Extracorporeal Membrane Oxygenation in Children with Pulmonary Atresia and Intact Ventricular Septum: Mortality and Associated Outcomes

Data on outcomes of extracorporeal membrane oxygenation (ECMO) are limited in patients with pulmonary atresia intact ventricular septum (PAIVS). The objective of this study was to describe the use of ECMO and the associated outcomes in patients with PAIVS. We retrospectively reviewed neonates with PAIVS who received ECMO between 2009 and 2019 in 19 US hospitals affiliated with the Collaborative Research for the Pediatric Cardiac Intensive Care Society (CoRe-PCICS). Patients who received ECMO were compared to those who did not and patients on ECMO who died were compared to those who survived by bivariate analysis and multivariable logistic regression. The predictive ability of a risk score for inpatient mortality (using beta coefficients) was assessed by receiver operator curve analysis. Of 295 identified patients, 32 (11%) were supported with extracorporeal membrane oxygenation. Of these, 15 (46%) experienced mortality. A higher left pulmonary artery z-score (beta coefficient 0.72) and the presence of ventriculocoronary connections by cardiac catheterization (beta coefficient 1.25) were associated with an increased risk of ECMO (p-value < 0.01). The resulting risk score had an area under the curve of 0.71 (p-value 0.03) for the prediction of need for ECMO. In a multicenter cohort of patients with PAIVS, 11% received ECMO. Of those supported with ECMO, 46% experienced inpatient mortality. A higher left pulmonary artery z-score and the presence of ventriculocoronary connections appear to be risk factors for the use of ECMO.