Surgery for Congenital Heart Diseases Seoul National University Children’s Hospital Yong Jin Kim,M.D.

Etiologic Basis of Congenital Heart Diseases 1. Primary genetic factors (10%) 1) Chromosomal ; 5-10% 2) Single mutant gene ; 3% Recessive Dominant 2. Genetic-environmental interaction (90%) 1) Multifactorial inheritance ; majority 2) Risks to offspring of an affected parent 3) Environmental contribution Drugs Infections Maternal conditions

Potential Cardiovascular Teratogens 1. Drugs Alcohol Amphetamines Anticonvulsants Chemotherapy Sex hormone Thalidomide Retinoic acid 2. Infections Rubella Coxsakie virus 3. Maternal conditions Old age Diabetes Lupus Phenylketonuria 4. Others

Maternal Risk Factors Factors Malformation Advanced age Trisomy 21 Maternal CHD Various Diabetes mellitus VSD, TGA, cardiomyopathy SLE Heart block Phenylketonuria TOF, VSD, COA, HLHS Viruses Teratogenic, myocarditis (*cytomegalovirus, herpes, coxsacki B, parvovirus)

Maternal Drug Exposures Drug Malformation Diphenylhydantoin PS, AS Trimetadione VSD, TOF, TGA, HLHS Thalidomode TOF, Truncus arteriosus Lithium Ebstein anomalies Alcohol VSD, ASD, PDA, TOF Amphetamine VSD, ASD, PDA, TGA Birth control pills VSD, TOF, TGA

Stages of Heart Formation 1. Early blood vessel formation 1) Intraembryonic blood vessel at 13 days 2) Extraembryonic blood vessels at 17 days 2. Development of heart 1) Position and cardiac tube at 23 days Heart beating at 26 days. 2) Formation of heart loop at 8 somites 3) Formation of ventricle 4) Development of sinus venosus 3. Formation of cardiac septa 4. Formation of cardiac valves 5. Formation of arterial system 6. Formation of great systemic veins

Incidence of Congenital Heart Diseases 1. Lt to Rt Shunt ( 53 % ) PDA 17 % ASD 16.5 % VSD 13 % AVSD 3.5 % Abn. PV return 3 % 2. Rt to Lt Shunt (11 % ) TOF 4.5 % TA 3 % PA+VSD 2.5 % PA+IVS 0.5 % 3. Admixture Lesion ( 15 % ) TGA 5 % Univ. Ht. 5 % Atrial isomerism < 2 % DORV < 2 % Truncus 0.8 % Corrected TGA < 0.5 % 4. Obstructive Lesion ( 15 % ) Coarctation 9.5 % PS 2 % MS etc. 1.5 % LVOTO 1.3 % HLHS 0.9 % IAA 0.6 % 5. Valvular Lesion Ebstein < 1 % AR < 0.5 % MR < 0.5 % SV aneurysm < 0.5 % 6. Miscellaneous Arrhythmia 5 % Vascular ring 0.5 %

Evaluation of CHD by History Taking 1. Infants 1) Murmur 2) Symptoms of CHF poor feeding, low weight gain, tachypnea, tachycardia, sweating, anxiety, irritability, frequent URI 3) Symptoms of hypoxemia cyanosis, hypoxic spell 2. Children 1) Murmur 2) Symptoms of CHF exercise intolerance, dyspnea on exertion, frequent URI, palpitation 3) Syncope, chest pain 4) Symptoms of Hypoxemia cyanosis, hypoxic spell,clubbing

To Be Corrected in Neonate Critical AS Hypoplastic left heart syndrome Mitral valve hypoplasia Aortic valve and arch hypoplasia Hypoplastic left ventricle Interrupted aortic arch Symptomatic COA TGA Obstructive TAPVC Truncus Arteriosus PA with IVS PA with VSD Other symptomatic complex heart diseases

To Be Corrected in Infancy(I) Cardiac anomalies with pulmonary outflow tract obstruction Double inlet ventricle Critical PS Tricuspid atresia DORV DOLV TGA TOF PA with or without VSD Corrected TGA

To Be Corrected in Infancy(II) Cardiac anomalies with CHF Large VSD AVSD Double inlet ventricle Tricuspid atresia TAPVC COA DOLV PA with IVS Corrected TGA Cardiac mass TGA Truncus arteriosus Severe AS, AR Severe MS, MR Interrupted aortic arch DORV Aortopulmonary window ALCAPA PDA TAPVR

To Be Corrected in Infancy & Childhood ASD AS(LVOTO) Partial AVSD Ebstein’s anomaly VSD with PS VSD PS Mitral stenosis Atrial isomerism Coronary artery anomaly (A-V fistula) Anomalous pulmonary venous drainage Valvular heart diseases Complex anomalies with previous palliation Late presenting cardiac anomalies Cardiomyopathy

Surgical Indications and Optimal Timing of Operation

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

Systemic–Pulmonary Artery Shunt Systemic–pulmonary artery shunt is indicated due to age, size, anatomy or other conditions when: Complex anomaly with severe cyanosis, irritability, hypoxic episode Critically ill neonates or infants due to decreased pulmonary flow Facilitating growth of hypoplastic pulmonary artery

Pulmonary Artery Banding Pulmonary artery banding is indicated to decrease pulmonary blood flow & protect vascular disease when: Control of congestive heart failure Complex or multiple VSD (+/- coarctation) Single ventricle, Tricuspid atresia without PS CPB medically contraindicated Protection of pulmonary vascular bed Single ventricle – Fontan operation Preparation of LV for arterial switch operation TGA with IVS / restrictive VSD

Atrial Septectomy For the increasing of effective pulmonary flow and systemic oxygen saturation Indication of atrial septectomy : TGA Tricuspid atresia Pulmonary atresia + IVS MV and LV hypoplasia Decreasing tendency of indication due to early total correction or intervention

Reparative Surgery Non-open heart surgery Open heart surgery Palliative procedure Corrective procedure Anatomic correction Physiologic correction

Non-open Heart Surgery Palliative procedure Corrective procedure PDA COA Vascular ring and sling Coronary artery anomalies Stenotic valvular diseases Inflow occlusion technique Instrumental dilatation

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

Coarctation of the Aorta Congenital narrowing of upper thoracic aorta adjacent to the ductus arteriosus Operation is indicated when : Reduction of luminal diameter > 50% Upper body HT > 150mmHg in young infant With CHF at any age COA with VSD Two stage repair One stage repair COA with other important intracardiac defects

Vascular Ring Anomalies of the great arteries that compress the trachea or esophagus Double aortic arch Rt aortic arch with retroesophageal anomalous Lt. SCA and ligamentum arteriosum Rt aortic arch with retroesophageal ligamentum arteriosum Operation is indicated when : Obstructive Sx and radiologic signs of obstruction Division by thoracotomy CPB in associated cardiac anomaly

Vascular Sling Lt. pulmonary artery arises anomalously from Rt pulmonary artery extrapericardially, form a sling around trachea Sx & Signs of obstruction : indicated Tracheal anomaly or other airway problems (+) (ASD, VSD, PDA, Lt SVC) Thoracotomy without CPB Median sternotomy with CPB

Open Heart Surgery

ASD and PAPVR 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

Unroofed Coronary Sinus Syndrome A spectrum of cardiac anomalies in which part or all of the common wall between the coronary sinus and left atrium is absent Operation is advisable with diagnosis when With persistent Lt SVC Arterial desaturation Risk of cerebral embolism Good results of operation Without persistent Lt SVC(Coronary sinus ASD) Same as for other type ASD Associated with other major cardiac anomalies Clear indication for operation

Total Anomalous Pulmonary Venous Connection These are no direct connection between any pulmonary vein and the LA. But 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 nearly always before 6 mo Dx at 6-12 mo : prompt repair is indicated

Ventricular Septal Defect A hole (or multiple holes) between Lt & Rt ventricle Symptomatic large VSD : an indication of operation Before 3 mo : indicated in large VSDs with CHF or respiratory symptoms Moderate sized VSDs (Qp/Qs < 3.0) with few symptoms : observation during infancy Small VSDs (Qp/Qs < 1.5) : not indicated, risk of bacterial endocarditis Subarterial type : early repair is indicated before childhood

Atrioventricular Septal Defect Abnormalities of atrioventricular valve form & function and interatrial & interventricular communication 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 Complete AVSD with refractory CHF or respiratory Sx : indicated promptly Development of pulmonary vascular obstructive disease : not indicated

Congenital Aortic Stenosis The various forms of LVOTO occur in combination with other cardiac lesions (IAA, COA, MV anomalies, LV hypoplasia) and obstructive types are supravalvular, valvular, subvalvular, intraventricular Critical AS in neonates : urgent (severe CHF, LV dilatation, hypertrophy) Infants and children Pressure gradient > 75mmHg Sx of angina, syncope, exercise intolerance, LVH, pressure gradient > 50mmHg Pressure gradient over 40mmHg in subvalvular lesion to prevent progression

Aortopulmonary Window Abnormal development of aortopulmonary septum caused by incomplete formation of the right and left conotruncal ridges. Symptomatic AP window is an indication Symptomatic infants : indicated with Dx Repair is advised before 3 mo of age (size, increased shunt, pulmonary hypertension) Old children should be operated on unless PVR render them inoperable

Aneurysm of Sinus of Valsalva Thin walled, tubular outpouchings, nearly always Rt sinus or adjacent half of the noncoronary sinus and with an entirely intracardiac course, that may rupture into the right (rarely left) heart chamber to form a fistula Ruptured sinus of Valsalva : indicated promptly because of abrupt development of CHF With VSD or VSD+AI : prompt repair is indicated Large aneurysms producing hemodynamic derangement : indicated Small and moderate sized aneurysm without symptom : not indicated

Cor Triatriatum A rare congenital cardiac anomaly in which pulmonary veins typically enter a proximal left atrial chamber separated from the distal left atrial chamber by a diaphragm in thich there are one or more restrictive ostia (sinister, dexter) Indication for operation Restrictive aperture in the partition is an urgent indication Symptoms usually develop early, and operation is necessary in the 1st year of life Life expectancy after repair in infancy is excellent

Congenital MS and MR A developmental malformation of one or more of the components of MV apparatus, including LA wall adjacent insufficiency or a combined lesion Infancy Mild and moderate Sx without MVR : Supravalvular ring, commmissural fusion Other circumstances : only for infants with heart failure Childhood Considerations for operation are similar CHF Severe pulmonary HT MVR should be withheld whenever possible

Ebsteins 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 first week Valve repair and ASD closure : with important TR moderate and severe cyanosis WPW syndromes : ablation of accessory conduction pathway

Pulmonary Stenosis Percutaneous balloon valvotomy A form of RV outflow obstruction in which stenosis is usually valvar or both valvar & infundibular or only infundibular Critical PS in neonate : indicated with Dx Percutaneous balloon valvotomy Valvotomy with CPB Transannular RVOT patch widening Valvotomy with inflow occlusion technique PS in infants and children : indicated with Sx & Pr gradient over 50mmHg Surgical treatment is not indicated with mild stenosis

PA with Intact Ventricular septum A cardiac anomaly in which the pulmonary valve is atretic, coexisting with variable degree of right ventricle and tricuspid valve hypoplasia Dx is an indication of operation Size of the TV : Z-value of the tricuspid valve < -4 --- Systemic-pulmonary artery shunt -2~-4 --- RVOT patch + shunt > -2 --- RVOT patch Evaluation after 6-12 mo after initial procedure : Two ventricle repair One and half ventricle repair Fontan procedure

Tetralogy of Fallot Characterized by underdevelopment of RV infundibulum with anterior and left ward displacement Dx is an indication of operation Symptomatic complicated in early life : Early total correction or Shunt (1-2 mo) and total correction (1 year) Asymptomatic uncomplicated : Total correction at 3-24 mo Multiple VSDs, LAD from RCA : Initial shunt and total correction

Pulmonary Atresia with VSD These are no luminal continuity between RV & pulmonary trunk among the group of TOF. It is usually congenital, but may be acquired. Dx is an indication of operation Confluent and normally distributing PAs: Complete repair in early life Palliation and repair (3-5 year) with conduit Confluent PAs distributing to the major segments : Pul. Segments > 15 --- need not unifocalization repair with conduit in 3-5 years Nonconfluent PAs distributing to the minor segment : Mmultistage operation with unifocalization Palliative shunt only

Double Outlet Right Ventricle A congenital cardiac anomalies 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 Simple DORV with subaortic VSD : repair by 6 mo with PS --- repair like TOF DORV with subpulmonic VSD (Taussig-Bing heart) : arterial switch operation within 1 mo with PS --- REV or Rastelli operation at 3-5 years DORV with noncommitted VSD : VSD enlargement and intraventricular tunnel Fontan operation

Transposition of Great Arteries A cardiac anomaly in which the Ao arises entirely or in large part from the RV, and PA from LV (atrioventricular concordant connection and ventriculoarterial discordant connection) Simple TGA in neonate : arterial switch operation within 1 mo Simple TGA beyond 30 days : rapid two-stage operation atrial switch operation (Mustard, Senning) TGA with VSD : arterial switch operation as early TGA with VSD and LVOTO Lecompte operation at 6-18 mo Rastelli or Lecompte operation at 3-5 years

Tricuspid Atresia A cardiac anomaly in which RV fails to open into a ventricle through a AV valve. There is thus a univentricular AV connection PVR is an important indicator > 4 unit -- contraindicaton 2-4 unit -- BCPS < 2 unit -- Fontan operation 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

Double Inlet Ventricle A cardiac anomaly in which both atria connect to only one ventricular chamber by either two separate AV valve or a common AV valve Dx is an indication of operation Septation : 1-2 years enlarged dominant ventricle two competent nonstenotic AV valve no pulmonary or systemic outflow obstruction Fontan operation : 1-2 years < 3-5 mo --- systemic outflow obstruction 6 - 12 mo --- BCPS 1 – 2 years --- Fontan operation Cardiac transplantation

Interrupted Aortic Arch Complete luminal and anatomic discontinuity between two segments of the aortic arch, and 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 intracardiac anomalies Single ventricle associated : alternative plan

Hypoplastic Left Heart Syndrome 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

Truncus Arteriosus A cardiac anomaly in which one great artery, arising from the base of the heart by way of a truncal valve, PAs proximal to the origin of the brachiocephalic branches. Presence of truncus arteriosus : an absolute surgical indication. Neonatal repair is recommended : Homograft interposition Prosthetic valve conduit Autologous tissue reconstruction Repair should be done before 6 mo Pulmonary vascular obstructive disease : contraindication

Congenitally Corrected TGA A cardiac anomaly with ventriculoarterial discordant connection & atrioventricular discordant connection. The circulatory pathways are therefore in series The presence of CCTGA per se is not an indication. With VSD : indications for VSD With VSD + PS : indications for TOF Isolated TR : indication for MR With complete heart block : pacing Fontan procedure indicated : straddling tricuspid valve complete AVSD left sided tricuspid valve incompetence Double Switch operation : anatomic correction

Anatomically Corrected Malposition of Great Arteries A cardiac anomaly in the position of the great arteries and not in cardiac connection. The LV probably always exhibits a well formed subaortic conus and the RV has less well developed infundibulum than normal Diagnosis is not an indication for operation Coexisting cardiac anomalies are indications Large VSD always present Pulmonary stenosis is usual Subaortic stenosis may occur Tricuspid atresia or TV hypoplasia in half

Double Outlet Left Ventricle A cardiac anomaly in which both great arteries arise from the left ventricle with nearly always with large VSD, and frequent occurrence of PS. Dx is an indication for op. Without PS : Performed in the 1st 6mo of life Banding and delayed repair Intracardiac baffling Conduit repair With PS : Classic shunt before the age of 2 years Simple intraventricular repair LeCompte procedure in infancy Conduit repair in childhood

Coronary Arteriovenous Fistula Direct communication between coronary artery and lumen of any one of cardiac chamber, or coronary sinus, or its tributary veins, or SVC, PAs, or Pulmonary veins close to the heart Indicated unless the shunt is small (Qp/Qs<1.3) Increase in size Sx and heart failure SBE Low probability of spontaneous closure Safety and efficacy of operation Techniques Suture ligation without CPB Closure of transcoronary artery with CPB Transcardiac chamber closure with CPB

Anomalous LCA From the PA Whole of the left main or LAD, or circumflex branch connects anomalously to main pulmonary artery or rarely from proximal RPA. Very rarely, both coronary arteries connect to the pulmonary artery by a single trunk. Urgent operation is indicated in infancy. The condition is an also indication for op in older patient as well. Construction of a two coronary system LCA transfer CABG Subclavian-left coronary anastomosis Tunnel operation Ligation of LCA

Atrial Isomerism A condition which the right sided and left sided atria, normally morphologically different, are morphologically similar. Surgical treatment is indicated with coexisting anomaly, not by the atrial isomerism. Intraventricular complex repair Complex atrial baffling Fontan type repair Palliative operation

Cardiac Transplantation Indications Cardiac diseases that has a poor prognosis for short-term survival(<1 year) and that is not treatable by conventional therapy Contraindications Severe and life-limiting coexisting medical problems Active bacterial or viral infection Psychosocial family situation Presence of severely elevated PVR (>8 Wood Units) Immunologic status of the recipient panel-reactive antibody (PRA) > 25%