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.

Persistent Oxygen Requirement beyond Prematurity: A Case of Acquired Pulmonary Vein Stenosis

“…However, in cases of acquired PVS, initial echocardiogram may show normal pulmonary venous anatomy prior to the development of stenosis, making the diagnosis of PVS particularly difficult. Challenging the diagnosis further, NEC, BPD, and/or severe IUGR can be seen comorbid with PVS [14]. While the gold standard for diagnosis is cardiac catheterization, the risks of this procedure must be taken into consideration and may only be justified with a high index of suspicion, particularly given the frequency of cardiopulmonary morbidities in this population [19].…”

“…Specifically, it has been suggested that vascular mediators such as vascular endothelial growth factor (VEGF) [3, 4, 7] lead to the intimal and medial fibromuscular proliferation seen on vascular pathology [5, 8, 9]. Prematurity and bronchopulmonary dysplasia (BPD) have also been implicated, but at current their specific roles in pathogenesis remain unclear [7, 10–14]. …”

“…Data suggests limited survival beyond 5 years [12, 13, 15, 16]. Furthermore, increasing number of pulmonary vessels involved, prematurity, and other comorbidities have each been shown to further decrease survival [7, 14, 17]. Both surgical correction and vascular stenting approaches have achieved limited success and are complicated by high rates of restenosis.…”

Recomendaciones del VIII Consenso Clínico de SIBEN para la Displasia Broncopulmonar

“…Si esta condición queda sin diagnóstico, los resultados son peores que en los niños con DBP severa con HP, pero sin estenosis adquirida de las venas pulmonares. (93) Como posibles marcadores de HP en DBP podrían ser de utilidad encontrar elevación de niveles del péptido natriurético alto y niveles bajos de citrulina, un precursor del óxido nítrico. (94) Y un tratamiento podría ser dar L-citrulina, como se menciona antes.…”

Imaging Bronchopulmonary Dysplasia—A Multimodality Update

“…Interestingly, a recent study has suggested an association with pulmonary vein stenosis (PVS) with 4.6% of a 213-patient cohort of infants with BPD affected, more frequently those with lower birth weights. Those with associated PVS experienced higher rates of mortality (16). …”