Abstract
INTRODUCTION Persistent pulmonary hypertension of the newborn (PPHN) remains an important cause of mortality and morbidity in the term neonate. The severity of the illness and the outcome are related to the underlying cause. Therapies include inhaled nitric oxide (iNO), intravenous prostacyclin and sildenafil. We report the successful treatment of a neonate suffering from PPHN secondary to pulmonary hypertension caused by a skeletal dysplasia.
CASE REPORT the female infant was delivered at 37 weeks gestation with antenatal ultrasound findings a skeletal dysplasia. The diagnosis of spondyloepiphyseal dysplasia congenita was made based on the skeletal survey and clinical picture. She required significant ventilatory support and up to 100% oxygen because of PPHN. iNO resulted in a decrease in oxygen requirement to 80%. On day 36 oral sildenafil was commenced at a dose of 0.5mg/kg every 6 hours and increased over the next week to 2mg/kg every 6 hours. iNO was successfully weaned and ceased 4 days after commencing sildenafil. Within a week of commencing sildenafil the infant required less ventilation with a decrease in the oxygen requirement from 80% to 40%. She was successfully extubated to CPAP 4 weeks later. She has subsequently been discharged home on nasopharyngeal CPAP facilitated by home carers.
DISCUSSION PPHN occurs in 1–2/1000 live births PPHN is associated with significant mortality of up to 20%. Sildenafil is a specific PDE-5 inhibitor and is mostly known for its use in the treatment of erectile dysfunction. Preliminary findings suggest that sildenafil is effective in the long-term therapy of pulmonary hypertension and is particularly effective in reducing rebound hypertension after discontinuation of NO in patients with arterial pulmonary hypertension. Preliminary but limited data suggests that there might be a role for sildenafil in the treatment of PPHN. Randomised-controlled trials are required to determine its efficacy and safety.
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Kecskes, Z., Kent, A. & Reynolds, G. 196 Successful Treatment of Pulmonary Hypertension with Sildenafil in a Neonate with Spondyloepiphyseal Dysplasia Congenita. Pediatr Res 58, 388 (2005). https://doi.org/10.1203/00006450-200508000-00225
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DOI: https://doi.org/10.1203/00006450-200508000-00225
