OBJECTIVE. Pulmonary vein stenosis is a rare, although often lethal, anomaly. Risk factors for the diagnosis of pulmonary vein stenosis are poorly characterized. In this study we sought to identify factors associated with pulmonary vein stenosis, paying particular attention to preterm birth.

METHODS. By review of the cardiac database we identified all of the subjects with pulmonary vein stenosis over a 10-year period at our institution. Those children with anomalous pulmonary venous connection were not included. Patient-related variables were analyzed for their association with pulmonary vein stenosis. Pulmonary vein stenosis was diagnosed by spectral Doppler interrogation of the pulmonary veins (continuous, turbulent flow with calculated mean gradient > 5 mm Hg) and confirmed by cardiac catheterization in nearly all of the cases.

RESULTS. Twenty-six patients with pulmonary vein stenosis were identified. The median age at diagnosis was 7.4 months; range: 1 day to 35 months. Congenital heart defects were present in the majority of subjects. Associated genetic syndromes were present in 8 subjects (31%). The 2-year survival rate from diagnosis was 43%. The majority of subjects (16 [61%]) were preterm. Gestational ages ranged from 24.2 to 41.0 weeks, and birth weights ranged from 460 to 4445 g. Preterm birth was strongly associated with the diagnosis of pulmonary vein stenosis, odds ratio 10.2 (95% CI 4.7–22.6), p < .001. Eleven (42%) of the 26 subjects were treated for bronchopulmonary dysplasia before being diagnosed with pulmonary vein stenosis.

CONCLUSIONS. Prematurity is associated with the diagnosis of pulmonary vein stenosis. It is interesting to note that many of these patients also have intracardiac shunt lesions, which may act in concert with preterm endothelium to produce pulmonary vein stenosis.

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