Objective: Neonates with an irreversible pulmonary dysplasia such as alveolar capillary dysplasia, surfactant protein deficiency, and pulmonary lymphangiectasis may have a deteriorating clinical course requiring cardiopulmonary support with extracorporeal membrane oxygenation. These neonates are often difficult to distinguish from those with persistent pulmonary hypertension of the newborn. The objective of this study was to identify clinical variables that distinguish infants with irreversible pulmonary dysplasia from those with persistent pulmonary hypertension of the newborn before, and while receiving, extracorporeal membrane oxygenation support.
Design: A retrospective analysis of the Extracorporeal Life Support Registry from 2000 to 2010 was performed.
Setting: A total of 114 extracorporeal membrane oxygenation centers providing data to the Extracorporeal Life Support Registry.
Patients: All neonates day of life 0-31 reported to the Extracorporeal Life Support Registry with irreversible pulmonary dysplasia and persistent pulmonary hypertension of the newborn were identified.
Measurements and main results: Patient demographics, pre-extracorporeal membrane oxygenation variables, and survival were analyzed. Univariate analysis was performed using Student's t test or Fisher's exact test, and variables found to be significant underwent multivariate analysis by logistic regression. Neonates with irreversible pulmonary dysplasia were placed on extracorporeal membrane oxygenation later (day of life 5.3 vs. 3.0, p = .045) and for a longer duration (11.1 vs. 6.8 days, p < .001) than those with persistent pulmonary hypertension of the newborn. Initiation of extracorporeal membrane oxygenation at day of life ≥5 (p = .026) and a duration of extracorporeal membrane oxygenation ≥10 days (p = .003) were independent predictors of irreversible pulmonary dysplasia by multivariate analysis. No differences in demographics, blood gas values, or vascular access were observed. Survival to discharge was significantly lower for neonates with irreversible pulmonary dysplasia (3%) vs. persistent pulmonary hypertension of the newborn (81%, p < .001).
Conclusion: Although neonates with irreversible pulmonary dysplasia and persistent pulmonary hypertension of the newborn have similar presentations, those with irreversible pulmonary dysplasia require extracorporeal membrane oxygenation support later in the perinatal period and for a longer duration. For neonates with a diagnosis of persistent pulmonary hypertension of the newborn, irreversible pulmonary dysplasia should be considered when extracorporeal membrane oxygenation is initiated on day of life ≥5 and/or the duration of extracorporeal membrane oxygenation ≥10 days.