Surgery II 4.

3b

CONGENITAL CARDIAC DISEASES

OUTLINE
I.
II.
III.

IV.

PATENT DUCTUS ARTERIOSUS (PDA)

Classification of Congenital Heart

Disease Accdg to Type of Shunting
Classification Based on Treatment
Option
Patent Ductus Arteriosus
a.
Embryology and Anatomy
b.
Fetal Circulation
c.
Definition
d.
Postnatal Closure
e.
Mechanism of Closure
f.
Determinants of Magnitude of
Shunting
g.
Physical Examination
h.
Natural Course
i.
Management
Atrial Septal Defect
a.
Types of ASD
b.
Diagnosis
c.
Spontaneous Closure
d.
Management

V.

VI.

VII.

Ventral Septal Defect

a.
Pathology
b.
Causes
c.
Classification Based on
Location
d.
Physiology
e.
Classification Based on
Defect Size
f.
Classical Manifestation
g.
Diagnosis
h.
Natural History
i.
Management
Tetralogy of Fallot
a.
Manifestation
b.
Diagnosis
c.
Management
Transposition of Great Arteries
a.
Pathophysiology
b.
Diagnosis
c.
Management

References
1. PowerPoint
2.
Recording
3.
Schwartzs Principles of Surgery

TRADITIONAL CLASSIFICATION OF CONGENITAL HEART DISEASES

(ACCORDING TO TYPE OF SHUNTING)

LEFT-TO-RIGHT
(ACYANOTIC)
Patent Ductus Arteriosus (PDA)
Atrial Septal Defect (ASD)
Ventricular Septal Defect (VSD)
Endocardial Cushion Defect (ECD)
Partial Anomalous Pulmonary
Venous Return (PAPVR)

RIGHT-TO-LEFT
(CYANOTIC)
Tetralogy of Fallot (TOF)
Transposition of the Great
Arteries (TGA)
Hypoplastic
Left
Heart
Syndrome (HLHS)

NEWER CLASSIFICATION
(BASED ON BEST TREATMENT OPTION)

REPAIR
ONLY
PALLIATION
ONLY

REPAIR OR
PALLIATION

Dr. VILLANUEVA
Nov. 10, 2014

PDA
Truncus arteriosus
ASD
TAPVR
Aortic stenosis
Cor triatriatum
Aortic coarctation
Tricuspid atresia
Hypoplastic Left Heart Syndrome (HLHS)
VSD
TOF
TGA
Taussig-Bing Syndrome (TBS) w/ or w/o pulmonary
stenosis (PS)
Atrioventricular canal defects
Interrupted aortic arch
Ebsteins Anomaly
DORV (Double Outlet Right Ventricle)
DORV with NON-committed VSD
DORV with Subaortic/doubly committed VSD w/o PS
DORV with Subaortic or doubly committed

Group 15 | Gi, Shei, Chup, Ella, Celene

Figure 1. Patent Ductus Arteriosus

EMBRYOLOGY AND ANATOMY
Ductus arteriosus develops from distal portion of the left 6th aortic
arch

A right PDA joins the right pulmonary artery and the right aortic arch
just distal to the right subclavian artery

A bilateral ductus may be present

Locally produced and circulating prostaglandins E2 and

prostaglandin I2 induce active relaxation of the ductal musculature,
maintaining maximal patency during the fetal period
**SEE APPENDIX C FOR PRENATAL VS POSTNATAL CIRCULATION

DUCTUS ARTERIOSUS IN THE FETAL CIRCULATION

At 6 weeks AOG, the DA is developed sufficiently to carry most of the
right ventricular output.
The placenta receives the largest amount of combined (i.e., right and
left) ventricular output (55%) and has the lowest vascular resistance
in the fetus
SVC: drains the upper part of the body
o the brain (15% of combined ventricular output)
o goes to the right ventricle (RV) PA DA descending
aorta
IVC: drains the lower part of the body and the placenta
o 70% of combined ventricular output
o Oxygen saturation in the IVC (70%) > in the SVC (40%)
o 1/3 blood is directed by the crista dividens to the left atrium (LA)
through the foramen ovale
o 2/3 enters the RV and pulmonary artery (PA)
DEFINITION OF PDA
Postnatal communication between the main pulmonary trunk and
descending thoracic aorta
Due to persistent patency of the fetal ductus arteriosus
Ductus arteriosus
o Normally connects the main pulmonary trunk or the proximal
left pulmonary trunk with the descending thoracic aorta
approximately 5 to 10 mm distal to the origin of the left
subclavian artery
o PDA shape conical; large aortic end tapering to smaller PA
end
o PDA length variable; few mm to cm.

EPIDEMIOLOGY OF PDA IN PRETERM INFANTS

Incidence of PDA in preterm infants: 8 in1,000 live births

o 45% PDA prevalence in infants <1,750 g birth weight
o 80% PDA prevalence in infants <1,200 g birth weight

Edited by: MJ NG

Page 1 of 12

SURGERY II 4.3B
EPIDEMIOLOGY OF PDA IN FULL TERM INFANTS

Incidence of PDA in full-term infants: 1 in 2000 live births

o 5% to 10% of all congenital heart lesions

2:1 female-to-male preponderance

Exposure to rubella during the first trimester of pregnancy

o 60% association in the cardiovascular system

Often associated w/ peripheral pulmonary stenosis & renal

artery stenosis

May be genetic

Leading cause of death in infants with large PDA: Congestive heart

failure (CHF) with respiratory infection as secondary cause

POSTNATAL CLOSURE OF THE DUCTUS ARTERIOSUS

FUNCTIONAL CLOSURE (10-15 HRS AFTER BIRTH)
Contraction and cellular migration of the medial smooth muscle in
the wall of the ductus arteriosus
o Shortening, increased wall thickness, protrusion into the lumen
of the thickened intima (intimal cushions or mounds)
ANATOMIC CLOSURE (BY 2 TO 3 WEEKS OF AGE)
Infolding of the endothelium, disruption and fragmentation of the
internal elastic lamina, proliferation of the subintimal layers,
hemorrhage and necrosis in the subintimal region
Permanent changes in the endothelium and subintimal layers of the
ductus
o Fibrosis results in permanent sealing of the lumen to produce
the ligamentum arteriosum

MECHANISM OF CLOSURE OF DUCTUS ARTERIOSUS AFTER BIRTH

A. Increase in pO2 with ventilation after birth

Fetal life pO2: 18- 28mmHg

postnatal increase in systemic pO2 (50 mm Hg after lung

expansion) is the strongest stimulus for constriction
B. Decrease in prostaglandins with increase in pulmonary blood flow

PGE2 is the more potent relaxer of the Ductus Arteriosus

PGE2 and PGI2 are formed intramurally in the Ductus Arteriosus

and exert their action locally on muscle cells

Due to low fetal pulmonary blood flow in utero, there is

decreased catabolism of PG in the lungs

At birth, there is marked increase in the pulmonary blood flow

allowing effective removal of the PG (decrease in PGE2 levels)
Note: The patency or closure of the DA represents a balance between the
constricting effects of oxygen, relaxing effects of prostaglandins, and
certain vasoconstrictive substances.

1.

2.

THREE DETERMINANTS OF THE MAGNITUDE OF PDA SHUNTING

Diameter and length of ductus arteriosus

After birth: degree of left-right shunts is regulated by size of the

duct
Relationship of the systemic (SVR) and pulmonary vascular
resistances (PVR)

16-18 weeks postnatally: PVR falls, shunt increases, flow

determined by relative resistance of pulmonary and systemic
circulations

With decrease in PVR, SVR becomes greater than PVR; leads to

left right shunt

Unrestricted ductal shunt leads to:

o Left ventricular volume overload
o Increased la and pa pressures
o Right ventricular strain due to augmented afterload

Group 15 | Gi, Shei, Chup, Ella, Celene

3.

Pressure difference between the aorta and pulmonary artery

SMALL PDA MODERATE
LARGE
FLOW RESISTANCE
High
Medium
Low
LEFT RIGHT SHUNT
Small
Medium
Large
PULMONARY BLOOD
No increase
Moderate
High
FLOW
LEFT VENTRICLE
None
Tolerable
Possible
HYPERTROPHY

SMALL PDA

MODERATE
PDA

LARGE PDA

CLINICAL MANIFESTATIONS
Few patients are symptomatic
Attention is often brought to this condition only by the
murmur
Detected at a routine physical examination
Symptomatology related to left ventricular failure
o Poor feeding, irritability, and tachypnea
o Poor weight gain
The symptoms increase until about the second to third
month of age
Symptoms indicative of severe left ventricular failure
with pulmonary edema may occur early in infancy
o Irritable, feed poorly, fail to gain weight, sweat
excessively
o Increased respiratory effort and respiratory rates
o Recurrent upper respiratory infections and
pneumonia

PHYSICAL EXAMINATION
**SEE APPENDIX A

Murmur
o Grade 1-4/6 continuous (machinery) murmur is best audible
at the left infraclavicular area or upper left sternal border
o The heart murmur may be crescendo systolic at the upper left
sternal border in small infants or infants with pulmonary
hypertension
o An apical diastolic rumble may be heard when the PDA shunt is
large

Hyperdynamic circulation with widened pulse pressure

Hyperactive precordium

NO cyanosis in uncomplicated PDA

Figure 2. Pattern of Murmur in PDA

LABORATORY RESULTS
Chest radiograph
o Increased pulmonary vascularity
o Cardiomegaly
ECG
o LV strain, LA enlargement, possible RV hypertrophy
Echocardiogram
o Demonstration of duct patency and shunt size

Edited by: MJ NG

Page 2 of 12

SURGERY II 4.3B

NATURAL COURSE
Spontaneous closure in premature infants.
In term infants spontaneous closure rarely occurs
CHF and/or recurrent pneumonia develop in large PDA
PVOD
Sub-acute bacterial endocarditic
Aneurysm rare
MANAGEMENT
MEDICAL
For preterm infants: Indomethacin or Ibuprofen (Contraindications:
renal insufficiency, necrotizing enterocolitis)
For term infants: those unresponsive to Indomethacin must undergo
mechanical closure
No exercise restriction is needed in the absence of pulmonary
hypertension
Prophylaxis for subacute bacterial endocarditis is indicated when
indications arise
Treatment of symptomatic patients with CHF
o Diuretics to decrease blood volume going to pulmo circulation
o Digoxin to increase heart contractility
o After-loader reducing agents to prevent blood shunt from
aorta; lower systemic resistance so blood goes preferentially to
systemic circulation)
CATHETER CLOSURE (STUDY)
COIL CLOSURE PROCEDURE
Treatment of choice for all children more than a few months of age
with ducts <3 mm in diameter
Performed using conscious sedation allowing the children to return to
full activity by the next day
A catheter is advanced from femoral artery or vein across the PDA
An occluding coil is placed in the PDA with a single coil loop on the
pulmonary artery side and the remaining three to four loops in the
ductal ampulla
>97% successful with zero mortality and no significant morbidity
Percutaneous technique
AMPLATZER DUCT OCCLUDER
For larger PDAs 12 mm in diameter
Procedure is similar in duration, risk, and recovery time to the coil
closure procedure
The devices are implanted antegrade from the femoral vein using
long sheaths sized 6 to 8 French.
> 98% closure rate at 6 months with minimal complications and no
mortality

PDAs larger than 12 mm

o Use of septal closure devices

AGA septal occluder, VSD device, NMT CardioSEAL device

o Covered stents in select cases
SURGICAL CLOSURE
Indication
o Anatomic existence of PDA regardless of size because of the
increased mortality and risk of endocarditis
Contraindication
o Presence of Pulmonary Veno-Occlusive Disease (PVOD)
Timing
o Between 6 months and 2 years of age or when the diagnosis is
made in an older child.
o In infants with congestive heart failure, pulmonary
hypertension or recurrent pneumonia, surgery is performed
on an urgent basis.
The surgical approach is the most common method used for closure
of PDA in newborns.
o Left posterolateral thoracotomy via the 3rd or 4th ICS (4th or 5th
ICS in Schwartz)
LIGATION
Lesser probability to kill patient and thus, it is a much common
[2]
procedure today; safer
The ductus is ligated with multiple large silk ligatures so that it is
obliterated in its entire course.
In neonates, PDA is singly ligated with surgical clip or permanent
suture
In smaller children, this usually amounts to two or three large silk
ligatures.
Care must be taken during the procedure to avoid the recurrent
laryngeal nerve, which usually courses around the PDA. In extreme
cases of PDA, the use of cardiopulmonary bypass (CPB) may be
needed to decompress the large ductus during ligation. [3]
TRANSECTION
Usually done in patients with a large PDA, especially older children
Adequate mobilization can afford the opportunity to clamp both the
pulmonary arterial and aortic ends. This is followed by the ductal
division and oversewing of the two stumps.
Advisable for short, broad ductus (width = length)
VIDEO-ASSISTED THORACOSCOPIC (VATS) CLIPPING
Seldom used because you can accidentally rupture PDA before you
[2]
can open the patient, he can already die.
This offers few advantages over the standard open surgical approach.

Figure 3. Non-surgical closure of PDA using Amplatzer occluder

Group 15 | Gi, Shei, Chup, Ella, Celene

Edited by: MJ NG

Page 3 of 12

SURGERY II 4.3B
ATRIAL SEPTAL DEFECT

Figure 4. Atrial Septal Defect.

Pathway of blood: From IVC to RA and goes through the interarterial
septum, crosses to LA LV then blood is ejected this is normal in
perinatal circulation, but abnormal postnatally.

Defined as an opening in the interatrial septum that enables the

mixing of blood from the systemic venous and pulmonary venous
circulation
F > M (2:1)
Occurs as isolated anomaly in 5-10% of all CHDs
30-50% of CHDs have ASD as part of cardiac defect
TYPES OF ASD

OSTIUM SECUNDUM
Most prevalent (80% of ASD)
Only type that may spontaneously close
Results from failure of closure in neonate

OSTIUM PRIMUM
Near the area of the valve
May lead to mitral regurgitation Heart failure

SINUS VENOSUS
Nearest to the roof of the heart
Partial anomalous pulmonary venous return
Ostium Primum and Sinus Venosus are both failure in the growth of
septum

LAB FINDINGS
Chest Radiograph: right atrial enlargement, right ventricular
enlargement, prominent pulmonary artery, increased pulmonary
vascular markings
ECG: right axis deviation, mild right ventricular hypertrophy, right
bundle-branch block
2D Echo
o 'Dropout' mid atrial septum indicative of an ostium secundum
type of ASD
o Drop out is lower ostium primum
o Drop out is higher sinus venosus
[2]
**Drop-out is the defect
Scimitar sign: true anomalous venous return of the right pulmonary
veins into the inferior vena cava (rather than directly to the left
atrium)

SPONTANEOUS CLOSURE RATE

100% spontaneous closure if <3mm at 1 y/o
80% spontaneous closure if 3-8mm at 1 y/o
>8mm rarely close spontaneously
87% overall spontaneous closure

MANAGEMENT
NONSURGICAL OR PERCUTANEOUS CLOSURE
Usually used for ostium secundum
Use of septal occlude or pericardium

Figure 5. Left to right: Ostium Primum, Ostium Secundum, Sinus Venosus

Auscultation: Murmur is due to flow across Pulmonic valve (soft

systolic murmur, loud P2)
o Prominence of the first heart sound with fixed splitting of the
second heart sound (results from the relatively fixed left-toright shunt throughout all phases of the cardiac cycle; the
increased blood flow across the pulmonic valve going to the
pulmonary arterial circulation causes a delay in the P2
component of the second heart sound)

SURGICAL CLOSURE
Surgery indication:
o Qp/Qs > 1.5:1
Qp pulmonary flow; Qs systemic flow
Contraindication: pulmonary vascular resistance ( 10 U/m2 )
Timing of surgery is delayed until 3-4 years old because of possible
spontaneous closure
Complications: CVA, arrhythmia
o Heart block: ostium primum AV node
o Sinus node dysfunction: sinus venosus posterosuperior of SVC

PRIMARY CLOSURE
Usually done in pediatric patients

PATCH CLOSURE
Using pericardium or Teflon
Usually done in adult patients; pericardium is usually used except for
high pressure like LV, synthetic grafts are used instead[2]

DIAGNOSIS
Associated with right ventricular hypertrophy from the very start; in
contrast to most L-R shunts where LVH is present
Usually asymptomatic (patients with complaints are usually in their
2ndto 3rd decade of life already)
The amount of blood shunted across the defect is low because the
atria are both low-pressure chambers.

Group 15 | Gi, Shei, Chup, Ella, Celene

Edited by: MJ NG

Page 4 of 12

SURGERY II 4.3B
VENTRICULAR SEPTAL DEFECT

Figure 6. Ventricular Septal Defect

Most common form of congenital heart disease

VSD in newborns: 5-50/1000
Most common lesion in many chromosomal syndromes
56: 44 (F/M)
Multifactorial etiology : hereditary and environmental
PATHOLOGY
Normal development of the heart: the interventricular foramen
closes and becomes the membranous part of the interventricular
septum
Failure of the interventricular foramen to close: Ventricular Septal
Defect
o Left-to-right ventricular shunt during systole
CAUSES [1]
Deficient development of the proximal conus swellings.
Failure of the muscular portion of the interventricular septum to fuse
with the free edge of the conus septum. (Membranous VSD)
Failure of the endocardial cushions to fuse.
Excessive diverticulation of the muscular septum- perforations in the
muscular interventricular septum. (Muscular VSD)
Depending on the cause of the VSD, that would be the type or
classification of VSD, the structure that did not fuse.
Four components of the ventricular septum:
o Inlet septum
o Trabecular septum
o Outlet or infundibular septum
o Membranous septum

CLASSIFICATION BASED ON LOCATION

PERIMEMBRANOUS (INFRACRISTAL, MEMBRANOUS)
Most common type of defect
Involves the membranous septum and includes the misalignment
defects seen in Tetralogy of Fallot
Usually extends into muscular, inlet or outlet areas
Minor anomalies of the tricuspid valve forming as extra septal leaflets
or pouches can partially or completely occlude the defect -- called the
aneurysms
Age of optimum surgery: 2 year old if symptomatic or 5 year old if
asymptomatic
Highest chance of spontaneous closure

OUTFLOW
Outlet, supracristal, infundibular, doubly committed subarterial
5 to 7%
A defect within the conal septum
Beneath the pulmonary valve
No chance of spontaneous closure

Group 15 | Gi, Shei, Chup, Ella, Celene

INLET
Atrioventricular canal defects
5 to 8%
Part or all of the septum of the AV canal is absent
Posterior and inferior to perimembranous defect, beneath the septal
leaflet of tricuspid valve and inferior to the papillary muscles
MUSCULAR
5 to 20%
Can occur anywhere along the trabecular septum
o Central: mid-muscular, may have multiple apparent channels on
right ventricle side; coalesce to a single defect on left ventricle
side
o Apical: multiple apparent channels on RV, maybe single defect in
LV side as with central defect
o Marginal: along septal margin
o Swiss cheese septum: large number of muscular defects
[1]

A.

B.

PHYSIOLOGY (not discussed)

DEFECT SIZE: primary anatomic variable determining physiologic
state

Small or medium size: limits LR shunt

Large: no resistance to flow across the defect

PULMONARY VASCULAR RESISTANCE: determines magnitude of
shunting in infancy

The small, muscular pulmonary arteries in the fetus become

thin-walled with increased luminal size following birth.

Normal rate of decline in PVR that accompanies the above

changes: right ventricular pressure reaches the adult values
within 7-10 days

CLASSIFICATION BASED on DEFECT SIZE[1]

Basis of classification by size is relative to the size of aortic valve

SMALL VSD OR
MEDIUM-SIZED
LARGE- SIZED
ROGETS DEFECT
</= 1/2 of the
Size of the aortic
<1/3 of aortic root
aortic orifice
orifice
Large systolic pressure
difference between
Systemic pressure in
>/=20 mmHg
the 2 ventricles (high
both ventricles
resistance to flow)
Gradual decline in
No tendency for
Unusual to have
PVR 1st few months
increase in pulmonary
marked elevation
of life LA and
vascular resistance
of PVR
pulmonary HPN
Moderate LR
Large LR shunts
shunt (volume
(volume overload of
Continuous LR shunt
overload of LA &
LA & LV and LVH)
LV and LVH)
CHF: 2-8 weeks old
CLINICAL MANIFESTATIONS

[1]

SMALL

MEDIUM

LARGE

Murmur: 1-6 weeks

Course benign Risk:
endocarditis (rare before
2 y/o) Appears healthy

Symptoms: 2 weeks old

(tachypnea, excessive
sweating, fatigue, during
feeds) May be preceded by
respiratory infection

Poor weight
gain Signs of
CHF

Normal precordial
activity (+/-) thrill in the
lower left sternal border
associated with Grade 46 holosystolic murmur
Heart sound: usually
normal

Hyperdynamic precordium
Extended L & R ventricular
areas (+) thrill, holosystolic
murmur S2 widely split P2
normal or slightly increased

Left anterior
precordial
bulge after
4-6 months

Edited by: MJ NG

Page 5 of 12

SURGERY II 4.3B

SMALL

DIAGNOSIS[1]
MEDIUM

ECG: Normal

LVH/LAH

CXR: Normal

LVE, LAE Increased

pulmonary
vascular markings

LARGE
Combined ventricular
hypertrophy
Biventricular enlargement
Increased pulmonary vascular
marking

Figure 7. Radiographic findings of

Medium VSD (LEFT) vs Large VSD (RIGHT)

NATURAL HISTORY
Spontaneous Closure most small VSDs, 20-30% close before 1 year
old
Congestive heart failure large VSDs
o Extra blood flows from the LV thru the RV to the lungs LA
LV causes LA and LV overload increase workload on the
heart increased HR and bodys demand for energy
o Extra blood flow in the lungs rapid breathing, also increasing
the bodys demand for energy
Growth failure seen in infants
o Baby is not able to eat enough to keep up with the bodys
increased energy demands
Bacterial endocarditis
Arrhythmia
Pulmonary HPN sustained flow under higher pressure into the
pulmonary arteries causes the arteries to become thickened and stiff.
The amount of blood flow to the lungs decreases over time as the
resistance to blood flow into the pulmonary arteries increases.
However, this causes the right ventricle to work harder.

Figure 8. VSD Repair (RA approach).

Defect(Orange-red)Tatapalan lang ng patch (yellow) yung butas

TETRALOGY OF FALLOT

MANAGEMENT
NONSURGICAL
Small: None except antibiotic prophylaxis
Moderate to large:
o Furosemide (1-3 mg/k/day)
o High caloric diet
o Captopril (0.1 -0.3 mg/kg TID)
o Digoxin if diuresis and afterload reduction not enough and
surgery not advisable

SURGICAL APPROACH
Indications

Uncontrolled CHF, including growth failure, recurrent respiratory

infection
o
Control CHF through diuretics or Lanoxin; treat any infection; if
not controlled, then do surgery. If controlled, wait for patient to
grow up a little before doing surgery.

Large defects prior to 2 y/o even without symptoms if with

pulmonary artery hypertension

Older, asymptomatic children with normal pulmonary artery pressure

if the pulmonary-systemic flow ratio (Qp/Qs) >2:1
Group 15 | Gi, Shei, Chup, Ella, Celene

Requires the use of cardiopulmonary bypass with moderate

hypothermia and cardioplegic arrest
How to expose the anomalies?
o Right atrial approach is preferable for most defects (regardless
of the type of defect present, this is the approach used for the
initial inspection of the cardiac anatomy)
o Left ventriculotomy is usually used for apical muscular defects
After careful inspection of the heart for any associated
malformations, a patch repair is used to repair the septal defect,
taking care not to avoid the conduction system.
If a definitive VSD closure cannot be accomplished such in Swisscheese VSD, temporary placement of pulmonary artery band can be
used to control pulmonary flow. This allows time for spontaneous
closure of many of the smaller defects, thus simplifying surgical
repair.

Figure 9. Tetralogy of Fallot: VSD, pulmonary stenosis,

overriding of the aorta and RV hypertrophy

Arises as a result of the underdevelopment and anteroleftward

malalignment of the infundibular septum
10% of all congestive heart diseases
Most common cyanotic heart defect beyond infancy
Components
o VSD large perimembranous VSD adjacent to tricuspid valve
o pulmonary stenosis - right ventricular outflow tract obstruction
o overriding of the aorta
o RV hypertrophy
o If with Atrial Septal Defect PENTALOGY
Edited by: MJ NG

Page 6 of 12

SURGERY II 4.3B

MANIFESTATION
Cyanosis (significant when 6-12mos of life), tachypnea, clubbing
Grade 3-5/6 systolic ejection murmur
Polycythemia develops secondary to cyanosis
Hypoxic spells in infants (tet spells), periods of extreme hypoxemia
Subacute bacterial endocarditis a common complication
DIAGNOSIS
ECG: right axis deviation, right ventricular hypertrophy, combined
ventricular hypertrophy, right atrial hypertrophy
CXR: boot-shaped heart (couer en sabot)
Fig11. TOF with Modified Blalock-Taussig Shunt
Pag maliit ang pulmonary artery (due to stenosis), you cannot do an
outright connection. So, we increase the blood flow to pulmonary arteries.
We create a shunt shunt systemic blood to PA, para dumami yung blood
na dadaan sa lungs. Thus, more oxygenated blood to go to LA and LV.
[3]

TRANSATRIAL REPAIR
Use of transannular patch
It involves the use of cardiopulmonary bypass
TRANSPOSITION OF GREAT ARTERIES

Figure 10. Chest X-ray of TOF. Boot-shaped heart is produced

because PA is blocked by pulmonary stenosis.
MANAGEMENT
NONSURGICAL

Propranolol
Treat Fe deficiency anemia
Dental hygiene & subacute bacterial endocarditis prophylaxis
Balloon dilatation of RVOT & pulmonary valve
SURGICAL APPROACH
Optimal age of approach is not yet determined. Currently primary
elective repair is preferred for infancy[3]
Surgical correction of TOF can either be:
o a staged approach of antecedent palliation in infancy followed
by intracardiac repair
o primary repair during first few months of life without palliative
surgery.
Primary goal: increase the flow of blood going to the pulmonary
circulation. For total correction of TOF, the stenotic outflow tract is
opened and the septal defect is repaired (i.e. patch closure). This is
not possible at all times, which is why palliation may be initially
required.
DISADVANTAGE: it has the resultant scar that would significantly
[3]
impair right ventricula function that may lead to lethal arrhythmias
MODIFIED BLALOCK-TAUSSIG SHUNT (BTS)
Antecedent palliation with the use of systemic-to-pulmonary shunts
is preferred in unstable infants younger than 6 months of age
o Those who have pulmonary atresia, significant branch
pulmonary artery hypoplasia or require an extracardiac conduit
because of an anomalous left anterior descending coronary
artery
3% mortality rate
Complications: right bundle-branch block, complete heart block,
congestive heart failure, pulmonary regurgitatation

Group 15 | Gi, Shei, Chup, Ella, Celene

Figure 12. Transposition of the Great Arteries.

Aorta from the Right Ventricle, Pulmonary artery from the Left Ventricle

Connection of the atria to their appropriate ventricles with

inappropriate ventriculoarterial connections
Your pulmonary and systemic circulation, instead of series, naging
parallel. So, umiikot-ikot lang yung blood. For the patient to survive,
there should be a shunt. It can be in the form of an ASD, VSD, or PDA,
as long as there is connection between left and right because of the
parallel circulation.
7-8 % of all CHD
Male predominance; M:F =2:1
75% with interventricular septum
o
Simple TGA: with Patent Ductus Arteriosus
o
Complex TGA:

20% with Ventricular Septal Defect

20% with Left Ventricular Outflow Tract Stenosis

7-10% with Aortic Arch Obstruction

PATHOPHYSIOLOGY
Transposition of Great Arteries (TGA) results in parallel pulmonary
and systemic circulations
o Oxygenated blood circulates thru the lungs & the left side of the
heart while deoxygenated blood circulates thru the systemic
circulation and the right side of the heart.
o This is incompatible with life needs an intracardiac shunt
(e.g., ASD, VSD, PDA) to survive.

Edited by: MJ NG

Page 7 of 12

SURGERY II 4.3B

After birth, both ventricles are relatively noncompliant and thus,

infants initially have higher pulmonary flow
Left atrial
enlargement
Left-to-right shunt via patent foramen ovale
o Pulmonary blood flow > systemic blood flow
o PULMONARY VASCULAR DISEASES Right Ventricular
Hypertrophy (because the right ventricle works against systemic
vascular resistance)
DIAGNOSIS
ECG: RVH
CXR: classic egg-shaped consideration
CARDIAC CATHERIZATION
o Rarely used, usually in infants requiring surgery after the
neonatal period to assess the suitability of the LV to support the
systemic circulation

ADDITIONAL NOTES(NOT DISCUSSED)

COR TRIATRIATUM

Figure 15.CorTriatriatum.
(A) Common chamber draining to right atrium directly. (B) Common
chamber draining into systemic venous circulation via an anomalous vein.

MANAGEMENT
ARTERIAL SWITCH OPERATION by JATENE

PATHOPHYSIOLOGY AND DIAGNOSIS

Results in obstruction of the pulmonary venous return to the left
atrium
If communication between superior and inferior chamber <3 mm,
patients are symptomatic during first year of life
The afflicted infant will present with the stigmata of
o Low cardiac output
o Pulmonary venous hypertension
o Congestive Heart Failure
o Poor feeding
Physical examination may demonstrate
o Loud pulmonary S2 sound
o Right ventricular heave
o Jugular venous distention
o Hepatomegaly
Chest radiograph: cardiomegaly and pulmonary vascular prominence
ECG: right ventricular hypertrophy
2D echocardiography provides a definitive diagnosis

THERAPY
Cardiopulmonary bypass & cardioplegic arrest are used.
Just remove the membrane

Figure 13. JATENE switch procedure.

A coronary arteries are removed from the aorta
B coronary arteries are connected to pulmonary artery (PA)
C Aorta and pulmonary artery is transected above level of coronary
ostia, then aorta is translocated posteriorly [LeCompte maneuver]
D Aorta is attached to the previous PA and becomes the neoaorta.

A rare CHD characterized by the presence of a diaphragm or

membrane that partitions the left atrium into two chambers:
o Superior chamber: receives drainage from the pulmonary veins
o Inferior chamber: communicates with the mitral valve and the
left ventricle

Good candidates for arterial switching procedures

o LV pressure > 85%
o LV post wall thickness > 4.5 mm
o Coronary pattern amenable to transfer to neoaorta with
distention or kinking
o LV inflow and outflow must be free of significant structural
abnormalities
2015B: The most important consideration is the timing of the surgical
repair because it should be performed within 2 weeks after birth,
before the left ventricle loses its ability to pump against systemic
afterload.

TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION

SENNING OPERATION

Figure 16. Comparison between TAPVR and A Normal Heart.

Characterized by the abnormal drainage of the pulmonary veins into

the right heart, whether through connections into the right atrium or
into its tributaries

Fig 14. Senning Operation.

Group 15 | Gi, Shei, Chup, Ella, Celene

Edited by: MJ NG

Page 8 of 12

SURGERY II 4.3B

The only mechanism by which oxygenated blood can return to the

left heart is through an ASD, which is almost uniformly present with
TAPVC.
Unique to this lesion is the absence of a definitive form of palliation.
Thus, TAPVC with concomitant obstruction represents one of the only
true surgical emergencies across the entire spectrum of congenital
heart surgery.
ANATOMY AND EMBRYOLOGY
TAPVC arises when the pulmonary vein evagination from the
posterior surface of the left atrium fails to fuse with the pulmonary
venous plexus surrounding the lung buds.
Obstruction to pulmonary venous drainage is a powerful predictor of
adverse natural outcome.
PATHOPHYSIOLOGY AND DIAGNOSIS
Because both pulmonary and systemic venous blood return to the
right atrium in all forms of TAPVC, a right-to-left intracardiac shunt
must be present for the afflicted infant to survive.
The child with TAPVC may present with severe cyanosis and
respiratory distress necessitating urgent surgical intervention if a
severe degree of pulmonary venous obstruction is present.
Two-dimensional echocardiography is very useful in establishing the
diagnosis. It can usually identify the pulmonary venous connections.
Cardiac catheterization is not recommended in these patients
because the osmotic load from the IV contrast can exacerbate the
degree of pulmonary edema. When cardiac catheterization is
performed, equalization of oxygen saturations in all four heart
chambers is a hallmark finding in this disease because the mixed
blood returned to the right atrium gets distributed throughout the
heart.
THERAPY
Operative correction of TAPVC requires the following:
o Anastomosis of the common pulmonary venous channel to the
left atrium (uses a baffle to direct the blood from the pulmonary
veins into the left atrium)
o Obliteration of the anomalous venous connection
o Closure of the ASD
The perioperative care of these infants is crucial because episodes of
pulmonary hypertension can occur within the first 48 hours.
o This contributes significantly to mortality following repair
o Muscle relaxants and narcotics should be administered during
this period to maintain a constant state of anesthesia.
The most significant postoperative complication of TAPVC repair is
Pulmonary Venous Obstruction.

o
o

AORTIC COARCTATION

Predominantly involves intercostals and mammary arteries

This translates into the well-known finding of the ff:

"Rib-notching" on chest radiograph

Prominent pulsation underneath the ribs

Other associated anomalies may be seen with COA but the most
common is that of a bicuspid aortic valve
PATHOPHYSIOLOGY
Symptoms develop with left ventricular outflow obstruction - This
translates to backflow to the pulmonary circulation which causes
pulmonary overcirculation
Later in the disease the patient develops biventricular failure.
Systemic hypertension also develops as a result of the ff:
o Obstruction to ventricular ejection
o Hypoperfusion which activates reninangiotensinaldosterone
system (RAAS)
Early surgical correction may prevent the development of long-term
hypertension which causes the development of aneurysms, aortic
dissection and myocardial infarction later in life.
DIAGNOSIS
COA is seen in two stages of life:
o In the newborn period if other anomalies are present
o Present in the late adolescent period with the onset of left
ventricular failure
Physical examination presents with the ff:
o Hyperdynamic precordium with a harsh murmur localized to the
left chest and back
o Femoral pulses dramatically decreased when compared to upper
extremity pulses
o Cyanosis may be apparent until ductal closure
Echocardiography will demonstrate narrowed aortic segment
THERAPY
Management of COA in all age groups has traditionally been
surgical.
The most common surgical techniques
o Resection with end-to-end anastomosis
o Extended end-to-end anastomosis

Allows treatment of transverse arch hypoplasia

May promote arch growth

Not feasible when segment is long or with previous surgery

The most common complications after COA repair at the repair site
are late restenosis & aneurysm formation
Particularly common after patch aortoplasty when using Dacron
material COA
TRUNCUS ARTERIOSUS

Figure 17. Sites of Aortic Coarctation

ANATOMY
Defined as a luminal narrowing in the aorta that causes an
obstruction to blood flow, usually located distal to the left subclavian
artery.
Causes extensive collateral circulation

Group 15 | Gi, Shei, Chup, Ella, Celene

Figure 18. Truncus arteriosus

ANATOMY AND EMBRYOLOGY

A rare anomaly characterized by a single great artery that arises
from the heart -It overrides the ventricular septum, and supplies all
circulations (pulmonary, systemic, and coronary circulations).
Edited by: MJ NG

Page 9 of 12

SURGERY II 4.3B

The two major classification systems are those of

o Collett and Edwards

This focuses on the origin of the pulmonary arteries from

the common arterial trunk

Type I: truncus one pulmonary artery two lateral

pulmonary arteries

Type II: truncus two posterior/posterolateral pulmonary

arteries

Type III: truncus two lateral pulmonary arteries

o Van Praagh

Based on the presence or absence of a VSD formation, the

degree of formation of the aorticopulmonary septum, and
the status of the aortic arch

Figure 19. Collete Edwards (Type I-III) and Van Praagh (A1-A4)

During normal embryonic life, truncusarteriosus normally begins to

separate and spiral into a distinguishable anterior pulmonary artery
and posterior aorta.
Persistent truncus represents an arrest in embryologic development
at this stage.
Neural crest plays a crucial role in the normal formation of the great
vessels.
o Neural crest also develops into pharyngeal pouches that give rise
to the thymus and parathyroid glands.
o This explains the prevalent association of truncus arteriosus and
DiGeorge syndrome.
In majority of cases, the leaflets are thickened and deformed, which
leads to valvular insufficiency.
o usually three leaflets (60% of cases)
o bicuspid (50% of cases)
o quadricuspid valve (25% of cases)

Truncusarteriosus usually present in the neonatal period, with signs

and symptoms of
o CHF and mild to moderate cyanosis due to left-to-right shunting
o A pansystolic murmur at the left sternal border
Chest radiography will be consistent with
o Pulmonary overcirculation
o Right aortic arch can be appreciated (~35%)
ECG: Non-specific; normal sinus rhythm with biventricular
hypertrophy
Echocardiography with Doppler color flow or pulsed Doppler is
diagnostic, provides information to determine
o The type of truncus arteriosus
o The origin of the coronary arteries, and their proximity to the
pulmonary trunk
o The truncal valves, and the extent of truncal insufficiency
Cardiac catheterization can be helpful in cases where pulmonary
hypertension is suspected.
The presence of truncus is an indication for surgery and repair should
be undertaken in the neonatal period, or as soon as the diagnosis is
established.
Eisenmenger's physiology found in older children is the only
absolute contraindication to correction surgery.
Repair is completed by
o Separation of the pulmonary arteries from the aorta
o Closure of the aortic defect (occasionally with a patch) to
minimize coronary flow complications
AORTOPULMONARY WINDOW

PHYSIOLOGY AND DIAGNOSIS

Figure 21. Aortopulmonary window

Figure 20. Truncus Arteriosus. Note the Purple lines.

The truncusarteriosus fails to properly divide into the pulmonary trunk
and aorta.

The two main pathophysiologic consequences of truncusarteriosus

are:
o The mixing of systemic and pulmonary venous blood that leads
to arterial saturations near 85%
o The presence of a nonrestrictive left-to-right shunt, which occurs
during both systole and diastole, the volume of which is
determined by the resistances of the pulmonary and systemic
circulations
The presence of these lesions often results in severe heart failure and
cardiovascular instability early in life

Group 15 | Gi, Shei, Chup, Ella, Celene

A rare congenital lesion characterized by incomplete development of

the septum that normally divides the truncus into the aorta and the
pulmonary artery
PATHOPHYSIOLOGY AND DIAGNOSIS
A large left-to-right shunt with increased pulmonary flow PLUS the
early development of CHF
o Like other lesions with left-to-right flow, the magnitude of the
shunt is determined by the size of the defect, as well as the
Pulmonary Vascular Resistance
Infants with APW present with frequent respiratory tract infections,
tachypnea with feeding, and failure to thrive
Cyanosis is usually absent because these infants deteriorate before
the onset of significant pulmonary hypertension
THERAPY
All infants with APW require surgical correction once the diagnosis is
made.
Repair is undertaken through a median sternotomy and the use of a
cardiopulmonary bypass.
Edited by: MJ NG

Page 10 of 12

Surgery II 4.3b

Dr. VILLANUEVA
Nov. 10, 2014

CONGENITAL CARDIAC DISEASES

APPENDIX A. PHYSICAL EXAMINATION FINDINGS OF PDA DEPENDING ON ITS DIAMETER AND LENGTH
PHYSICAL EXAMINATION
PERIPHERAL
PULSE

ARTERIAL
PULSE
PRESSURE

PRECORDIAL
ACTIVITY

HEART SOUND

SMALL PDA

May be full

Slightly
increased

MODERATE
PDA

Increased
HR
Bounding

Wide
(Low
diastolic
Pressure)

Hyperdynamic
Thrusting
apical pulse

S1 and S2
difficult
to
hear due to
loud murmur
S3 apex

LARGE PDA

Increased
HR
Bounding

Wide

Markedly
hyperdynamic
Thrusting left
ventricular
apical impulse

S1 and S2
accentuated
S3 Apex

Normal

S1 and S2
Normal

MURMUR

OTHERS

In early infancy
May be a short period when no murmur is
heard
Short systolic murmur may then be heard
In late infancy to older children
Typical, continuous murmur heard best in the
second lics
Often accentuated at recumbent position or
during inspiration
Continuous murmur:
o More intense, more extensive radiation, and
well heard posteriorly
o Much harsher quality with low-frequency
components
o Eddy sounds that vary from beat to beat
give the murmur a machinery quality
No murmur is heard with severe failure
With control of left ventricular failure:
moderately loud systolic murmur in the
pulmonary area or occasionally in 3rd or 4th ics
The typical continuous murmur is less usual
Prominent mid-diastolic mitral flow rumble is
commonly audible at the apex

Systolic thrill may

be palpable at the
ULSB

If w/ pulmonary
edema, rales on
all lung fields

APPENDIX B. DIAGNOSTIC TEST FINDINGS OF PDA

CXR
SMALL
PDA

MEDIUM
PDA

Normal
Slight prominence of MPA

Cardiomegally
o LVH and LAE
prominent MPA
increased PVM
prominent ascending
unfolding of arch

LARGE PDA

ECG

2D Echo

Normal

Delineate the PDA size and flow

patterns

Demonstrate LAE, LVH and PDA

Flow and velocity patterns

with

Left ventricular hypertrophy (LVH) in

older infants and children
Left atrial enlargement (LAE) may be
present

Markedly enlarged MPA

Accentuated
pulmonary
vascular
markings are markedly
Interstitial fluid
Enlarged left atrium or pulmonary
arteries
Lobar collapse or emphysema owing to
bronchial compression may occur

Prominent LVH, with deep Q and taller R

waves
T waves diphasic or even inverted
RVH may be evident, w/ upright T waves
in the right precordial leads and
increased R-wave amplitude in the right
precordial leads
LAE, widened P wave

LAE and ventricular diameters,

hypertrophy if present, and the PDA
itself
Doppler evaluation will demonstrate
flow and velocity patterns and will
allow for an estimate of pulmonary
arterial pressure

Group 15 | Gi, Shei, Chup, Ella, Celene

aorta

Edited by: MJ NG

Page 11 of 12

Surgery II 4.3b

CONGENITAL CARDIAC DISEASES

Dr. VILLANUEVA
Nov. 10, 2014

APPENDIX C. PRENATAL VS POSTNATAL CIRCULATION AS WELL AS THE ANATOMIC CHANGES

Group 15 | Gi, Shei, Chup, Ella, Celene

Edited by: MJ NG

Page 12 of 12