Objective: To describe clinical features, morphology, management and outcome of pulmonary vein stenosis (PVS) in childhood.
Design and setting: Retrospective international collaborative study involving 19 paediatric cardiology centres in UK, Ireland and Sweden.
Patients: Cases of PVS presenting between 1/1/95 and 31/12/04 were identified. Cases where pulmonary veins connected to a morphological left atrium were included. Functionally univentricular hearts and total anomalous pulmonary venous connection were excluded. All available data and imaging were reviewed.
Results: Fifty-eight cases were identified. In 22 cases (38%) there was premature delivery. Forty-six (79%) had associated cardiac lesions. Sixteen (28%) had undergone previous cardiac surgery prior to PVS diagnosis. Sixteen children (28%) had a syndrome or significant extra-cardiac abnormality.
Thirty-six presented with unilateral disease of which 86% was on the left. Where there was adequate sequential imaging, disease progression was shown with discrete stenosis leading to diffusely small pulmonary veins. Collateral vessels often developed.
Thirteen patients had no intervention. Initial intervention was by catheter in 17 and surgery in 28. Overall three-year survival was 49% (95% CI 35-63) with patients undergoing initial surgical intervention having greater freedom from death or re-intervention (Hazard ratio 0.44, 95% CI 0.2-0.99, p=0.023).
Conclusions: PVS is a complex disease of uncertain cause and frequently associated with prematurity. Early intervention may be indicated to deter irreversible secondary changes.
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