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 the UK, Ireland and Sweden.
Patients: Cases of PVS presenting between 1 January 1995 and 31 December 2004 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: 58 cases were identified. In 22 cases (38%) there was premature delivery. 46 (79%) had associated cardiac lesions; 16 (28%) had undergone previous cardiac surgery before PVS diagnosis. 16 children (28%) had a syndrome or significant extracardiac abnormality. 36 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. 13 patients had no intervention. Initial intervention was by catheter in 17 and surgery in 28. Overall 3-year survival was 49% (95% CI 35% to 63%) with patients undergoing initial surgical intervention having greater freedom from death or re-intervention (hazard ratio 0.44, 95% CI 0.2 to 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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Funding Funding from the Harrison Heart Foundation and Joe Gandon Memorial Trust.
Competing interests None.
Provenance and peer review Not commissioned; externally peer reviewed.
Participating institutions Alder Hey Children’s Hospital, Liverpool; Birmingham Children’s Hospital, Birmingham; Bristol Royal Hospital for Children, Bristol; Cardiothoracic Unit Southampton General Hospital, Southampton; Evelina Children’s Hospital, London; Great Ormond Street Hospital for Children, London; Leeds General Infirmary, Leeds; Lund University Hospital, Lund; Our Lady’s Hospital for Sick Children, Dublin; Royal Brompton Hospital, London; Royal Hospital for Sick Children, Edinburgh; Royal Hospital for Sick Children Yorkhill, Glasgow; Royal Manchester Children’s Hospital, Manchester; The Children’s Hospital for Wales, Cardiff; The Freeman Hospital, Newcastle; The Glenfield Hospital, Leicester; The John Radcliffe Hospital, Oxford; The Queen Silvia Children’s Hospital, Gothenburg; The Royal Belfast Hospital for Sick Children, Belfast.