SCIMITAR SYNDROME COMPLICATED BY STENOSIS OF THE RIGHT PULMONARY VEIN- 4 CASE-REPORTS

The authors report 4 cases of the scimitar syndrome with pulmonary hyp ertension by stenosis of an abnormally draining right pulmonary vein a nd they also review the literature. All cases were symptomatic from in fancy. The diagnosis was confirmed by catheterisation which showed a s ignificant pressure gradient between the fight 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 treatme nt of the stenosis and reimplantation of the fight pulmonary vein in t he left atrium, or pneumonectomy) were satisfactory. However, it is po ssible that earlier treatment could be effective as changes in the pul monary vascular bed seem to occur very early in these patients.