Repair of

Congenital Aortic Valve Disease

Repair of

Congenital Aortic Valve Disease

Department of Thoracic and Cardiovascular Surgery

Seoul National University Hospital

Yong Jin Kim, M.D.

Patient Profile ( I )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

Patient Profile ( II )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 DiagnosisInitial Diagnosis

{A, D, L}

RV type SV ( Rudimentary LV )

SA (Common AV valve)

Bilateral SVC, IVC to hemiazygos( Left SVC )

PS & PDA

00

Age : 3yr

Bwt 13kg / Height 94cm / BSA 0.58m2

Fenestrated Fontan Operation Fenestration 5.5mm

Lateral tunneling with GoreTex patch

Bilateral BCPC

PDA division

MPA division

Lateral Tunnel Fontan Op.Lateral Tunnel Fontan Op.

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

Preoperative Diagnosis 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 CatheterizationPre-Op Cardiac Catheterization

Operation (2001-04-16)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

Operative Technique ( I )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

A B

Operative Technique ( II )Operative Technique ( II )

Ascending aorta reduction plasty

Subcommissural annuloplasty

Operation (2001-04-16)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

Post-Op Chest X-ray Post-Op Chest X-ray

Postoperative EchoCG 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