Objectives Pulmonary vein stenosis (PVS) is a rare, often lethal anomaly associated with poor outcomes. Given the association between bronchopulmonary dysplasia (BPD) and cardiovascular complications, we tested the hypotheses that (1) a subgroup of neonates with severe BPD develop PVS (BPD-PVS) and have worse outcomes than do neonates with severe BPD alone (BPD); (2) among a cohort of neonates with severe BPD-associated pulmonary hypertension (BPD-PH), PVS is an additional risk factor for adverse outcomes and mortality. Study Design We performed a retrospective review of neonates with severe BPD, based on the Eunice Kennedy Shriver National Institute of Child Health and Development (NICHD) criteria, at our institution between June 1, 2009, and June 30, 2013. PVS was determined based on serial review of echocardiograms performed during their hospitalization. Neonates with congenital heart disease or chromosomal anomalies were excluded. Results Of 213 patients with severe BPD, 10 (4.7%) were found to have PVS (BPD-PVS). Neonates with BPD-PVS had lower birth weight (634 ± 178 vs. 767 ± 165 g; p < 0.01) and were more likely to be intrauterine growth restricted (80 vs. 11%; p < 0.01) than neonates with BPD alone. Time on mechanical ventilation and length of hospitalization were longer in the BPD-PVS group than BPD group. Survival was lower in the BPD-PVS group than BPD group (5/10 [50%] vs. 196/203 [97%]; log-rank test p < 0.01). Among a subgroup of neonates with BPD-PH, survival was lower among infants with PVS than those without PVS (5/9 [56%] vs. 26/30 [86%]; log-rank test p = 0.01). Conclusions Compared with neonates with severe BPD alone, those with acquired PVS are at increased risk for worse outcomes, including higher mortality. Evidence-based recommendations regarding screening protocols and surveillance are needed in this high-risk subgroup of BPD neonates.
Practice patterns for extracorporeal membrane oxygenation (ECMO) use in newborns with Trisomy 21 (T21) have not been fully reported. The goals of this study were to 1) determine the incidence of ECMO use in T21 neonates; 2) identify clinical and demographic characteristics associated with ECMO use in this population; 3) describe outcomes of neonates with T21 supported with ECMO. This was a retrospective cohort study using the Pediatric Health Information System database (January 2000 to January 2014). Given the exploratory nature, only descriptive statistics were used. p < 0.05 was considered significant. Within 43 pediatric hospitals, the incidence of ECMO use in neonates with T21 was 2.3% (131/5,737). Neonates with T21 supported with ECMO were more likely to be admitted earlier; have higher birth weight, gestational age, and longer hospitalization; and have congenital diaphragmatic hernia or select cardiac anomalies versus those who did not require ECMO. T21 neonates supported with ECMO also had higher incidence of bronchopulmonary dysplasia, necrotizing enterocolitis, and mortality (unadjusted odds ratio 12.3 [95% confidence interval: 8.6-17.6]) compared with T21 neonates not exposed to ECMO. Compared with T21 neonates not requiring ECMO, those supported with ECMO had increased morbidity and mortality. Additional investigation on timing, indications, and risk/benefit profiles, for ECMO use in T21 neonates is needed.
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