Pulmonary hypertension (PH) is the most important complication of congenital diaphragmatic hernia (CDH) and still has a high mortality rate. The aim of this study was to evaluate the effectiveness of inhaled nitric oxide therapy in PH due to CDH.

Hospital records of children who had undergone inhaled nitric oxide therapy for PH due to CDH between June 2009 and December 2011 were reviewed.

Twenty-nine patients had a diagnosis of CDH at the time of study, and eight of these patients underwent nitric oxide therapy because of failure of conventional ventilation techniques, which was successful in five of these patients. Patients who had a good overall outcome of nitric oxide therapy experienced rapid improvement (pretreatment, mean PaO₂ = 44.8 mmHg; after the first hour of therapy, mean PaO₂ = 96.8 mmHg), whereas patients with no response did not have a similar course (pretreatment, PaO₂ = 37 mmHg; after the first hour, PaO₂ = 54.6 mmHg).

Inhaled nitric oxide therapy seems to increase survival in PH due to CDH. No predictive parameters to orient patient selection could be identified; however, the early response seemed to predict the overall outcome. Good results in our series were attributed to routine use of sildenafil and dopamine, along with the nitric oxide inhalation.