The Use of Inhaled Nitric Oxide in Congenital Diaphragmatic Hernia

Congenital diaphragmatic hernia (CDH) is a complex congenital defect of the diaphragm that allows abdominal contents to herniate into the chest cavity, altering pulmonary development, and leading to pulmonary hypoplasia and hypertension. Patient presentation is variable in severity, making management difficult. Many common management strategies have improved the survival rate of infants with CDH, including gentle ventilation and permissive hypercapnia. Inhaled nitric oxide (iNO) is a potent, pulmonary vasodilator that has shown to improve oxygenation while decreasing pulmonary pressure in infants with pulmonary hypertension. While many institutions utilize iNO in the treatment of pulmonary hypertension, the role of iNO as a treatment for CDH is controversial.

The purpose of this literature review is to establish a better understanding of CDH including embryology, patient presentation, and management strategies, and to analyze the use and effect of iNO on patients in this population.

A systemic search of PubMed, CINHAL, and Medline was performed to identify research articles studying the use of iNO on patients with pulmonary hypertension secondary to CDH.

Three studies were utilized in this review to assess the current use and effectiveness of iNO in patients with CDH.

This review summarized the pathophysiology, embryology, patient presentation, and management strategies, as well as reviewed literature on the effectiveness of iNO on pulmonary hypertension secondary to CDH.

Further research is indicated to determine alternative treatment modalities and establish evidence-based practice protocols to standardize care in infants with CDH.