Surgical Approach To A Congenital Mitral Stenosis Case With Left Persistent Superior Vena Cava

Congenital mitral valve stenosis is a rare pathology. In this study, we aimed to present our surgical approach and successful valve repair to a case with congenital mitral valve stenosis combined with left persistent superior vena cava. Conservative surgery of the congenital mitral valve stenosis can be performed with acceptable early and midterm outcome in terms of mortality and reoperation rate.For this reason it is the procedure of choice for congenital mitral valve stenosis. INTRODUCTION Congenital malformations of the mitral valve are rare, complex, and frequently associated with other cardiac malformations(1). In congenital mitral anomalies, mostly because of the presence of the dysplastic leaflet group, the anatomy overlaps the functional groups and repair strategies can be identical(1). The current risk of mitral valve operation in the pediatric age group is low, and the long-term results are satisfactory, irrespective of severe deformation of the mitral valve apparatus and associated complex cardiac anomalies(2). The long-term results of conservative surgery are confirmed with a low incidence of reoperation except in mitral valve stenosis(3). CASE PRESENTATION Our case was a 5-year-old girl who was admitted to the Department of Pediatric Cardiology with complaints of fatigue. Echocardiographic investigations revealed severe mitral stenosis and she was hospitalized for surgical correction. She was operated under endotracheal general anesthesia and in supine position.Following a median sternotomy,pericardium was opened longitudinally. After heparinization, extra-corporeal circulation was established between the venae cavae and the ascending aorta. A cross clamp was placed on aorta and by antegrade intermittent isothermic blood cardioplegia,cardiac arrest was established.Hypothermia was moderate (28oc).A vent was placed via the right superior pulmonary vein. After standard cannulation, right atriotomy was performed revealing an excessive amount of blood draining from coronary sinus ostium. Persistent left superior vena cava was found and suspended and snared (Figure 1).