Pulmonary vein stenosis: the UK, Ireland and Sweden collaborative study.
OBJECTIVE
To describe clinical features, morphology, management and outcome of pulmonary vein stenosis (PVS) in childhood.


METHODS
Retrospective international collaborative study involving 19 paediatric cardiology centres in the UK, Ireland and Sweden.


METHODS
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.
Seale
A N
AN
Royal Brompton Hospital, and National Heart and Lung Institute, Imperial College, London SW3 6NP, UK.
Webber
S A
SA
Uemura
H
H
Partridge
J
J
Roughton
M
M
Ho
S Y
SY
McCarthy
K P
KP
Jones
S
S
Shaughnessy
L
L
Sunnegardh
J
J
Hanseus
K
K
Rigby
M L
ML
Keeton
B R
BR
Daubeney
P E F
PE
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003251:Constriction, Pathologic / Q000401:mortality / Q000473:pathology / Q000628:therapy; D018450:Disease Progression; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications / Q000401:mortality; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007234:Infant, Premature; D007235:Infant, Premature, Diseases / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D007494:Ireland; D053208:Kaplan-Meier Estimate; D008297:Male; D011667:Pulmonary Veins / Q000002:abnormalities / Q000473:pathology; D011668:Pulmonary Veno-Occlusive Disease / Q000209:etiology / Q000473:pathology* / Q000628:therapy*; D012189:Retrospective Studies; D013548:Sweden; D016896:Treatment Outcome; D006113:United Kingdom