Case presentation:
A ten-year-old boy presented to our hospital with progressive exertional dyspnea and episodes of chest pain. He had undergone surgical repair of subaortic stenosis and supramitral ring 7 years ago. Transthoracic echocardiogram (TTE) revealed the following findings: situs inversus with dextrocardia, LVOTO with peak gradient (PG) of 90 mmHg along with moderate to severe aortic insufficiency (AI), moderate mitral regurgitation (MR) with mitral stenosis (MS) (PG across the mitral valve was 35 mmHg). Diagnostic cardiac catheterization was performed, and showed tunnel type LVOTO (Figure 1). Based upon these findings, the indication of Konno-Rastan operation was established. The patient was prepared appropriately and scheduled for elective surgical repair. The operation was performed via median sternotomy with great caution to avoid inadvertent rupture of any cardiac cavity due to the heavy adhesions from previous operation, and dextrocardia was kept in mind. The aorta was cannulated just below the take off of the innominate artery to gain as much length as possible on the ascending aorta. Bicaval cannulation was performed on the left side (Dextrocardia) to have a bloodless field. We placed a left ventricular vent via the left superior pulmonary vein. Aortic cross clamp was applied and the ascending aorta was opened longitudinally on the anterior aspect (Figure 2), and the cardioplegic solution was administered via the coronary ostia due to the severe AI. This incision was extended into the right coronary sinus to the right of the right coronary ostium (Dextrocardia) (Figure 3). By staying close to the right/left commissure, the conduction system is protected. The right ventricular outflow tract was opened and then cutting through the aortic annulus and ventriculo-infundibular fold into the ventricular septum (Figure 4). This incision is made between the right ostium and right/left commissure, staying closer to the right/left commissure as shown in Figure 3. The septal incision is usually about 10-15 mm in length and allows the aortic annulus to separate nicely (Figure 5). A sizer was placed to estimate the patch width and decide if the septal incision is adequate. The width of the patch will equal the additional annular circumference. The inferior aspect of the patch was sewn to the defect created in the ventricular septum (Figure 6). The superior aspect of the patch was used to augment the aortic sinus and sinotubular junction. Then, 2-0 ethibond horizontal mattress sutures were passed through the aortic annulus as per any aortic valve replacement and some of these sutures were passed through the patch in correspondence with the aortic annulus. The superior aspect of the patch was sutured to the aortotomy, and a second patch was used to augment the right ventricular outflow tract. The remainder of the operation was completed uneventfully. Postoperative TTE showed residual subaortic stenosis with PG of 40 mmHg, and there was significant improvement of MS with PG across the mitral valve of 12 mmHg. The patient was followed up for 6 months and was asymptomatic with normal physical activity.