Supravalvular mitral stenosis associated with ventricular septal defect, subvalvular aortic stenosis and double aortic arch

We have presented the case of a 14 year old girl admitted to the hospital with irreversible heart failure. Clinically, a ventricular septal defect associated with congenital mitral stenosis or cor triatriatum was suspected. The anatomic examination showed the following congenital malformations: (1) A rare type of supravalvular mitral stenosis constituted by a semicircular fibrous band originating at the level of the posterior leaflet and protruding into the mitral orifice causing obstruction; (2) a large ventricular septal defect; (3) mild subvalvular aortic stenosis; (4) double aortic arch; and (5) persistent left superior vena cava. The clinical picture was dominated by the ventricular septal defect and mitral stenosis. The subvalvular aortic stenosis did not appear to produce a significant obstruction, and the double aortic arch had produced symptoms only in the first years of life.