Download Surgery for Congenital Heart Diseases

By: Dr. Shkar R. Saeed
Etiologic Basis of Congenital Heart
Diseases
1. Primary genetic factor
(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
Advanced age
Maternal CHD
Diabetes mellitus
SLE
Phenylketonuria
Viruses
(*cytomegalovirus,
herpes, coxsacki B,
parvovirus)
Malformation
Trisomy 21
Various
VSD, TGA, cardiomyopathy
Heart block
TOF, VSD, COA, HLHS
Teratogenic, myocarditis
Maternal Drug Exposures
 Drug
• Diphenylhydantoin
• Trimetadione
• Thalidomode
• Lithium
• Alcohol
• Amphetamine
• Birth control pills
Malformation
PS, AS
VSD, TOF, TGA, HLHS
TOF, Truncus arteriosus
Ebstein anomalies
VSD, ASD, PDA, TOF
VSD, ASD, PDA, TGA
VSD, TOF, TGA
classification of Congenital Heart Diseases
1.
Lt to Rt Shunt
PDA
ASD
VSD
AVSD
( 53 % )
17 %
16.5 %
13 %
3.5 %
2. Rt to Lt Shunt
TOF
4.5 %
TA
PA+VSD
PA+IVS
(11 % )
3 %
2.5 %
0.5 %
3. Admixture Lesion
TGA
5 %
Univ. Ht.
DORV
Truncus
Corrected TGA
5 %
<2 %
0.8 %
< 0.5 %
( 15 % )
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 %
6. Miscellaneous
Arrhythmia
Vascular ring
5 %
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
Investigations
1.CXR
Integral part of evaluation
TOF(boot shaped heart)
TGA(egg on side)
Dilated PA (PHTN)
2.Echo(TTE,TEE)
3. MRI
4. Cardiac catheterization(exact anatomy, PA pressure
messurements,Qp/QA messure, occluders and dilators
To Be Corrected in Neonate
 Critical AS
 Hypoplastic left heart syndrome
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Interrupted aortic arch
Symptomatic COA
TGA
Truncus Arteriosus
Other symptomatic complex heart diseases
To Be Corrected in Infancy
 Cardiac anomalies with pulmonary outflow
tract obstruction
• Critical PS
• Tricuspid atresia
• TGA
• TOF
• PA with or without VSD
• Corrected TGA
Surgical Indications and
Optimal Timing of Operation
Palliative Surgery
 Systemic – pulmonary artery shunt
Blalock-Taussig shunt
Cavopulmonary shunt (BCPS)
Hemifontan & Fontan procedures
 RVOT reconstruction
Valvotomy
Patch widening
Valved conduit
 Pulmonary artery banding
 Atrial septectomy
Systemic–Pulmonary Artery Shunt
( Qp<Qs i.e cyanosis)
 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
Qp>Qs i.e more Pulm. flow
 Pulmonary artery banding is indicated to decrease
pulmonary blood flow & protect vascular disease when:
• Control of congestive heart failure
Complex or multiple VSD (+/- coarctation)
CPB medically contraindicated
• Protection of pulmonary vascular bed
Atrial Septectomy
 For the increasing of effective pulmonary
flow and systemic oxygen saturation
 Indication of atrial septectomy :
TGA
Tricuspid atresia
Pulmonary atresia
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

Corrective procedure
PDA
COA
Vascular ring and sling
Coronary artery anomalies
Stenotic valvular diseases






Instrumental dilatation
Patent Ductus Arteriosus
Open communication usually between upper
descending Aorta and proximal portion of LPA
 Significant PDA : indicated after 1st month
 Prophylactic closure : 6-12 months
 Symptoms 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
 COA with other important intracardiac defects
One stage repair
Open Heart Surgery
ASD with or without 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) : is an indication of surgery.
• Optimal age : under 5 years but recently 1-2 years to
avoid RV volume overload
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
Diagnosis is an indication of operation
• Immediate repair with Diagnosis in any ill neonate :
Preoperative preparation is not needed
• Repair should be done nearly always before 6 months
• Diagnosis at 6-12 months: 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 months: 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 Diagnosis
• Partial AVSD : 1-2 years of age except CHF or growth
failure
• Complete AVSD with good condition : 3-6 months
• 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
Symtoms of angina, syncope, exercise intolerance, LVH,
pressure gradient > 50mmHg
Pressure gradient over 40mmHg in subvalvular lesion
to prevent progression
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
• Valve repair and ASD closure :
with important TR, moderate and severe cyanosis
• WPW syndromes :
ablation of accessory conduction pathway
Pulmonary Stenosis

A form of RV outflow obstruction in which stenosis is
usually valvar or both valvar & subvalvularor only
subvalvular
•
Critical PS in neonate : indicated with Dx
Percutaneous balloon valvotomy
Valvotomy with CPB
Transannular RVOT patch widening
•
•
PS in infants and children : indicated with Symptoms &
Pr gradient over 50mmHg
Surgical treatment is not indicated with mild stenosis
Tetralogy of Fallot
Characterized by underdevelopment of RV outflow
Diagnosis is an indication of operation
• Symptomatic complicated in early life :
Early total correction or
Shunt (1-2 months) and total correction (1 year)
• Asymptomatic uncomplicated :
Total correction at 3-24 months
• Multiple VSDs, LAD from RCA :
Initial shunt and total correction
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
Transposition of Great Arteries
 A cardiac anomaly in which the Aorta 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 months
• Simple TGA beyond 30 days :
atrial switch operation (Mustard, Senning)
• TGA with VSD :
arterial switch operation as early
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 -- contraindication
2-4 unit -- BCPS
< 2 unit -- Fontan operation
• Symptomatic in early life
early shunt or PAB
BCPS or hemi-Fontan at 6-12 months
Fontan at 12-24 months
• Asymptomatic
Fontan candidate : 12-30 months
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
• With complete heart block : pacing
• Double Switch operation : anatomic correction