Lower Distending Pressure Improves Respiratory Mechanics in Congenital Diaphragmatic Hernia Complicated by Persistent Pulmonary Hypertension.

Objective: To investigate the effects of distending pressures on respiratory mechanics and pulmonary circulation in newborn infants with congenital diaphragmatic hernia (CDH) and persistent pulmonary hypertension (PPHN).

Study Design: In total, 17 consecutive infants of ≥37 weeks of gestational age with CDH and PPHN were included in this prospective, randomized, crossover pilot study. Infants were assigned randomly to receive 2 or 5 cmHO of positive end-expiratory pressure (PEEP) for 1 hour in a crossover design.

Review shows that implementing a nationwide protocol for congenital diaphragmatic hernia was a key factor in reducing mortality and morbidity.

Unlabelled: The French Rare Disease Reference Center for congenital diaphragmatic hernia (CDH) was created in 2008, to implement a national protocol for foetuses and children with this serious condition. Neonatal mortality from CDH is 30-40%, mainly due to pulmonary hypoplasia and persistent pulmonary hypertension, and half of those who live have high respiratory, nutritional and digestive morbidity. CDH management requires long-term and specialised multidisciplinary care.

Safety and tolerability of subcutaneous treprostinil in newborns with congenital diaphragmatic hernia and life-threatening pulmonary hypertension.

Background: Prolonged pulmonary hypertension (PH) is highly predictive for pulmonary morbidity and death in infants with congenital diaphragmatic hernia (CDH).

Objectives: To report the effects and tolerability of subcutaneous treprostinil in newborns with severe CDH and late life-threatening PH.

Methods: We recorded clinical and echocardiography data before and after starting subcutaneous treprostinil, on patients with severe CDH and late PH, refractory to inhaled nitric oxide and oral sildenafil.