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was repeated in 30 minutes with no response. Baby died at 36 hours of age from persistent hypoxemia andcirculatory failure.
Case 2: A full term 3250 grams female newborn was
Oral Sildenafil for PPHN in
born by emergency LSCS for fetal bradycardia. Therewas a history of maternal antepartum hemorrhage one- Neonates: Selection of patients
week prior, that was managed conservatively. Motherhad poor nutritional status with severe anemia (Hb 4.4 Remains a Dilemma?
g%) and was delivered with thick old meconium. Babyrequired direct tracheal suction and IPPV with Apgar scores of 4, 7, 8 at 1, 5 and 10 minutes respectively. At20 minutes of age, baby developed respiratory distress Persistent pulmonary hypertension (PPHN) in neonates and prolonged capillary filling time. Baby was thus, has varied etiologies.1-4 It remains a condition with high referred to us. At 4 hours of age, baby was initiated on mortality. The mortality reported from Western literature mechanical ventilation for desaturations and increasing is 10-15%, which may be significantly more in Indian respiratory distress (Blood gas at this stage showed subcontinent due to non-availability of inhaled nitric hypoxemia PO2 42 mmHg without hypercapnia or oxide and extracorporeal membrane oxygenation.1 acidosis). Sepsis screen, hematocrit (45%) and USG Multiple pharmacological approaches have been cranium was normal. Chest X-ray done was consistent suggested for its management.4 Sildenafil, a phospho- with meconium aspiration syndrome. Baby remained diesterase inhibitor shows promise in our settings due to hypoxemic with maximum ventilation (30/5 rate 65 and ease of availability, administration and low cost.
FiO2 100%). A dose of surfactant was given with no However, it’s off label use is still not allowed due to response. Echocardiography ruled out cyanotic inconsistent responses.5 We share our experience of congenital heart disease but showed suprasystemicpulmonary pressures. Baby was managed with failure to demonstrate any beneficial response with oral hyperventilation, alkalization and vasopressor support with persistent hypoxemia. A rescue dose of oral Case 1: A 1450 grams near term small for gestational
sildenafil was given at a dose of 1.5 mg/kg at 10 hours age, male baby was delivered at a small peripheral of age without any improvement in oxygenation index.
health facility by emergency cesarean section, for Baby died at 16 hours of age due to refractory shock.
severe oligohydramnios and prolonged rupture of We used oral sildenafil as a rescue measure after membranes, was brought in respiratory distress having discussions with the family and verbal consent. We a positive sepsis screen at admission. Chest X-ray discussed in detail about the unavailability of inhaled revealed low volume lungs with generalized opacity nitric oxide and ECMO at other higher centres, and suggesting pulmonary hypoplasia and a small right instability of the babies for transport. In both the babies pneumothorax. Baby was given surfactant at eight oral sildenafil was used once the criteria for hours of age and ventilated on Synchronized extracorporeal membrane oxygenation were met Intermittent Mechanical Ventilation (SIMV) mode with (Oxygenation index > 40). We prepared sildenafil pressure support (Maquet, Servo I, Sweden). He (25 mg) by dissolving in 10 ml distilled water and gave required very high pressures PIP 28/5 rates 60 and 100 the solution through oro-gastric tube. Both the cases FiO2. Sequential X-rays showed an enlarging right represent common scenarios faced by neonatologists in pneumothorax that was managed with chest drainage.
India and other developing countries.6,12 Three major After a transient response, blood gases showed patho-physiological derangements in PPHN have been persistent severe hypoxemia (PO2 25-34 mmHg).
described: underdevelopment of the lung, mal- Echocardiography confirmed PPHN with suprasystemic development of the lung and mal-adaptation of the lung pulmonary pressures (gradient 25 mmHg). Baby was with the latter group showing the best response to started on maximum vasopressor support, hyper- vasodilator therapy.4 A search of electronic databases ventilated and given sodium bicarbonate in vain. Oral (MEDLINE, EMBASE, PsycINFO AND CINAHL) was Sildenafil 0.5 mg/kg was tried at 18 hours of age and done using key words sildenafil and neonates andsildenafil and PPHN. Reports in which sildenafil wasused for PPHN in only in neonatal age group (excluding Department of Pediatrics, Central Hospital and Research children) were retrieved. Case reports in which sildenafil was used for PPHN secondary to congenital heart Correspondence: Dr. Pankaj Garg, B-342, Sarita Vihar,
disease and bronchopulmonary dysplasia were excluded. Thus, only five publications were left, which E-mail: pankajparu18@rediffmail.com included one recent placebo randomized controlled trial Received April 12, 2007; accepted October 24, 2007. enrolling 13 newborns with use of oral sildenafil in seven Journal of The College of Physicians and Surgeons Pakistan 2008, Vol. 18 (2): 132-133 Oral sildenafil for PPHN in neonates: selection dilemma? newborns.5-9 Overall combining all reports, oral and admission, diagnosis), maternal variables, ventilator sildenafil have been used in 12 new borns with PPHN indices and parameters into account.
reporting dramatic responses in 11 babies who survived.
One report highlighted successful use of intravenous REFERENCES
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shown survival in impaired alveolarisation that was Dakshinamurti S. Pathophysiologic mechanisms of persistent pulmonary proven by lung biopsy.8 However, in the first case, we hypertension of the newborn. Pediatr Pulmonol 2005; 39: 492-503.
could not confirm pulmonary hypoplasia and poor Oishi P, Fineman JR. Pharmacologic therapy for persistent response may be due to slightly delayed and low dose pulmonary hypertension of the newborn: as “poly” as the of sildenafil use. Some studies have suggested repeat disease itself. Pediatr Crit Care Med 2004; 5:94-6.
dose of sildenafil at six hours, but was not used because Baquero H, Soliz A, Neira F, Venegas ME, Sola A. Oral sildenafil the perfusion had became markedly deteriorated and with persistent pulmonary hypertension of the newborn: a pilot second oral dose of drug would be futile. Baquero has randomized blinded study. Pediatrics 2006; 117: 1077-83.
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Juliana AE, Abbad FC. Severe persistent pulmonaryhypertension of the newborn in a setting where limited Table I: Ventilator indices pre and *post sildenafil
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*Post indices are 30 minutes after oral administration of sildenafil Sildenafil in the treatment of pulmonary hypertension. Ann Pediatr
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subcontinent. A clinical prediction rule needs to be 12. Patole S, Travadi J. Sildenafil for "blue babies". Ethics, developed, which includes neonatal variables (age, sex, conscience, and science have to be balanced against limited weight, and gestational age, condition of baby at birth resources. BMJ 2002; 325:1174.
Journal of The College of Physicians and Surgeons Pakistan 2008, Vol. 18 (2): 132-133

Source: http://jcpsp.pk/archive/2008/Feb2008/21.pdf