title: Pulmonary Vein Stenosis in Neonates with Severe Bronchopulmonary Dysplasia.
abstract: 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.
authors: Division of Neonatology, The Ohio State University Wexner Medical Center, Columbus, Ohio.
authors: Natasha L
authors: NL
authors: Swier
authors: The Heart Center at Nationwide Children's Hospital, Columbus, Ohio.
authors: Bernadette
authors: B
authors: Richards
authors: The Heart Center at Nationwide Children's Hospital, Columbus, Ohio.
authors: Clifford L
authors: CL
authors: Cua
authors: Division of Neonatology, The Ohio State University Wexner Medical Center, Columbus, Ohio.
authors: Susan K
authors: SK
authors: Lynch
authors: Department of Biostatistics, Nationwide Children's Hospital Research Center, Columbus, Ohio.
authors: Han
authors: H
authors: Yin
authors: Division of Neonatology, The Ohio State University Wexner Medical Center, Columbus, Ohio.
authors: Leif D
authors: LD
authors: Nelin
authors: Seattle Children's Research Institute, University of Washington School of Medicine, Seattle, Washington.
authors: Charles V
authors: CV
authors: Smith
authors: Division of Neonatology, The Ohio State University Wexner Medical Center, Columbus, Ohio.
authors: Carl H
authors: CH
authors: Backes
mesh_terms: D001997:Bronchopulmonary Dysplasia / Q000150:complications* / Q000628:therapy; D005260:Female; D005317:Fetal Growth Retardation / Q000453:epidemiology; D005865:Gestational Age; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000401:mortality*; D007223:Infant; D007231:Infant, Newborn; D019102:Infant, Very Low Birth Weight; D007902:Length of Stay; D008297:Male; D012121:Respiration, Artificial; D012189:Retrospective Studies; D012307:Risk Factors; D000071078:Stenosis, Pulmonary Vein / Q000209:etiology / Q000401:mortality*; D016019:Survival Analysis; D014481:United States