-575

  Abman, Steven H ^a,l   Kinsella, John P ^a   Rosenzweig, Erika B ^b   Krishnan, Usha ^b   Kulik, Thomas ^c   Mullen, Mary ^c   Wessel, David L ^d   Steinhorn, Robin ^e   Adatia, Ian ^f   Hanna, Brian ^g   Feinstein, Jeffrey ^h   Fineman, Jeffrey ⁱ   Raj, Usha ^j   Humpl, Tilman ^k  

^a Children's Hospital Colorado   (United States)

^b Och Spine at New York Presbyterian Hospitals   (United States)

^c BOSTON CHILDREN S HOSPITAL   (United States)

^d CHILDREN'S NATIONAL MEDICAL CENTER   (United States)

^e NORTHWESTERN UNIVERSITY   (United States)

^f UNIVERSITY OF ALBERTA   (Canada)

^g UNIVERSITY OF PENNSYLVANIA   (United States)

^h STANFORD UNIVERSITY   (United States)

ⁱ UNIVERSITY OF CALIFORNIA   (United States)

^j UNIVERSITY OF ILLINOIS AT CHICAGO   (United States)

^k HOSPITAL FOR SICK CHILDREN   (Canada)

^l UNIVERSITY OF COLORADO SCHOOL OF MEDICINE   (United States)

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Author keywords

Pediatric pulmonary hypertension; Pulmonary hypertension therapy; Sildenafil

Indexed keywords

PROSTACYCLIN; SILDENAFIL;

DISEASE COURSE; DISEASE SEVERITY; DRUG APPROVAL; DRUG EFFICACY; DRUG MEGADOSE; DRUG SAFETY; DRUG SURVEILLANCE PROGRAM; EXERCISE; FOLLOW UP; FOOD AND DRUG ADMINISTRATION; HUMAN; MONOTHERAPY; MORTALITY; OVERALL SURVIVAL; PATIENT MONITORING; PRIORITY JOURNAL; PULMONARY HYPERTENSION; REVIEW; RISK FACTOR; TREATMENT DURATION;

DISEASE PROGRESSION; DRUG MONITORING; HUMANS; HYPERTENSION, PULMONARY; PIPERAZINES; PRODUCT SURVEILLANCE, POSTMARKETING; PURINES; SULFONES; UNITED STATES; UNITED STATES FOOD AND DRUG ADMINISTRATION; VASODILATOR AGENTS;

EID: 84875156577     PISSN: 1073449X     EISSN: 15354970     Source Type: Journal    
DOI: 10.1164/rccm.201210-1928PP     Document Type: Review

References (25)

1. Haworth SG, Hislop AA. Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006. Heart 2009;95:312-317.
2. Berger RM, Beghetti M, Humpl T, Raskob GE, Ivy DD, Jing ZC, Bonnet D, Schulze-Neick I, Barst RJ. Clinical features of paediatric pulmonary hypertension: a registry study. Lancet 2012;379:537-546.
3. NINOS Study Group. Inhaled NO in full-term and nearly full term infants with hypoxic respiratory failure. N Engl J Med 1997;336:597-604.
4. Clarke RH, Kueser TJ,Walker MW, Southgate WM, Huckaby JL, Perez JA, Roy BJ, Keszler M, Kinsella JP. Low dose NO therapy for PPHN. N Engl J Med 2000;342:469-474.
5. Roberts JD Jr, Fineman JR, Morin FC, Shaul PW, Rimer S, Schreiber MD, Polin RA, Zwass MS, Zayek MM, Gross I, et al.; Inhaled Nitric Oxide Study Group. Inhaled NO and PPHN. N Engl J Med 1997;336: 605-610.
6. Barst RJ, Ertel SI, Beghetti M, Ivy DD. Pulmonary arterial hypertension: a comparison between children and adults. Eur Respir J 2011;37: 665-677.
7. Abman SH, Raj U. Towards improving the care of children with pulmonary hypertension: rationale for developing a pediatric pulmonary hypertension network. Prog Pediatr Cardiol 2009;27:3-6.
8. Robbins I, Moore T, Blaisdell C, Abman SH. NIHWorkshop: improving pulmonary vascular outcomes. Circulation 2012;125:2165-2170.
9. Gao Y, Raj U. Regulation of the pulmonary circulation in the fetus and newborn. Physiol Rev 2010;90:1291-1335.
10. Del Cerro MJ, Abman S, Diaz G, Freudenthal AH, Freudenthal F, Harikrishna S, Haworth DG, Ivy D, Lopez A, Raj U, et al. Pediatric pulmonary hypertensive vascular disease: suggestions for a novel pediatric classification. Report from the PVRI Pediatric Taskforce, Panama 2011. Pulm Circ 2011;1:286-298.
11. Lammers AE, Adatia I, CerroMJ, Diaz G, Freudenthal AH, Freudenthal F, Harikrishnan S, Ivy D, Lopes AA, Raj JU, et al. Functional classification of pulmonary hypertension in children: report from the PVRI pediatric taskforce, Panama 2011. Pulm Circ. 2011;1: 280-285.
12. Li AM, Yin J, Au JT, So HK, Tsang T, Wong E, Fok TF, Ng PC. Standard reference for the six-minute-walk test in healthy children aged 7 to 16 years. Am J Respir Crit Care Med 2007;176:174-180.
13. Lammers AE, Diller GP, Odendaal D, Tailor S, Derrick G, Haworth SG. Comparison of 6-min walk test distance and cardiopulmonary exercise test performance in children with pulmonary hypertension. Arch Dis Child 2011;96:141-147.
14. Humpl T, Reyes JF, Holtby H, Stephens D, Adatia I. Beneficial effects of oral sildenafil therapy on childhood pulmonary arterial hypertension: 12 month clinical trial of a single drug, open label, pilot study. Circulation 2005;21:3274-3280.
15. 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;154:379-384.
16. Steinhorn RH, Kinsella JP, Pierce C, Butrous G, Dilleen M, Oakes M, Wessel DL. Intravenous sildenafil in the treatment of neonates with persistent pulmonary hypertension. J Pediatr 2009;155:841-847.
17. Fraiss A, Butrous G, Taylor MB, Oakes M, Dilleen M, Wessel DL. Intravenous sildenafil for postoperative pulmonary hypertension for children with congenital heart disease. Intensive Care Med 2011;37:502-509.
18. Baquero H, Soliz A, Neira F, Venegas ME, Sola A. Oral sildenafil in infants with persistent pulmonary hypertension of the newborn: a pilot randomized blinded trial study. Pediatrics 2006;117:1077-1083.
19. Barst RJ, Ivy DD, Gaitan G, Szatmari A, Rudzinski A, Gardia AE, Sastry BKS, Pulido T, Layton GR, Serdaravic-Pehar M, et al. A randomized, double blind placebo-controlled, dose-ranging study of oral sildenafil citrate in treatment-naïve children with pulmonary arterial hypertension. Circulation 2012;125:324-334.
20. Barst RJ, Layton GR, Konourina I, Richardson H, Beghetti M, Ivy DD. STARTS-2: long-term survival with oral sildenafil monotherapy in treatment-naive patients with pediatric pulmonary arterial hypertension [abstract]. Eur Heart J 2012;33:979.
21. U.S. Food and Drug Administration. Revatio (sildenafil): drug safety communication - recommendation against use in children. Silver Spring, MD: U.S. Food and Drug Administration [updated 2012 Aug 30; accessed 2013 Feb 20]. Available from: http://www.fda.gov/Safety/MedWatch/SafetyInformation/ SafetyAlertsforHumanMedicalProducts/ucm317743.htm
22. van Loon RL, Roofthooft MT, Delhaas T, van Osch-Gevers M, ten Harkel AD, Strengers JL, Backx A, Hillege HL, Berger RM. Outcome of pediatric patients with pulmonary arterial hypertension in the era of new medical therapies. Am J Cardiol 2010;106:117-124.
23. Ivy DD, Rosenzweig EB, Lemarie JC, Brand M, Rosenberg D, Barst RJ. Long-term outcomes in children with pulmonary arterial hypertension treated with bosentan in real-world clinical settings. Am J Cardiol 2010; 106:1332-1338.
24. Barst RJ, McGoon MD, Elliott CG, Foreman AJ, Miller DP, Ivy DD. Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management. Circulation 2012;125:113-122.
25. European Medicines Agency. Assessment report for Revatio. International non-proprietary name: sildenafil. Procedure No. EMEA/H/C/000638/II/0028. London, UK: European Medicines Agency; 2011 [accessed 2013 Feb 20]. Available from: http://www.ema.europa.eu/docs/en-GB/document-library/EPAR-Assessment-Report- Variation/human/000638/WC500107804.pdf