Download Congenital cardiac surgery

Congenital Cardiac Surgery
Yong Jin Kim, M.D.
Seoul National University Children’s Hospital
WWW.Drheart.net
History
Pre-Heart-Lung Machine Era

1938. Gross.
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1944. Crafoord. Resection of coarctation of aorta
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1945. Blalock.
Blalock-Taussig operation
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1946. Gross.
Surgical closure of AP window

1958. Glenn.
Glenn shunt
SNU Children’s Hospital
First successful PDA ligation
First Blalock-Taussig Shunt
“ Most powerful stimulus to the development of cardiac surgery ”
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
SNU Children’s Hospital
Era of Cardiopulmonary Bypass II

1959. Senning.
Atrial switch operation for TGA

1966. Ross.
Ross procedure for TOF with PA
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1971. Fontan.
Fontan operation for TA
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1975. Jatene.
Arterial switch operation for TGA
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1983. Norwood. Norwood procedure for HLHS

1985. Bailey.
SNU Children’s Hospital
Pediatric heart transplantation
Development of CPB

Prerequisites
– Understanding of physiology of circulation
– Preventing the blood form clotting
– Pumping blood to pump
– Ventilating the blood
SNU Children’s Hospital
Development of CPB
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1951. Dodrill.
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1952. Dodrill.
 1953. Lewis.
 1953. Gibbon.
 1954. Lillihei.

1954. Kirklin.
SNU Children’s Hospital
Mitral valve surgery under left heart
bypass
Relief of PS under right heart bypass
ASD closure under surface cooling
ASD closure by heart-lung machine
VSD closure under controlled crosscirculation
Establishment of CPB with oxygenator
in cardiac surgery
Controlled Cross-circulation
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SNU Children’s Hospital
1954. Lillehei
First surgical closure of
VSD under controlled
cross-circulation
Used in 45 patients
between 1954 to 1955
VSD
TOF
AVSD
Dr.Lillehei
J. Gibbon and Heart-lung Machine
SNU Children’s Hospital
Fontan Operation for Functional
Single Ventricle
Fnacis Fontan
SNU Children’s Hospital
SNU Children’s Hospital
CPB Circuit

Pump
 Oxygenator
 Heat exchanger
 Reservoir
 Filter
 Sucker & vent
 Cardioplegic solution
delivery system
SNU Children’s Hospital
SNU Children’s Hospital
Diseases
To be Corrected in Neonate

Critical LVOTO
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– Critical AS
– Interrupted aortic arch
– Symptomatic CoA
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Critical RVOTO
– PA with IVS
– PA with VSD
SNU Children’s Hospital
Critical pulmonary
venous return
– Obstructive TAPVC

Other complex heart
disease
– TGA
– Truncus arteriosus
– Hypoplastic left heart
syndrome
To be Corrected in Infancy

Pulmonary outflow
obstruction
– Functional single
ventricles
– TOF
– PA
– Critical PS
– TGA & CC-TGA
SNU Children’s Hospital

CHF
– LR shunt
( Large VSD, AVSD,
PDA, TAPVR etc.)
– Severe valvular
diseases
– Other LVOTO lesions
( IAA, CoA etc.)
– Other complex
anomalies
Palliative Surgery
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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
SNU Children’s Hospital
Palliative Surgery
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Increase pulmonary blood flow
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BT shunt
Potts shunt
Watterston shunt
Glenn shunt
RVOT reconstruction
Decrease pulmonary blood flow
– Pulmonary artery bancing
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Increase pulmonary-systemic mixing
– Rashikind
– Blalock-Hanlon
– Palliative Mustard or Senning
SNU Children’s Hospital
Rt. Modified BT Shunt
SNU Children’s Hospital
Pulmonary Artery Banding
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”)
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)
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.
SNU Children’s Hospital
Pulmoary Stenosis
PS – membranous type
SNU Children’s Hospital
Poststenotic dilatation
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
SNU Children’s Hospital
CoA Extended End-to-End Anastomosis
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
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Coexisting cardiac anomaly : not contraindication
One-stage repair : preferred
Two-stage repair : in complicated anomalies
Single ventricle associated : alternative plan
SNU Children’s Hospital
Interruption of Aortic Arch (IAA)
SNU Children’s Hospital
IAA type B - Operation
SNU Children’s Hospital
LVOTO

The various forms of
LVOTO occur in
combination with other
cardiac lesions (IAA, CoA,
MV anomalies, LV
hypoplasia)
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Obstructive types
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Supravalvular
Valvar
Subvalvular
intraventricular
SNU Children’s Hospital
Tunnel Stenosis – Konno Operation (1)
SNU Children’s Hospital
Tunnel Stenosis – Konno Operation (2)
SNU Children’s Hospital
Simple L-R Shunt
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Patent ductus arteriosus (PDA)
Atrial septal defect (ASD)
Ventricular septal defect (VSD)
Atrioventricular septal defect (AVSD)
Aortopulmonary window (AP window)
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 1st month
– Prophylactic closure : 6 -12 months
– Sx. of heart failure or failure to thrive : indicated at any time

Pulmonary vascular disease : contraindicated
SNU Children’s Hospital
PDA
SNU Children’s Hospital
PDA Ligation
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
SNU Children’s Hospital
ASD - Surgical Anatomy
SNU Children’s Hospital
Patch Closure of Secundum ASD
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
SNU Children’s Hospital
Types of VSD
SNU Children’s Hospital
VSD – PM Patch Closure
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
SNU Children’s Hospital
Types of AVSD
SNU Children’s Hospital
Partial AVSD
SNU Children’s Hospital
Complete AVSD
SNU Children’s Hospital
R-L Shunt
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Tetralogy of Fallot (TOF)
TOF with PA
Pulmonary atresia with intact ventricular septum
(PAIVS)
Ebstein’s anomaly
Tricuspid atresia
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
SNU Children’s Hospital
TOF
SNU Children’s Hospital
TOF – RVOT Patch Widening
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
SNU Children’s Hospital
Ebstein’s Anomaly
SNU Children’s Hospital
Ebstein’s Anomaly – Operation
(Danielson method)
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
SNU Children’s Hospital
Types of Tricuspid Atresia
SNU Children’s Hospital
Complex Cyanotic Lesions
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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)
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
SNU Children’s Hospital
Types of DORV
SNU Children’s Hospital
DORV – REV Operation
SNU Children’s Hospital
DORV (Taussig-Bing); Rastelli operation
SNU Children’s Hospital
Rastelli Operation ( Conduit Placement)
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.
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.
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–
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Systemic to pulmonary artery shunt
Pulmonary artery banding
Damus-Kaye-Stansel procedure, Norwood operation
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)
SNU Children’s Hospital
Lateral Tunnel Fontan
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
SNU Children’s Hospital
TGA – Operative View
SNU Children’s Hospital
Arterial Switch Operation
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.
SNU Children’s Hospital
TAPVC
Anatomical types
SNU Children’s Hospital
TAPVC Repair
Supracardiac type
SNU Children’s Hospital
Infracardiac type
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
SNU Children’s Hospital
HLHS
SNU Children’s Hospital
Norwood Procedure
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
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High PVR (>4 wood units)
Multiorgan failure
Immune deficiency
Active infection
Neurologic or chromosomal abnormalities that impair survival
SNU Children’s Hospital
Candidates for Heart Transplantation
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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
SNU Children’s Hospital
Heart Transplantation
SNU Children’s Hospital