• Ali Shabbir Hussain The Aga khan university hospital Karschi, Pakistan http://orcid.org/0000-0002-3335-8768
  • Rehan Ali Associate professor The Aga khan university hospital
  • Shakeel Ahmed The aga khan university hospital
  • Farah Naz aga khan university
  • Anila Haroon memon medical complex


Background: The prevalence of PPHN has been estimated at 1.9 per 1000 live births. After the discovery of iNO’s, its efficacy and benefit in PPHN is well established. Even in the best of centers equipped with iNo and ECMO the mortality is around 20%. Also, iNO is expensive and difficult to administer and monitor which makes it difficult choice in our part of the world. Furthermore About 40% of patients do not respond or have rebound pulmonary hypertension after discontinuation. Owing to these reasons, other treatment modalities like phosphodiesterase inhibitors such as Sildenafil need to be evaluated. Methods: We report a retrospective case series of eighteen patients with PPHN admitted in NICU and treated with oral sildenafil. Results: Three (17%) babies had mild, 5 (28%) moderate and 10 (55%) severe PPHN based on echocardiography. Sildenafil was started on all patients on a mean of 1.67 days and stopped on mean 12.6 days. Initial fio2 was 100%, which after starting sildenafil decreased gradually to 40% on mean 10 days. Average length of stay in NICU was 13 days. Twelve (67%) patients survived whereas 6 (33%) expired (Figure 2). No improvement in oxygen Index after 36 hours (p<0.05) was the independent predicting risk factor for PPHN related mortality in the expired patients. Conclusion: Oral sildenafil can be a used in conjunction with other treatment modalities for PPHN especially in resource limited settings. However further studies regarding its comparative efficacy need to be done.Keywords: PPHN; Sildenafil; Neonate; Pulmonary hypertension; Management; Neonatal ICU

Author Biography

Ali Shabbir Hussain, The Aga khan university hospital Karschi, Pakistan

Senior instructor and Consultant neonatologist


Porta NF, Steinhorn RH. Pulmonary vasodilator therapy in the NICU: inhaled nitric oxide, sildenafil, and other pulmonary vasodilating agents. Clin Perinatol 2012;39(1):149–64.

Simiyu DE, Okello C, Nyakundi EG, Tawakal AH. Sildenafil in management of persistent pulmonary hypertension of the newborn: report of two cases. East Afr Med J 2006;83(6):337–40.

Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med 2004;351(14):1425–36.

Travadi JN, Patole SK. Phosphodiesterase inhibitors for persistent pulmonary hypertension of the newborn: a review. Pediatr Pulmonol 2003;36(6):529–35.

Deruelle P, Grover TR, Abman SH. Pulmonary vascular effects of nitric oxide-cGMP augmentation in a model of chronic pulmonary hypertension in fetal and neonatal sheep. Am J Physiol Lung Cell Mol Physiol 2005;289(5):L798–806.

Dhillon R. The management of neonatal pulmonary hypertension. Arch Dis Child Fetal Neonatal Ed 2012;97(3):F223–8.

Castillo J, Herrera R, Concha E, Valencia P, Torbati D, Soliz A. 385 Oral Sildenafil Treatment as an Alternative to Inhaled no Therapy for Persistent Pulmonary Hypertension of the Newborn. Pediatr Res 2005;58(2):420.

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(10):626–9.

Michelakis E, Tymchak W, Lien D, Webster L, Hashimoto K, Archer S. Oral sildenafil is an effective and specific pulmonary vasodilator in patients with pulmonary arterial hypertension: comparison with inhalwe nitric oxide. Circulation 2002;105(20):2398–403.

Shah PS, Ohlsson A. Sildenafil for pulmonary hypertension in neonates. Cochrane Database Syst Rev 2011(8):CD005494.

Baquero H, Soliz A, Neira F, Venega ME, Sola A. Oral sildenafil in infants with persistent pulmonary hypertension of the newborn: a pilot randomized blindedstudy. Pediatrics 2006;117(4):1077–83.

Abrams D, Schulze-Neick I, Magee AG. Sildenafil as a selective pulmonary vasodilator in childhood primary pulmonary hypertension. Heart 2000;84(2):E4.

Huddleston AJ, Knoderer CA, Morris JL, Ebenroth ES. Sildenafil for the treatment of pulmonary hypertension in pediatric patients. Pediatr Cardiol 2009;30(7):871–82.

Shekerdemian LS, Ravn HB, Penny DJ. Intravenous sildenafil lowers pulmonary vascular resistance in a model of neonatal pulmonary hypertension. Am J Respir Crit Care Med 2002;165(8):1098–102.



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