[Scimitar syndrome complicated by stenosis of the right pulmonary vein. Apropos of 4 cases].
The authors report 4 cases of the scimitar syndrome with pulmonary hypertension by stenosis of an abnormally draining right pulmonary vein and they also review the literature. All cases were symptomatic from infancy. The diagnosis was confirmed by catheterisation which showed a significant pressure gradient between the right pulmonary vein and the inferior vena cava, and by angiography which demonstrated the stenosis. None of the treatments proposed (interventional catheterisation with dilatation and eventual implantation of a stent, surgery with treatment of the stenosis and reimplantation of the right pulmonary vein in the left atrium, or pneumonectomy) were satisfactory. However, it is possible that earlier treatment could be effective as changes in the pulmonary vascular bed seem to occur very early in these patients.
Dupuis
C
C
Service de cardiologie infantile, Hôpital cardiologique, Lille.
Rey
C
C
Godart
F
F
Vliers
A
A
Gronnier
P
P
D002404:Catheterization; D003251:Constriction, Pathologic; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology; D007223:Infant; D008297:Male; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000601:surgery; D012587:Scimitar Syndrome / Q000150:complications* / Q000628:therapy
Syndrome du cimeterre compliqué de sténose de la veine pulmonaire droite. A propos de 4 observations.