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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%

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surgery for congenital heart diseases

Surgery for Congenital Heart Diseases

Seoul National University Children’s Hospital

Yong Jin Kim,M.D.

etiologic basis of congenital heart diseases
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





Sex hormone


Retinoic acid

2. Infections


Coxsakie virus

3. Maternal conditions

Old age




4. Others

Potential Cardiovascular Teratogens
maternal risk factors
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


herpes, coxsacki B,


maternal drug exposures
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
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 %

Incidence of Congenital Heart Diseases
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,


3) Syncope, chest pain

4) Symptoms of Hypoxemia


hypoxic spell,clubbing

Evaluation of CHD by History Taking
to be corrected in neonate
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
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


Double inlet ventricle

Tricuspid atresia




PA with IVS

Corrected TGA

Cardiac mass


Truncus arteriosus

Severe AS, AR

Severe MS, MR

Interrupted aortic arch


Aortopulmonary window




To Be Corrected in Infancy(II)
to be corrected in infancy childhood
To Be Corrected in Infancy & Childhood
  • ASD
  • 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
palliative surgery
Palliative Surgery
  • Systemic – pulmonary artery shunt

Blalock-Taussig shunt

Unifocalization and shunt

Cavopulmonary shunt (BCPS)

  • RVOT reconstruction


Patch widening

Valved conduit

  • Pulmonary artery banding
  • Atrial septectomy
systemic pulmonary artery shunt
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
  • 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
Atrial Septectomy
  • For the increasing of effective pulmonary

flow and systemic oxygen saturation

  • Indication of atrial septectomy :


Tricuspid atresia

Pulmonary atresia + IVS

MV and LV hypoplasia

  • Decreasing tendency of indication due to early total correction or intervention
reparative surgery
Reparative Surgery
  • Non-open heart surgery

Open heart surgery

  • Palliative procedure

Corrective procedure

    • Anatomic correction
    • Physiologic correction
non open heart surgery
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
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
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

One stage repair

vascular ring
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
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 (+)


  • Thoracotomy without CPB
  • Median sternotomy with CPB
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
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


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
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


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
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
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
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
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
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
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
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
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
Pulmonary Stenosis
  • 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
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
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
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
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
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
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
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
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


    • Single ventricle associated : alternative plan
hypoplastic left heart syndrome
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



    • Third stage correction : 18-24 mo

Completion Fontan

    • Cardiac transplantation :

Aortic diameter < 2.5 mm

truncus arteriosus
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 :


congenitally corrected tga
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
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
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
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


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
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


Subclavian-left coronary anastomosis

Tunnel operation

    • Ligation of LCA
atrial isomerism
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
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%