Continuous intravenous sildenafil as an early treatment in neonates with congenital diaphragmatic hernia.

BACKGROUND: Pulmonary hypertension (PH) is an important contributor of morbidity and mortality in infants with congenital diaphragmatic hernia (CDH). Treatment options are limited, but sildenafil might improve oxygenation and PH in neonates with CDH.

OBJECTIVE: Aim of this study is to assess effects of intravenous sildenafil on oxygenation and PH in neonates with CDH.

METHODS: A retrospective chart review was performed in all neonates with CDH born in our institution between September 2012 and December 2014. Indication for sildenafil was an OI > 15, PH > 2/3 systemic pressure, or a difference in pre- and postductal oxygen saturation (≥8%). A sildenafil bolus was administered followed by a maintenance infusion of 1.6 mg/kg/d. Primary outcome was improved oxygenation after starting sildenafil. Patients were compared according to improvement in oxygenation (responder vs non-responder).

RESULTS: A total of 26 of 44 neonates were treated with intravenous sildenafil and in all sildenafil were initiated within the first 24 h of life (median age 3.1 h). Improved oxygenation was observed in 11 infants (42.3%). Among the 15 non-responders (57.6%) ECMO was started in 13 and two infants died without ECMO. Vasopressor support increased significantly during the first hours after commencing sildenafil in responders and non-responders. Echocardiographic indices demonstrated an effect on pulmonary arterial pressure within the first 24 h after starting sildenafil.

CONCLUSIONS: Treatment of neonates with intravenous sildenafil during the first day of life was associated with acute improvement in oxygenation in more than 40% of patients. However, a significant increase in vasopressor support was observed.