1. Repair of Congenital Aortic Valve Disease Department of Thoracic and Cardiovascular Surgery Seoul National University Hospital Yong Jin Kim, M.D.

2. Patient Profile ( I ) Male / 9 years History Heart murmur detected at birth (1992-02-13) 95-05-11 Lateral tunnel Fontan Op. 00-12-19 Coil embolization RPA & RUPV arteriovenous collateral Left IMA collateral to left lung

3. Patient Profile ( II ) Chief complaints DOE (Functional Class II- III) Systemic review Clubbing, cyanosis Physical examination Gr II~III/VI systolic murmur at apex Peripheral O2 saturation : 80% at room air EKG HR : 80~100 beats/min NSR with occasional PVC

4. Initial Diagnosis {A, D, L} RV type SV ( Rudimentary LV ) SA (Common AV valve) Bilateral SVC, IVC to hemiazygos( Left SVC ) PS & PDA

5. 0 0 Age : 3yr Bwt 13kg / Height 94cm / BSA 0.58m 2 Fenestrated Fontan Operation Fenestration 5.5mm Lateral tunneling with GoreTex patch Bilateral BCPC PDA division MPA division

6. Lateral Tunnel Fontan Op.

7. Pre-op Chest X-ray ( 2001.4.10)

8. Preoperative Diagnosis Hepatic vein into pulmonary atrium Adjacent to the coronary sinus & intrahepatic collateral formation Atrioventricular valve regurgitation ; moderate Aortic insufficiency ; moderate to severe Aortic Root (Echocardiography) Aortic Root (Echocardiography) Aortic annulus : 24 mm. Sinus : 35~37 mm. Sinotubular junction : 30~32 mm

9. Pre-Op Cardiac Catheterization

10. Operation (2001-04-16) Extracorporeal circulation CPB time : 159 min / ACC time : 88 min Cannulation Arterial cannular : Ascending aorta Venous cannula : Into the lateral tunnel Cardioplegics Blood cardioplegics : Direct antegrade 400cc/time × 3 times / Total Amount : 1200 cc

11. Operative Technique ( I ) A. Triangular resections of the dilated sinus wall Excision varies with the redundancy of the sinus wall & degree of reduction of the sinotubular junction B. Reconstruction of the aortic root AB

12. Operative Technique ( II ) Ascending aorta reduction plasty Subcommissural annuloplasty

13. Operation (2001-04-16) Hepatic vein ligation & clipping Aortic root reconstruction Subcommissural annuloplasty, reduction of sinus & sinotubular junction by triangular resection ( Valve sizer 21mm in reduced sinotubular junction) Ascending aorta reduction plasty Atrioventricular valve repair

14. Post-Op Chest X-ray

15. Postoperative EchoCG Aortic valve regurgitation Trivial Atrioventricular valve regurgitation Trivial Atrioventricular valve stenosis None Ligated left hepatic vein Good ventricular contraction Good pulmonary venous inflow