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

Patient Profile ( I ) Male / 9 years History Heart murmur detected at birth ( ) Lateral tunnel Fontan Op Coil embolization RPA & RUPV arteriovenous collateral Left IMA collateral to left lung

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

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

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

Lateral Tunnel Fontan Op.

Pre-op Chest X-ray ( )

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

Pre-Op Cardiac Catheterization

Operation ( ) 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

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

Operative Technique ( II ) Ascending aorta reduction plasty Subcommissural annuloplasty

Operation ( ) 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

Post-Op Chest X-ray

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