1. Congenital Cardiac Surgery Yong Jin Kim, M.D. Seoul National University Children’s Hospital WWW.Drheart.net

2. History

3. SNU Children’s Hospital Pre-Heart-Lung Machine Era 1938. Gross. First successful PDA ligation 1944. Crafoord. Resection of coarctation of aorta 1945. Blalock. Blalock-Taussig operation 1946. Gross. Surgical closure of AP window 1958. Glenn. Glenn shunt

4. SNU Children’s Hospital First Blalock-Taussig Shunt “ Most powerful stimulus to the development of cardiac surgery ”

5. SNU Children’s Hospital Era of Cardiopulmonary Bypass I 1953. Gibbon. ASD closure 1953. Lillehei. VSD closure 1954. Lillehei. TOF correction 1956. Kirklin. TAPVR correction 1957. Kirkin. DORV correction

6. SNU Children’s Hospital Era of Cardiopulmonary Bypass II 1959. Senning. Atrial switch operation for TGA 1966. Ross. Ross procedure for TOF with PA 1971. Fontan. Fontan operation for TA 1975. Jatene. Arterial switch operation for TGA 1983. Norwood. Norwood procedure for HLHS 1985. Bailey. Pediatric heart transplantation

7. SNU Children’s Hospital Development of CPB Prerequisites –Understanding of physiology of circulation –Preventing the blood form clotting –Pumping blood to pump –Ventilating the blood

8. SNU Children’s Hospital Development of CPB 1951. Dodrill. Mitral valve surgery under left heart bypass 1952. Dodrill. Relief of PS under right heart bypass 1953. Lewis. ASD closure under surface cooling 1953. Gibbon. ASD closure by heart-lung machine 1954. Lillihei. VSD closure under controlled cross- circulation 1954. Kirklin. Establishment of CPB with oxygenator in cardiac surgery

9. SNU Children’s Hospital Controlled Cross-circulation 1954. Lillehei First surgical closure of VSD under controlled cross-circulation Used in 45 patients between 1954 to 1955 VSD TOF AVSD Dr.Lillehei

10. SNU Children’s Hospital J. Gibbon and Heart-lung Machine

11. SNU Children’s Hospital Fontan Operation for Functional Single Ventricle Fnacis Fontan

12. SNU Children’s Hospital

13. CPB Circuit Pump Oxygenator Heat exchanger Reservoir Filter Sucker & vent Cardioplegic solution delivery system

14. SNU Children’s Hospital

15. Diseases

16. To be Corrected in Neonate Critical LVOTO –Critical AS –Interrupted aortic arch –Symptomatic CoA Critical RVOTO –PA with IVS –PA with VSD Critical pulmonary venous return –Obstructive TAPVC Other complex heart disease –TGA –Truncus arteriosus –Hypoplastic left heart syndrome

17. SNU Children’s Hospital To be Corrected in Infancy Pulmonary outflow obstruction –Functional single ventricles –TOF –PA –Critical PS –TGA & CC-TGA CHF –LR shunt ( Large VSD, AVSD, PDA, TAPVR etc.) –Severe valvular diseases –Other LVOTO lesions ( IAA, CoA etc.) –Other complex anomalies

18. SNU Children’s Hospital Palliative Surgery Systemic – pulmonary artery shunt Blalock-Taussig shunt Unifocalization and shunt Cavopulmonary shunt (BCPS) RVOT reconstruction Valvotomy Patch widening Valved conduit Pulmonary artery banding Atrial septectomy

19. SNU Children’s Hospital Palliative Surgery Increase pulmonary blood flow –BT shunt –Potts shunt –Watterston shunt –Glenn shunt –RVOT reconstruction Decrease pulmonary blood flow –Pulmonary artery bancing Increase pulmonary-systemic mixing –Rashikind –Blalock-Hanlon –Palliative Mustard or Senning

20. SNU Children’s Hospital Rt. Modified BT Shunt

21. SNU Children’s Hospital Pulmonary Artery Banding

22. SNU Children’s Hospital Classification of Congenital Cardiac Diseases Pure obstructive lesions Simple L-R shunt R-L shunt (Cyanotic defects with decreased PBF) Complex cyanotic defects (“mixing defects”)

23. SNU Children’s Hospital Pure Obstructive Lesions Pulmonary stenosis (PS) Mitral stenosis (MS) Left ventricular outflow tract obstruction (LVOTO) Coarctation of aorta (CoA) Interrupted aortic arch (IAA)

24. SNU Children’s Hospital Pulmonary Stenosis (PS) A form of RV outflow obstruction in which stenosis is usually valvar or both valvar & infundibular or only infundibular Indications of intervention –Critical PS in neonate : indicated with diagnosis –PS in infants and children : indicated with sx. & pressure gradient over 50mmHg –Surgical treatment is not indicated with mild stenosis.

25. SNU Children’s Hospital Pulmoary Stenosis PS – membranous typePoststenotic dilatation

26. SNU Children’s Hospital Coarctation of Aorta (CoA) Congenital narrowing of upper thoracic aorta adjacent to the ductus arteriosus Op. indications –Reduction of luminal diameter > 50% –Upper body hypertension > 150 mmHg in infant –With CHF at any age

27. SNU Children’s Hospital CoA Extended End-to-End Anastomosis

28. SNU Children’s Hospital Interruption of Aortic Arch Complete luminal & anatomic discontinuity between two segments of aortic arch, & generalized narrowing of LVOT, posterior malalignment, muscle of Moulaert, small aortic annulus, aortic hypoplasia Dx. is an indication of operation –Coexisting cardiac anomaly : not contraindication –One-stage repair : preferred –Two-stage repair : in complicated anomalies –Single ventricle associated : alternative plan

29. SNU Children’s Hospital Interruption of Aortic Arch (IAA)

30. SNU Children’s Hospital IAA type B - Operation

31. SNU Children’s Hospital LVOTO The various forms of LVOTO occur in combination with other cardiac lesions (IAA, CoA, MV anomalies, LV hypoplasia) Obstructive types –Supravalvular –Valvar –Subvalvular –intraventricular

32. SNU Children’s Hospital Tunnel Stenosis – Konno Operation (1)

33. SNU Children’s Hospital Tunnel Stenosis – Konno Operation (2)

34. SNU Children’s Hospital Simple L-R Shunt Patent ductus arteriosus (PDA) Atrial septal defect (ASD) Ventricular septal defect (VSD) Atrioventricular septal defect (AVSD) Aortopulmonary window (AP window)

35. SNU Children’s Hospital Patent Ductus Arteriosus Open communication usually between upper descending Ao. and proximal portion of LPA Surgical indications –Significant PDA : indicated after 1 st month –Prophylactic closure : 6 -12 months –Sx. of heart failure or failure to thrive : indicated at any time Pulmonary vascular disease : contraindicated

36. SNU Children’s Hospital PDA

37. PDA Ligation

38. SNU Children’s Hospital Atrial Septal Defect  A hole of variable size in the atrial septum and is most common cardiac malformation with various location of defect, fossa ovalis, posterior, ostium, primum, coronary sinus, subcaval (sinus venosus)  Uncomplicated ASD or of PAPVC with RV volume overload (Qp/Qs>1.5 or 2.0) : an indication –Scimitar syndrome –Isolated PAPVC –Optimal age : under 5 years but recently 1-2 years to avoid RV volume overload

39. SNU Children’s Hospital ASD - Surgical Anatomy

40. SNU Children’s Hospital Patch Closure of Secundum ASD

41. SNU Children’s Hospital Ventricular Septal Defect A hole (or multiple holes) between Lt & Rt ventricle Surgical Indication –Symptomatic large VSD : indication of operation –Moderate sized VSDs (Qp/Qs < 3.0) with few sx. : observation in infancy –Small VSDs (Qp/Qs < 1.5) : not indicated, risk of bacterial endocarditis –Subarterial type : early repair before childhood

42. SNU Children’s Hospital Types of VSD

43. SNU Children’s Hospital VSD – PM Patch Closure

44. SNU Children’s Hospital Atrioventricular Septal Defect Abnormalities of atrioventricular valve form & function, and interatrial and interventricular communication resulted from maldevelopment of the endocardial cushions Presence of AVSD : indicated with Dx. –Partial AVSD : 1-2 years of age except CHF or growth failure –Complete AVSD with good condition : 3-6 mo of age –Complete AVSD with CHF or respiratory Sx : indicated promptly –Pulmonary vascular disease : not indicated

45. SNU Children’s Hospital Types of AVSD

46. SNU Children’s Hospital Partial AVSD

47. SNU Children’s Hospital Complete AVSD

48. SNU Children’s Hospital R-L Shunt Tetralogy of Fallot (TOF) TOF with PA Pulmonary atresia with intact ventricular septum (PAIVS) Ebstein’s anomaly Tricuspid atresia

49. SNU Children’s Hospital Tetralogy of Fallot Characterized by underdevelopment of RV infundibulum with anterior and left-ward displacement of conal septum Dx. is an indication of operation –Symptomatic complicated in early life : Early total correction or initial shunt (1-2 mo) & total correction (1 year) –Asymptomatic uncomplicated : Total correction at 3-24 mo –Multiple VSDs, LAD from RCA : Initial shunt and total correction

50. SNU Children’s Hospital TOF

51. TOF – RVOT Patch Widening

52. SNU Children’s Hospital Ebstein’s Anomaly A congenital defect of tricuspid valve in which the origin of septal and posterior leaflets or both are displaced downward into the right ventricle and the leaflets are variably deformed Symptomatic Ebstein’s anomaly is an indication. –Neonates presenting in extremes : Starnes procedure in the first week –Valve repair and ASD closure : Cardiomegaly with important TR Moderate and severe cyanosis –WPW syndromes : Ablation of accessory conduction pathway

53. SNU Children’s Hospital Ebstein’s Anomaly

54. SNU Children’s Hospital Ebstein’s Anomaly – Operation (Danielson method)

55. SNU Children’s Hospital Tricuspid Atresia A cardiac anomaly in which RA fails to open into a ventricle through an AV valve. There is thus a univentricular AV connection PVR is an important indicator > 4 unit – contraindicaton, 2-4 unit – BCPS, Fontan –Symptomatic in early life Early shunt or PAB BCPS or hemi-Fontan at 6-12 mo Fontan at 12-24 mo –Nonsymptomatic Fontan candidate : 12-30 mo

56. SNU Children’s Hospital Types of Tricuspid Atresia

57. SNU Children’s Hospital Complex Cyanotic Lesions Double outlet right ventricle (DORV) Univentricular heart Transposition of great arteries (TGA) Total anomalous pulmonary venous connection (TAPVC) Truncus arteriosus Hypoplastic left heart syndrome (HLHS)

58. SNU Children’s Hospital Double Outlet Right Ventricle A congenital cardiac anomalies in which both great arteries rise wholly or in large part from the RV. It is, then, a type of ventriculoarterial connection. Dx is an indication of operation Surgical options –Simple DORV with subaortic VSD : repair by 6 mo –DORV with subpulmonic VSD (Taussig-Bing heart) : Arterial switch operation within 1 mo –DORV with PS : REV or Rastelli op. at 3-5 years –DORV with noncommitted VSD : VSD enlargement & intraventricular tunnel Fontan operation

59. SNU Children’s Hospital Types of DORV

60. SNU Children’s Hospital DORV – REV Operation

61. SNU Children’s Hospital DORV (Taussig-Bing); Rastelli operation

62. SNU Children’s Hospital Rastelli Operation ( Conduit Placement)

63. SNU Children’s Hospital Univentricular Heart A wide variety of congenital defects in which there is functionally only a single ventricular chamber The pathophysiology is determined primarily by obstruction of outflow to either systemic or pulmonary circulation and by the presence or absence of obstruction to pulmonary venous return.

64. SNU Children’s Hospital Surgical Strategy for Single Ventricle (1) Neonatal period If pulmonary venous obstruction is not present, surgical management is dictated by whether there is inadequate or excessive pulmonary blood flow, with or without obstruction to systemic blood flow. –Systemic to pulmonary artery shunt –Pulmonary artery banding –Damus-Kaye-Stansel procedure, Norwood operation

65. SNU Children’s Hospital Surgical Strategy for Single Ventricle (2) Beyond the neonatal period –The major goals are to minimize the pressure and volume loads on the single ventricle. –Removal of systemic-to-pulmonary artery shunts or division of the main pulmonary artery and the creation of direct connections between the systemic venous circulation and the pulmonary arteries Bidirectional cavopulmonary connection Modified Fontan operation (in series circulation)

66. SNU Children’s Hospital Lateral Tunnel Fontan

67. SNU Children’s Hospital Transposition of Great Arteries A cardiac anomaly in which the Ao. arises entirely or in large part from the RV, and the PA from LV. (atrio-ventricular concordant connection and ventriculo-arterial discordant connection) Surgical Options –Simple TGA in neonate : ASO within 1 mon –Simple TGA beyond 30 days : Rapid two-stage operation Atrial switch operation (Mustard, Senning) –TGA with VSD : ASO as early as possible –TGA with VSD and PS ; Rastelli or REV operation

68. SNU Children’s Hospital TGA – Operative View

69. SNU Children’s Hospital Arterial Switch Operation

70. SNU Children’s Hospital Total Anomalous Pulmonary Venous Connection (TAPVC)  There is no direct connection between any pulmonary vein and the left atrium. Rather, all the pulmonary veins connect to the RA or one of its tributaries.  Dx. is an indication of operation. –Immediate repair with Dx. in any ill neonate : Preop. preparation is not needed. –Repair should be done always before 6 mo. –Dx. at 6-12 mo : prompt repair is indicated.

71. SNU Children’s Hospital TAPVC Anatomical types

72. SNU Children’s Hospital TAPVC Repair Supracardiac type Infracardiac type

73. SNU Children’s Hospital Hypoplastic Left Heart Syndrome (HLHS)  A wide spectrum of cardiac anomaly with various degree of hypoplasia of the structure of the left side of the heart  Dx. is an indication of operation First stage palliation : 1-30days Norwood operation Second stage palliation : 6-12 mo BCPS Hemi-Fontan Third stage correction : 18-24 mo Completion Fontan Cardiac transplantation : Aortic diameter < 2.5 mm

74. SNU Children’s Hospital HLHS

75. Norwood Procedure

76. SNU Children’s Hospital Heart Transplantation Indications –Cardiac disease that has a poor patient prognosis for short-term survival (<1 year) and that is not treatable by conventional therapy Contraindications –High PVR (>4 wood units) –Multiorgan failure –Immune deficiency –Active infection –Neurologic or chromosomal abnormalities that impair survival

77. SNU Children’s Hospital Candidates for Heart Transplantation HLHS with TAPVC / with IAA Hypoplastic LV with hypoplastic ascending aorta AS with severe LV dysfunction s/p valvotomy AVSD unbalanced (hypoplastic LV) Truncus arteriosus with truncal valve stenosis Double inlet ventricle with TGA IAA type B with sever AS Subaortic AS with multiple VSDs or TV straddling PA IVS with Ebstein’s anomaly or RV-dependent coronary circulation TA with TGA or double orifice mitral vavle CCTGA with hypoplstic RV, complete heart block LA or RA isomerism Anomalous origin of left coronary artery CHD and CMP with biventricular outflow obstruction CMP, dilated or restrictive, hypoplastic RV Cardiac tumor

78. SNU Children’s Hospital Heart Transplantation