CongenitalCardiacSurgery

Congenital Cardiac Surgery : 

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

History : 

History

Pre-Heart-Lung Machine Era : 

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

First Blalock-Taussig Shunt : 

First Blalock-Taussig Shunt “ Most powerful stimulus to the development of cardiac surgery ”

Era of Cardiopulmonary Bypass I : 

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

Era of Cardiopulmonary Bypass II : 

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

Development of CPB : 

Development of CPB Prerequisites Understanding of physiology of circulation Preventing the blood form clotting Pumping blood to pump Ventilating the blood

Development of CPB : 

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

Controlled Cross-circulation : 

Controlled Cross-circulation 1954. LilleheiFirst surgical closure of VSD under controlled cross-circulation Used in 45 patients between 1954 to 1955 VSDTOFAVSD Dr.Lillehei

J. Gibbon and Heart-lung Machine : 

J. Gibbon and Heart-lung Machine

Fontan Operation for Functional Single Ventricle : 

Fontan Operation for Functional Single Ventricle Fnacis Fontan

CPB Circuit : 

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

Diseases : 

Diseases

To be Corrected in Neonate : 

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

To be Corrected in Infancy : 

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

Palliative Surgery : 

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

Palliative Surgery : 

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

Rt. Modified BT Shunt : 

Rt. Modified BT Shunt

Pulmonary Artery Banding : 

Pulmonary Artery Banding

Classification of Congenital Cardiac Diseases : 

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”)

Pure Obstructive Lesions : 

Pure Obstructive Lesions Pulmonary stenosis (PS) Mitral stenosis (MS) Left ventricular outflow tract obstruction (LVOTO) Coarctation of aorta (CoA) Interrupted aortic arch (IAA)

Pulmonary Stenosis (PS) : 

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.

Pulmoary Stenosis : 

Pulmoary Stenosis PS – membranous type Poststenotic dilatation

Coarctation of Aorta (CoA) : 

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

CoA Extended End-to-End Anastomosis : 

CoA Extended End-to-End Anastomosis

Interruption of Aortic Arch : 

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

Interruption of Aortic Arch (IAA) : 

Interruption of Aortic Arch (IAA)

IAA type B - Operation : 

IAA type B - Operation

LVOTO : 

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

Tunnel Stenosis – Konno Operation (1) : 

Tunnel Stenosis – Konno Operation (1)

Tunnel Stenosis – Konno Operation (2) : 

Tunnel Stenosis – Konno Operation (2)

Simple L-R Shunt : 

Simple L-R Shunt Patent ductus arteriosus (PDA) Atrial septal defect (ASD) Ventricular septal defect (VSD) Atrioventricular septal defect (AVSD) Aortopulmonary window (AP window)

Patent Ductus Arteriosus : 

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

PDA : 

PDA

PDA Ligation : 

PDA Ligation

Atrial Septal Defect : 

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

ASD - Surgical Anatomy : 

ASD - Surgical Anatomy

Patch Closure of Secundum ASD : 

Patch Closure of Secundum ASD

Ventricular Septal Defect : 

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

Types of VSD : 

Types of VSD

VSD – PM Patch Closure : 

VSD – PM Patch Closure

Atrioventricular Septal Defect : 

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

Types of AVSD : 

Types of AVSD

Partial AVSD : 

Partial AVSD

Complete AVSD : 

Complete AVSD

R-L Shunt : 

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

Tetralogy of Fallot : 

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

TOF : 

TOF

TOF – RVOT Patch Widening : 

TOF – RVOT Patch Widening

Ebstein’s Anomaly : 

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

Ebstein’s Anomaly : 

Ebstein’s Anomaly

Ebstein’s Anomaly – Operation(Danielson method) : 

Ebstein’s Anomaly – Operation(Danielson method)

Tricuspid Atresia : 

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

Types of Tricuspid Atresia : 

Types of Tricuspid Atresia

Complex Cyanotic Lesions : 

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)

Double Outlet Right Ventricle : 

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

Types of DORV : 

Types of DORV

DORV – REV Operation : 

DORV – REV Operation

DORV (Taussig-Bing); Rastelli operation : 

DORV (Taussig-Bing); Rastelli operation

Rastelli Operation ( Conduit Placement) : 

Rastelli Operation ( Conduit Placement)

Univentricular Heart : 

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.

Surgical Strategy for Single Ventricle (1) : 

Surgical Strategy for Single Ventricle (1) Neonatal periodIf 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

Surgical Strategy for Single Ventricle (2) : 

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)

Lateral Tunnel Fontan : 

Lateral Tunnel Fontan

Transposition of Great Arteries : 

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

TGA – Operative View : 

TGA – Operative View

Arterial Switch Operation : 

Arterial Switch Operation

Total Anomalous Pulmonary Venous Connection (TAPVC) : 

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.

TAPVCAnatomical types : 

TAPVCAnatomical types

TAPVC Repair : 

TAPVC Repair Supracardiac type Infracardiac type

Hypoplastic Left Heart Syndrome (HLHS) : 

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

HLHS : 

HLHS

Norwood Procedure : 

Norwood Procedure

Heart Transplantation : 

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

Candidates for Heart Transplantation : 

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

Heart Transplantation : 

Heart Transplantation