Advanced

Pulmonary vein stenosis: the UK, Ireland and Sweden collaborative study

Seale, A. N.; Webber, S. A.; Uemura, H.; Partridge, J.; Roughton, M.; Ho, S. Y.; McCarthy, K. P.; Jones, S.; Shaughnessy, L. and Sunnegardh, J., et al. (2009) In Heart 95(23). p.1944-1949
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... (More)
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. (Less)
Please use this url to cite or link to this publication:
author
, et al. (More)
(Less)
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Heart
volume
95
issue
23
pages
1944 - 1949
publisher
BMJ Publishing Group
external identifiers
  • wos:000271947600013
  • scopus:72049098170
ISSN
1355-6037
DOI
10.1136/hrt.2008.161356
language
English
LU publication?
yes
id
208b88a3-3ade-4801-93ac-fdddff5d19ef (old id 1518361)
date added to LUP
2010-01-13 11:03:20
date last changed
2017-11-05 03:41:07
@article{208b88a3-3ade-4801-93ac-fdddff5d19ef,
  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.},
  author       = {Seale, A. N. and Webber, S. A. and Uemura, H. and Partridge, J. and Roughton, M. and Ho, S. Y. and McCarthy, K. P. and Jones, S. and Shaughnessy, L. and Sunnegardh, J. and Hanséus, Katarina and Rigby, M. L. and Keeton, B. R. and Daubeney, P. E. F.},
  issn         = {1355-6037},
  language     = {eng},
  number       = {23},
  pages        = {1944--1949},
  publisher    = {BMJ Publishing Group},
  series       = {Heart},
  title        = {Pulmonary vein stenosis: the UK, Ireland and Sweden collaborative study},
  url          = {http://dx.doi.org/10.1136/hrt.2008.161356},
  volume       = {95},
  year         = {2009},
}