Pulmonary vein stenosis with normal connection: associated cardiac abnormalities and variable outcome.
BACKGROUND
Pulmonary vein (PV) stenosis with anatomically normal connection is considered rare, unresponsive to treatment, progressive, and usually fatal.


METHODS
We reviewed the records of 13 children with this diagnosis at our center since 1990.


RESULTS
The number of stenosed PVs ranged from all PVs (n = 5); three PVs (n = 1); two PVs (n = 5); and one PV (n = 2). All patients had associated congenital cardiac abnormalities. Operation on PV stenosis was attempted in 7 patients (54%), 2 of whom have done well and 5 of whom have not. Two patients underwent heart transplantation for inoperable associated cardiac lesions. Significantly more patients with three or four stenosed PVs died (83%) compared with patients with one or two stenosed PVs (0%).


CONCLUSIONS
(1) Pulmonary vein stenosis with anatomically normal connection is associated with other congenital cardiac abnormalities, (2) presentation and outcome are contingent on the number of stenosed PVs, (3) surgical palliation may be helpful in some patients, and (4) heart transplantation for inoperable associated cardiac abnormalities may be an option in patients with only one or two stenosed PVs.
Department of Pediatrics, Primary Children's Medical Center and University of Utah, Salt Lake City 84113, USA.
J P
JP
Breinholt
J A
JA
Hawkins
L A
LA
Minich
L Y
LY
Tani
G S
GS
Orsmond
S
S
Ritter
R E
RE
Shaddy
D000293:Adolescent; D013504:Cardiovascular Surgical Procedures; D002648:Child; D002675:Child, Preschool; D003251:Constriction, Pathologic; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D008297:Male; D010166:Palliative Care; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000601:surgery; D016896:Treatment Outcome