Use of Oral Sildenafil in the Management of Persistent Pulmonary Hypertension of the Newborn: A Case Report and Review of Literature.
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Keywords
Case Report, Cyanosis, Neonate, Nigeria, Persistent Pulmonary Hypertension of the Newborn, Resource-Limited Setting, Sildenafil
Abstract
Persistent pulmonary hypertension of the newborn (PPHN) arises from failure of the normal circulatory transition that occurs postnatally and is characterised by increased pulmonary vascular resistance, leading to significant respiratory distress and hypoxaemia. Oral sildenafil administration is a potential alternative treatment for PPHN, especially when inhaled nitric oxide is unavailable.A 72-hour-old term male neonate was referred on account of respiratory distress, and cyanosis was noticed at 30 minutes of life with RR of 76 bpm and SPO2 of 57% in room air. The baby was pale, cyanosed, icteric, and febrile (39.5°C). An initial assessment of Severe NNJ 20 to EONNS with background cyanotic congenital heart disease was made. An echocardiogram with colour Doppler interrogation confirmed the diagnosis of Persistent pulmonary hypertension of the newborn. The baby was commenced on compounded oral sildenafil @ 2mg/kg/dose 6 hourly in addition to oxygen therapy. Baby improved and was subsequently discharged home on oral sildenafil, which was discontinued after a normal repeat ECHO Scan done at 2 months of life. Oral compounded sildenafil was found to be a useful alternative in managing PPHN.
References
2. Martinho S, Adão R, Leite-Moreira AF, Brás-Silva C. Persistent Pulmonary Hypertension of the Newborn: Pathophysiological Mechanisms and Novel Therapeutic Approaches. Front Pediatr. 2020; 8:342 Doi: 10.3389/fped.2020.00342
3. Montasser M, Patel N. Pulmonary hypertension in newborn infants: pathophysiology, clinical assessment and management. Paediatrics and Child Health 2021;31(1):32–37. Doi: 10.1016/j.paed.2020.10.005
4. Singh Y, Lakshminrusimha S. Pathophysiology and Management of Persistent Pulmonary Hypertension of the Newborn. Clinics in perinatology. 2021;48 3:595–618. Doi: 10.1016/j.clp.2021.05.009
5. Neto J de RBJ, Mapurunga ÂR, Belchior AG, Costa FR, Boscov VT. Use of sildenafil for the treatment of neonatal persistent pulmonary hypertension. Research, Society and Development 2024;13(1): e14713144910–e14713144910. Doi:10.33448/rsd-v13i1.44910
6. Abman SH, Hansmann G, Archer SL, Ivy DD, Adatia I, Chung WK, et al. Pediatric Pulmonary Hypertension: Guidelines from the American Heart Association and American Thoracic Society. Circulation. 2015;132(21):2037-99 Doi:10.1161/CIR.0000000000000329.
7. Dillard J, Perez M, Chen B. Therapies that enhance pulmonary vascular NO-signaling in the neonate. Nitric Oxide. 2020; 95:45–54. Doi: 10.1016/j.niox.2019.12.003
8. El-Ghandour M, Hammad B, Ghanem M, Antonios MA. Efficacy of Milrinone Plus Sildenafil in the Treatment of Neonates with Persistent Pulmonary Hypertension in Resource-Limited Settings: Results of a Randomized, Double-Blind Trial. Paediatr Drugs. 2020; 22:685–93. Doi:10.1007/s40272-020-00412-4
9. Lavie-Nevo K, Harris KC, Ting JY. Use of sildenafil in an infant with persistent pulmonary hypertension secondary to lung and renal hypoplasia - a case report. BMC Pediatr. 2019;19(1):416. Doi:10.1186/s12887-019-1801-3
10. Schroeder L, Monno P, Strizek B, Dresbach T, Mueller A, Kipfmueller F. Intravenous sildenafil for treatment of early pulmonary hypertension in preterm infants. Sci Rep. 2023;13(1):8405. Doi:10.1038/s41598-023-35387-y
11. AlSalman F, Howlett M, Breatnach C, Kelly H, O'Brien F. Supporting the use of sildenafil infusions in paediatric and neonatal intensive care - A compatibility study. Eur J Pharm Biopharm. 2020; 151:153-161. Doi: 10.1016/j.ejpb.2020.04.008
12. Kamran A, Rafiq N, Khalid A, Amin F, Kumari V, Shaikh AS, et al. Effectiveness of oral sildenafil for neonates with persistent pulmonary hypertension of newborn (PPHN): a prospective study in a tertiary care hospital. The Journal of Maternal-Fetal & Neonatal Medicine. 2021; 35:6787–6793. Doi:10.1080/14767058.2021.1923003
13. Gupta A, Kumar M, Anand T. Role of oral sildenafil in neonates with persistent pulmonary hypertension of newborn. International Journal of Contemporary Paediatrics. 2019;6(5): 2222–2224. Doi:10.18203/2349-3291.ijcp20193757.
14. Kelly LE, Ohlsson A, Shah PS. Sildenafil for pulmonary hypertension in neonates. Cochrane database of Syst Rev. 2017;8(8):CD005494. Doi: 10.1002/14651858.CD005494.pub4.
15. Daniyan O, Ezeanosike O, Obu D, Ujunwa F. Use of off-label compounded oral sildenafil in the management of persistent pulmonary hypertension of the newborn: A case report. Journal of Cardiovascular and Thoracic Research. 2022; 14:141–143. Doi:10.34172/jcvtr.2022.03
Ogugua AI, Maduabuchi C, Amauche UF. Non-cardiac central cyanosis in the newborn: A case report of persistent pulmonary hypertension of newborn (PPHN) and review of literature. Nigerian Journal of Paediatrics 2022;49(3):276-278 Doi:10.4314/njp.v49i3.9
17. Monazea E, Khattab S, Ebrahim A, Mohamed L, Ahmed H. The outcome of oral sildenafil therapy on persistent pulmonary hypertension of the newborn with respiratory distress. The Scientific Journal of Al-Azhar Medical Faculty, Girls. 2020; 4:535–539. Doi: 10.4103/sjamf.sjamf_72_20
18. Vorhies EE, Ivy DD. Drug Treatment of Pulmonary Hypertension in Children. Paediatr Drugs 2014;16(1):43–65. Doi:10.1007/s40272-013-0052-2
19. Al-lawama M, Badran E, Abdelfattah AS, Jaddalla R, Almahameed HA, Al-Ammouri I. Outcomes of newborn infants with pulmonary hypertension treated with oral sildenafil in a tertiary care center in Amman, Jordan. International Journal of Clinical Paediatrics. 2019;8(2):27–31. Doi:10.14740/ijcp349
20. Naveed M, Rehman F, Shahnawaz K, Zahoor F, Madni B, Imran M. Efficacy of sildenafil in persistent pulmonary hypertension of the newborn. Professional Med J. 2022; 29(4):516-520. Doi:10.29309/TPMJ/2022.29.04.6617.
21. Kallimath A, Deshpande S, Singh P, Garegrat R, Lakshminrusimha S, Maheshwari R, et al. Oral sildenafil versus bosentan for treatment of persistent pulmonary hypertension of the newborn: a randomized controlled trial. BMC Pediatr 2024;24: 698 (2024). Doi:10.1186/s12887-024-05107-0
22. Abounahia FF, Abu-Jarir R, Abounahia MF, Al-Badriyeh D, Abushanab D, Abu-Ghalwa M, et al. Prophylactic Sildenafil in Preterm Infants at Risk of Bronchopulmonary Dysplasia: A Pilot Randomized, Double-Blinded, Placebo-Controlled Trial. Clin Drug Investig. 2019;39(11):1093–1107. Doi:10.1007/s40261-019-00834-0
23. Márquez-González H, Ríos DI, Jean Tron MG, Barajas-Nava LA. Use of sildenafil for pulmonary hypertension in neonates. Bol Med Hosp Infant Mex. 2020;77(4):202–206. Doi:10.24875/BMHIM.20000041
24. Sun L, Wang C, Zhou Y, Sun W, Wang C. Clinical Efficacy and Safety of Different Doses of Sildenafil in the Treatment of Persistent Pulmonary Hypertension of the Newborn: A Network Meta-analysis. Front Pharmacol. 2021; 12:697287. Doi:10.3389/fphar.2021.697287
25. Cohen JL, Nees SN, Valencia GA, Rosenzweig EB, Krishnan US. Sildenafil Use in Children with Pulmonary Hypertension. J Pediatr. 2019; 205:29-34. e1. Doi: 10.1016/j.jpeds.2018.09.067
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