Pulmonary vein stenosis and necrotising enterocolitis: is there a possible link with necrotising enterocolitis?

Objectives: While acquired pulmonary vein stenosis (PVS) is an often lethal anomaly with poor long-term prognosis and high mortality, little is known about the causes of this disease process. The purpose of this study was to describe the possible association between acquired PVS and necrotising enterocolitis (NEC) in premature infants.

Study Design: We performed a retrospective review of all premature infants (<37 weeks' gestation) diagnosed with acquired PVS in our institution. Babies with congenital heart disease with known association with PVS were excluded. The hospital records were reviewed for prior history of NEC, as defined by Bell's staging criteria. We also reviewed serial echocardiograms performed during their hospitalisation. Outcomes assessed were worsening or resolution of the PVS and death.

Results: Twenty patients met inclusion criteria and were diagnosed with acquired PVS. The median gestational age was 27 weeks. 50% (10/20) of the infants had NEC during their hospital course. The NEC group had significantly lower birth weights in comparison to the non-NEC group. There was no difference between groups with regards to the age at diagnosis of PVS. The mean gradient across the pulmonary veins was higher in the NEC group, as was mortality.

Conclusions: There appears to be a high incidence of NEC in premature infants who are diagnosed with acquired PVS. Future large controlled studies are needed to further analyse this association and to evaluate the possible role of abdominal inflammation in the development of PVS in premature infants.