Continuous intrapulmonary distension with perfluorocarbon accelerates lung growth in infants with congenital diaphragmatic hernia: initial experience.
The authors have shown previously in an animal model that neonatal lung growth can be accelerated by continuous intrapulmonary distension with a perfluorocarbon (PFC). The authors now describe a preliminary clinical experience with this therapeutic concept in a select group of infants with congenital diaphragmatic hernia (CDH). Neonates with very high predicted mortality rate caused by CDH had their lungs completely filled with PFC while on extracorporeal life support (ECLS); (n = 5). A continuous positive pressure of 7 to 10 cm H2O was maintained via the endotracheal tube for 3 to 7 days (mean, 5.6 +/- 0.87 days). The areas of both lungs (L) then were measured daily from digitized chest x-rays and divided by the area of the corresponding L1 vertebrae (V), to create an L/V index, so as to control for variable roentgenographic techniques. Immediately after removal of PFC, blood gas data were collected off ECLS. At the end of continuous pulmonary distension, all patients showed improvements in oxygenation and ventilation. The ipsilateral lungs showed significant increase of the L/V index with time (P =.003) and of L/V's daily change (P <.0001), suggesting accelerated lung growth. Overall survival rate was 40% (2 of 5). Of the 3 patients that had 7 days of distension, 2 survived. Continuous intrapulmonary distension with PFC for up to 1 week accelerated ipsilateral lung growth, improved gas exchange, and increased survival of CDH infants with profound pulmonary hypoplasia marooned on ECLS. Additional trials of PFC-based pulmonary distension in similar infants are warranted.