1. Surgery for Congenital Heart Diseases
By: Dr. Shkar R. Saeed

2. Etiologic Basis of Congenital Heart Diseases
1. Primary genetic factor (10%)
1) Chromosomal; %
2) Single mutant gene; %
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

3. 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

4. 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)

5. 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

6. classification of Congenital Heart Diseases
1. Lt to Rt Shunt ( 53 % )
PDA %
ASD %
VSD %
AVSD %
2. Rt to Lt Shunt (11 % )
TOF %
TA %
PA+VSD %
PA+IVS %
3. Admixture Lesion ( 15 % )
TGA %
Univ. Ht %
DORV < 2 %
Truncus %
Corrected TGA < 0.5 %
4. Obstructive Lesion ( 15 % )
Coarctation %
PS %
MS etc %
LVOTO %
HLHS %
IAA %
5. Valvular Lesion
Ebstein < 1 %
AR < 0.5 %
MR < 0.5 %
6. Miscellaneous
Arrhythmia %
Vascular ring %

7. 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

8. 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

9. To Be Corrected in Neonate
Critical AS
Hypoplastic left heart syndrome
Interrupted aortic arch
Symptomatic COA
TGA
Truncus Arteriosus
Other symptomatic complex heart diseases

10. 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

11. Surgical Indications and Optimal Timing of Operation

12. 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

13. 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

15. 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

17. 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

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

20. Non-open Heart Surgery
Corrective procedure
PDA
COA
Vascular ring and sling
Coronary artery anomalies
Stenotic valvular diseases
Instrumental dilatation

21. 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

24. 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

28. Open Heart Surgery

29. 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

32. 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

33. 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

36. 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

37. 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

38. 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

40. Pulmonary Stenosis Percutaneous balloon valvotomy
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

41. 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

42. 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

43. 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

44. 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 months
Asymptomatic
Fontan candidate : months

45. 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