A. N. Seale, S. A. Webber, H. Uemura, J. Partridge, M. Roughton, S. Y. Ho, K. P. McCarthy, S. Jones, L. Shaughnessy, J. Sunnegardh, K. Hanseus, M. L. Rigby, B. R. Keeton, P. E. F. Daubeney
Royal Brompton Hospital, and National Heart and Lung Institute and Imperial College.
United Kingdom, Ireland and Sweden
Heart
Heart 2009; 95: 1944-1949
DOI: 10.1136/hrt.2008.161356
Abstract
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.
Category
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Stenosis or Obstruction of Pulmonary Veins Following Surgical Repair of Anomalous Pulmonary Venous Connections
Catheter-mediated Interventions: Efficacy or Lack of Efficacy
Surgical Interventions for Pulmonary Venous Obstruction After the Onset of Disease
Length of Life Associated with Pulmonary Venous Obstruction
Year of Publication: 2009
Age Focus: Pediatric
Article Type: Retrospective Observational Cohort Studies (>10 patients)
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: No