khaikwan, nophawan

lariosa, michael jerome
le, man
liao, karen isabel
libres, kimberly
pandac, therese rondell
pangan, efrea lyndee
perocho, zichri keren
santos, edilberto de belen
thanathada, sunklang
tonagaya, sarah
yee, ma. stephanie
zamora, mary rose sugar

CASE 1
 During a routine athletic physical, a 15

year old boy is found to have a systolic
thrill that is palpable at the lower left
sternal border accompanied by a harsh,
pansystolic murmur that is heard best at
the site of the thrill. He is asymptomatic
and has no evidence of hypertension,
cyanosis or edema. An electrocardiogram
and chest radiograph are normal.

Clinical Scenario
 Male, 15 year old
 Presence of systolic thrill palpable at




the lower left sternal border
Accompanying sign: harsh, pansystolic
murmur heard best at site of thrill
Asymptomatic
No hypertension, cyanosis or edema
ECG Normal
Chest radiography Normal

HEART
 A hollow muscular organ
 Located in the thorax

between 2 lungs
 4 chambers
 4 valves
 2 atria (atrium) and 2

ventricles
 2 separate pumps (R & L

sides)
 Right side receives blood

from the body and send it
to the lungs (pulmonary)
 Left side receives blood

from the lungs and sends it
to the body (systemic)

 The heart lies

between the
lungs in the
region called the
mediastinum. The
heart is wrapped
by some
membranes that
also hold the
heart in its
position relative
to the diaphragm
and the lungs.

Position of the Heart

Pulmonary and Systemic
Circulation

Pericardium
 The pericardium is the set of membranes




around the heart. It is actually composed
of three layers of membranes.
Visceral pericardium- the innermost
Parietal pericardium- the middle,
Fibrous pericardium- the outer one is the
extra one and is tough.
Pericardial Activity- tiny space between
the visceral pericardium and the parietal
pericardium.

Layers of the Heart

Chambers of the Heart

Valves of the Heart
4 valves
One way flow
Leaky valve=heart murmur
2 atrioventricular valves
>Left AV valve-bicuspid or mitral
>Right AV valve-tricuspid
 2 semilunar valves
>Pulmonic semilunar valve
>Aortic Semilunar valve



Valves of the Heart

Atrioventricular Valves
1. Right AV valve

Between the right atrium and right ventricle

Also called the tricuspid valve because it has three
cusps

Cusps close when right ventricle contracts…preventing
blood from going back up into the right atrium
2. Left AV valve

Between the left atrium and the left ventricle

Also called the bicuspid valve because it only has 2
cusps

Also called the mitral valve

Cusps close when left ventricle contracts…preventing
blood from back-up into the left atrium

Semilunar Valves
1. Pulmonary semilunar valve
 When right ventricle contracts,
blood is forced through this valve to
enter pulmonary trunk.
2. Aortic semilunar valve
 When left ventricle contracts, blood
is forced through this valve to enter
the aorta.

Cardiac Conduction
System
 The cardiac conduction system

generates and transmits impulses
that stimulate contraction of the
myocardium.
 Under normal circumstances, the
conduction system first stimulate the
contraction of the atria and then the
ventricles.

Cardiac Cycle
 Refers to the events of one complete heartbeat.

The length of the cardiac cycle is usually about
0.8 sec.
 Systole (contraction of the muscle)- there is
ventricular pumping, the chambers of the heart
become smaller as the blood is ejected. Occurs
secondary to the depolarization of cells.
 Diastole (relaxation of the muscle)- there is
ventricular filling, the heart chambers fill with
blood in preparation for subsequent ejection.

Cardiac Output
 Volume of blood ejected per minute

-Each ventricle ejects approximately
70ml of blood/beat
 Averages between 4-8L/min
 CO=Stroke volume x Heart rate
=70ml x 60 beats/min
=4,200 ml/min

Anatomy of the
Interventricular Septum

The interventricular septum is the wall of the heart intermediate to
the right and left ventricles. Its surface markings correspond to the
anterior and posterior interventricular grooves. It runs obliquely to
the left and slightly inferiorly from posterior to anterior. Due to this
angulation, the right ventricle tends to lie anteriorly and the left
ventricle posteriorly.

Throughout most of its surface area, the septum is as muscular as
the left ventricle. It tends to bulge into the chamber of the right
ventricle producing a concavity on the left ventricular side. Nearer
to the aortic valve orifice beneath the margins of the right and
posterior leaflets, the septum becomes thinner and more fibrous.
This region is termed the pars membranacea septi, or the
membranous part of the interventricular septum. It is oval in shape.

The muscular and membranous parts of the interventricular
septum have different developmental origins. The membranous
part of the septum is the most frequent site for ventricular septal
defects which are usually congenital.

Ventricular Septation

By the start of the fifth
week of gestation, there
are two primitive
ventricles in continuity:
the left one is derived
from the true primitive
ventricle; the right is
derived from the bulbus
cordis. Both differentiate
to become trabeculated rough-surfaced internally. The muscular
ridge between them on
the caudal part of the
inner wall starts to
elongate cephalically by
the growth of cells on the
outside of the chamber
and the regression of cells
internally. This sheet is
termed the muscular
interventricular septum.

The growth of the muscular
septum does not extend as far
as the endocardial cushions.
The space between the two
provides a means of
communication between the
ventricles; it is termed the
interventricular foramen.
Eventually, the foramen is filled
by:

the inferior elongation of the conus
septum, the dividing sheet across
the conus cordis
growth of cells from the inferior
endocardial cushion along the
superior margin of the
intermuscular septum;

The interventricular foramen
forms the membranous part of
the interventricular wall in the
mature, normal heart.

a.
b.
c.

Patent ductus arteriosus (PDA)
Tetralogy of fallot
Tricuspid regurgitation

Ventricular septal defect
Murmur
Location: 3rd , 4th and 5th left
interspaces
Radiation: often wide
Intensity: often very loud, with
a thrill
Pitch: high holosystolic
Quality: often harsh

Patent ductus arteriosus Tetralogy of fallot
Timing
Primary four malformations
•Pulmonary stenosis
Continuous murmur in
•Overriding aorta
both systole and diastole,
•Right ventricular hypertrophy
often with a silent interval
•Ventricular septal defect(VSD)
late in diastole. Loudest in
late systole, obscures S2, and
fades in diastole.

Tricuspid regurgitation
Murmur
Location: lower left sternal
border
Radiation: to the right of
the sternum, to the xiphoid
area, and perhaps to the left
midclavicular line, but not the
axilla
Intensity: variable
Pitch: medium
Quality: blowing
holosystolic
Aids: unlike mitral
regurgitation, the intensity
may increase slightly with
inspiration.

Associated findings
Location
•S2 may be obscured by the loud
Left 2nd interspace
murmur
Radiation
• Findings vary with the severity of
Toward the left clavicle
the defect and with associated
Intensity
lesions
Usually loud, sometimes
associated with a thrill
Quality
Harsh, machinery-like
Pitch
Medium

Additional anomalies
Associated findings
1.stenosis of the left pulmonary artery, •the right ventricle impulse is
in 40% of patients
increased in amplitude and
2.a bicuspid pulmonary valve, in 40% of may be sustained.
patients
•An S3 may be audible along
3.right-sided aortic arch, in 25% of
the lower left sternal boarder.
patients
The jugular venous pressure is
4.coronary artery anomalies, in 10% of
often elevated, with large v
patients
waves in the jugular veins.
5.a foramen ovale or atrial septal
defect, in which case the syndrome is
sometimes called a pentalogy of
Fallot
6.an atrioventricular septal defect
7.partially or totally anomalous
pulmonary venous return
8.forked ribs and scoliosis

Mechanism
A VSD is a congenital
abnormality in which blood flows
from the relatively high-pressure
left ventricle into the low-pressure
right ventricle through a hole.

Mechanism
Mechanism
Low oxygenation of blood due to the
When the tricuspid valve
mixing of oxygenated and
fails to close fully in systole,
deoxygenated blood in the left ventricle blood regurgitates from the
via the VSD and preferential flow of the right ventricle to right atrium,
mixed blood from both ventricles
producing a murmur. The most
through the aorta because of the
common cause is right

Ventricular septal
defect

• A ventricular septal defect (VSD) is a defect in

the ventricular septum, the wall dividing the left and
right ventricles of the heart.
• The ventricular septum consists of an inferior muscular
and superior membranous portion and is extensively
innervated with conducting cardiomyocytes. The
membranous portion, which is close to
the atrioventricular node, is most commonly affected in
adults and older children.
• Congenital VSDs are collectively the most
common congenital heart defects.

Diagnosis
• Detected by cardiac auscultation(due to

pathognomonic holo- or pansystolic murmur)
-- murmur depends on the abnormal flow of
blood from the left ventricle, through the
VSD, to the right ventricle.
• Confirmation of cardiac auscultation can be
obtained by non-invasive
cardiac ultrasound (echocardiography).
• More accurately measure ventricular
pressures-cardiac catheterization.

PDA

Patent ductus arteriosus
 heart condition that is normal but reverses soon after birth.
 Persistent PDA, there is an irregular transmission of blood

between two of the most important arteries in close proximity to
the heart.
 Ductus arteriosus normally seals off within a few days, in PDA,

the newborn's ductus arteriosus does not close but remains
patent.
 common in neonates with persistent respiratory problems such

as hypoxia, and has a high occurrence in premature children. In
hypoxic newborns, too little oxygen reaches the lungs to produce
sufficient levels of bradykinin and subsequent closing of the DA.
 Premature children are more likely to be hypoxic and thus have

PDA because of their underdeveloped heart and lungs.

MOA of PDA
 allows a portion of the oxygenated blood from the left

heart to flow back to the lungs (following the pressure
gradient from the higher pressure aorta to the
pulmonary arteries).
 If this shunt amount is substantial, the neonate becomes

short of breath because there is not only the normal
amount of unoxygenated blood that has returned from
the body to go to the lungs but in addition there is the
amount shunted through the PDA.
 The neonate's work of breathing is increased, using up

more calories and often interfering with feeding in
infancy. This condition as a constellation of findings is
called congestive heart failure.

Exemptions:
 In some cases, such as in transposition of the great

vessels (the pulmonary artery and the aorta), a
PDA may need to remain open. In this
cardiovascular condition, the PDA is the only way
that oxygenated blood can mix with deoxygenated
blood. In these cases, prostaglandins are used to
keep the patent ductus arteriosus open.

Diagnosis
 Usually diagnosed using non-invasive techniques.
 Echocardiography, in which sound waves are used to capture

the motion of the heart, and
 Associated Doppler studies are the primary methods of
detecting PDA. 
 Electrocardiography (ECG), in which electrodes are used to
record the electrical activity of the heart, is not particularly
helpful as there are no specific rhythms or ECG patterns which
can be used to detect PDA.
 Chest X-ray may be taken, which reveals the overall size of
neonate's heart (as a reflection of the combined mass of the
cardiac chambers) and the appearance of the blood flow to the
lungs ---small PDA most often shows a normal sized heart and
normal blood flow to the lungs --- large PDA generally shows an
enlarged cardiac silhouette and increased blood flow to the
lungs.

Tetralogy of Fallot
 Accounting for about 5% of all congenital

cardiac malformations
 is the most common cause of cyanotic
congenital heart disease
 The four features of the tetralogy are
        (1)VSD,
         (2) obstruction to the right ventricular
outflow tract (subpulmonic stenosis),
         (3) an aorta that overrides the VSD, and
         (4) right ventricular hypertrophy

Clinical features
 -          right-to-left shunting,
 -          decreased pulmonary blood flow, and increased aortic




volumes.
-          If the pulmonic obstruction is mild, the condition
resembles an isolated VSD, because the high left-sided
pressures on the left side cause a left-to-right shunt with no
cyanosis.
-          , marked stenosis causes significant right-to-left
shunting and consequent cyanosis early in life.
-          As patients with tetralogy grow, the pulmonic orifice
does not enlarge, despite an overall increase in the size of the
heart.
-          patients develop erythrocytosis with attendant
hyperviscosity, and hypertrophic osteoarthropathy;
-          the right-to-left shunting also increases the risk for
infective endocarditis, systemic emboli, and brain abscesses.

Diagnosis
 -          is suggested by history and clinical examination,
 -           supported by chest x-ray and ECG, and
 -          established by 2-dimensional echocardiography



with color flow and Doppler studies
-          Chest x-rays show a boot-shaped heart with a
concave main pulmonary artery segment and
diminished pulmonary vascular markings.
-          A right aortic arch is present in 25%.
-          ECG shows right ventricular hypertrophy and
may also show right atrial hypertrophy.
-          Cardiac catheterization is often indicated before
surgery to detect concomitant abnormalities that may
complicate surgical repair.

Tricuspid Regurgitation
In this condition, the valve does
not close properly and blood flows
back into the right atrium.

CAUSE
 Right ventricle enlarges and Increase resistance of

blood flow from the right ventricle to the lungs
 Resistance may be increased by a severe, longstanding lung disorder
 Emphysema
 Pulmonary Hypertension

 pulmonic stenosis
 left side of the heart disorder (such as mitral valve
stenosis)

To compensate, the right ventricle enlarges, stretching
the tricuspid valve and causing regurgitation.

Signs and Symptoms
 Weakness and fatigue.
 Pulsations in the neck from the

elevated Right Atrial pressure
 Discomfort in the right upper part of
the abdomen due to an enlarged liver
 Heart failure results in accumulation
of fluid in the body, mainly in the legs

Physical Diagnosis
 Echocardiography
 Chest x-ray

Morphology
 -          heart is large and "boot shaped" as a result of right




ventricular hypertrophy
-          the proximal aorta is typically larger than normal,
with a diminished pulmonary trunk.
-          The left-sided cardiac chambers are normal sized,
while the right ventricular wall is markedly thickened and
may even exceed that of the left.
-          The VSD lies in the vicinity of the membranous
portion of the interventricular septum, and the aortic valve
lies immediately over the VSD.
-          The pulmonary outflow tract is narrowed, and, in a
few cases, the pulmonic valve may be stenotic.
-          Additional abnormalities are present in many cases,
including PDA or ASD; these are actually beneficial in
many respects, because they permit pulmonary blood
flow.

Three Major Categories:
 Malformations causing a left-to-right

shunt
 Malformations causing a right-to-left
shunt
 Malformations causing an
obstruction

A.) Left-to-right shunts
 Atrial Septal Defect
 Ventricular Septal Defect
 PDA (Patent Ductus Arteriosus)
 Arterioventricular Septal Defect

Atrial Septal Defect

Atrial Septal Defect
Three major types:
 Secumdum ASD – 90% of most cases
 Primum ASD – 5% of anomalies
 Sinus venosus

Ventricular Septal
Defect

Ventricular Septal
Defect
 Membranous VSD – 90% involves the

membranous septum
 Infundibular VSD – lies below the
pulmonary valve
 Swiss-cheese septum – multiple
muscular VSD

PDA (Patent Ductus
Arteriosus)

Arterioventricular
Septal Defect

Arterioventricular
Septal Defect
 Partial AVSD – consist of primum ASD

and a cleft anterior mitral leaflet
 Complete AVSD – large combined AV
septal defect and a large common
AV valve

B.) Right-to-left shunts
(cyanotic)
 Tetralogy of Fallot
 Transposition of the Great Arteries
 Persistent Truncus Arteriosus
 Tricuspid Atresia
 Total Anomalous Pulmonary Venous

Connection

Tetralogy of Fallot

Tetralogy of Fallot
Four features of the Tetralogy of Fallot:
 VSD
 Obstruction to the right ventricular
outflow tract (subpulmonary
stenosis)
 Aorta that overrides the VSD
 Right ventricular hypertrophy

Transposition of the
Great Arteries

Truncus Arteriosus

Tricuspid Atresia

Total Anomalous Pulmonary
Venous Connection

C.) Obstructive
Congenital Disease
 Coarctation of the Aorta
 Pulmonary Stenosis and Atresia
 Aortic Stenosis and Atresia

Coarctation of the Aorta

Coarctation of the Aorta
Two classic forms:
 Infantile form with tubular hypoplasia
of the aortic arch proximal to a PDA
 Adult form which there is a discrete
rigde-like infolding of the aorta

Pulmonary Stenosis and
Atresia

Aortic Stenosis and
Atresia

Natural History
 Small (& even some larger)

lesions often close spontaneously either by
muscular growth or plugging with tricuspid
valve tissue. Larger lesions will either
cause problems due to the size of the
shunt (failure to thrive & recurrent chest
infections) or cause irreversible pulmonary
vascular disease. Life expectancy and
exercise capacity will therefore be
restricted.

 Congenital VSDs are frequently associated

with other congenital conditions, such as
Down syndrome.
 A VSD can also form a few days after a

myocardial infarction (heart attack) due to
mechanical tearing of the septal wall,
before
scar
tissue
forms,
when
macrophages start remodelling the dead
heart tissue.

Epidemiology and
Etiology
 VSDs are the most common

congenital cardiac anomalies. They are found in
30-60% of all newborns with a congenital heart
defect, or about 2-6 per 10,000 births. It is
debatable whether all those defects are true
heart defects, or if some of them are normal
phenomena, since most of the trabecular VSDs
close spontaneously. Prospective studies give a
prevalence of 2-5 per 100 births of trabecular
VSDs that closes shortly after birth in 80-90% of
the cases.

Prognosis
Transcatheter closure has been undertaken
for muscular defects and a device has just
become available for perimembranous
defects and is under evaluation. This is
excellent for most patients. The vast
majority are able to live a normal and
unrestricted life. Re-operations for residual
VSDs are now uncommon.

Congenital Heart
defects(VSD)

 Caused by developmental abnormalities.
 However the genes involved in these defects

have been identified in only a minority of
conditions.
 Well defined genetic or environmental
influence are identifiable in only 10% of the
cases of congenital heart ds (VSD…).
 There is an association of congenital cardiac
malformations with certain chromosomal
abnormalities: trisomies 13, 15, 18 and 21
and the Turner Syndrome.

VSD
 Congenital heart defect in a parent or

preceeding sibling is the greatest risk factor for
developing a cardiac malformation.
 Trisomy 21 (associated with Down Syndrome)
is the most common known genetic cause of
congenital heart disease.
 Environmental factors, such as congenital
rubella infection or teratogens are responsible
for additional cases.
 Multifactorial genetic, environmental, and
maternal factors account for the remaining
majority of cases in which a cause is not
apparent.

VSD
 Mutation of the gene that encodes the

transcription factor TBX5 has been shown to
cause VSD and ASD observed in Holt-Oram
syndrome which is a rare condition associated
with heart, arm and hand defects.
 Developmental errors in mesenchymal tissue
migration: anomalies of outflow tract, failure of
fusion and failure of septation.
 Other mechanisms include extracellular matrix
abnormalities and situs and looping defects and
endocardial cushions defects.

During ventricular
contraction, or systole,
some of the blood from
the left ventricle leaks
into the right
ventricle, passes
through the lungs and
reenters the left
ventricle via the
pulmonary veins and left

First, the circuitous
refluxing of blood
causes volume overload
on the left ventricle.

Second, because the left
ventricle normally has a
much higher systolic
pressure (~120 mm Hg) than
the right ventricle (~20 mm
Hg), the leakage of blood
into the right ventricle
therefore elevates right
ventricular pressure and
volume, causing
pulmonary hypertension with

This effect is more
noticeable in patients with
larger defects, who may
present with
breathlessness, poor
feeding and failure to
thrive in infancy. Patients
with smaller defects may be
asymptomatic.

 Symptoms
 VSD is an acyanotic congenital

heart defect, aka a Left-to-right
shunt, so there are no signs of
cyanosis.

Ventricular septal
defect is usually
symptomless at birth. It
usually manifests a few
weeks after birth.

Signs
Pansystolic /
Holosystolic murmur
(depending upon the size
of the defect)

CLINICAL MANIFESTATIONS

Two basic types of VSD
 Perimembranous VSD - an opening in

the upper section of the ventricular
septum, near the valves, occurs in 75%
of all VSD cases.
 Muscular VSD - an opening in the lower

section of the ventricular septum
occurs in up to 20% of all VSD cases.

NORMAL HEART SOUNDVENTRICULAR SEPTAL DEFECT
SOUND

 CLICK
ME!!!

 CLICK ME TOO!!!

Presentation

The size of the ventricular septal opening will
affect the type of symptoms noted, the
severity of symptoms, and the age at which
they first occur. A VSD permits extra blood to
pass from the left ventricle through to the
right side of the heart, and the right ventricle
and lungs become overworked as a result.
The larger the opening, the greater the
amount of blood that passes through and
overloads the right ventricle and lungs.

Most common symptoms of
VSD
fatigue
sweating
rapid breathing
heavy breathing
congested breathing
disinterest in feeding, or tiring while
feeding
 poor weight gain





Signs of VSD
 Murmur- due to the sound of blood

as it passes between the left and
right ventricles.
 Thrill- palpable murmur

 VSD will produce a

systolic murmur. The
classic murmur is grade
¾, holosystolic, harsh
and blowing in
character.It will peak in
midsystole. It is best
heard along the lower
left sternal border and
apex. Smaller defects of
small and medium
defects will typically
produce a louder sound
than a larger defect as
the blood velocity is
higher.

Small left-to-right
shunt
 1st heart sound at the apex is normal;

2nd sound at pulmonary area is split
physiologically
 Grade 2-4/6,medium-to high-pitched,
harsh pansystolic murmur heard best
at the left sternal border in the 3rd
and 4th ICS

Moderate left-to-right
shunt
 Slight prominence of precordium
 Moderate left ventricular heave
 Systolic thrill palpable at the lower

left sternal border between the 3rd
and 4th ICS
 Grade 3-4/6, harsh pansystolic
murmur

Large ventricular septal
defects w/ pulmonary
hypertension
 Precordium is prominent, sternum bulges
 Left ventricular thrust and right

ventricular heave are palpable
 Thrill may or may not be present at the
lower left sternal border
 S2 usually single or narrowly split, w/
acdentuation of pulmonary component
 Harsh, pansystolic murmur

SIMPLE RECALL

The most common forms
are in the muscular portion
of the septum where they
may
lie
posterior
(1),
apically (2) or anteriorly
(3). The perimembranous
(4) form is the next most
common. It may extend
posteriorly in the septum
(inlet) or anteriorly towards
the aortic valve (subaortic).
More rarely (except in
Asians) it is situated below
both
the
aortic
and
pulmonary valves doubly
committed (5).

 The

diagnosis of a VSD is usually
suspected clinically by hearing a
characteristic heart murmur. A murmur
is a sound generated by abnormally
turbulent flow of blood through the
heart. This murmur is the result of blood
being shunted through the VSD from the
higher-pressure left ventricle into the
lower-pressure right ventricle.
 The loudness of the murmur is related
to the size of the defect and amount of
blood crossing the defect.

Chest x-ray
 Chest X-ray looks to see if there is a large heart with fluid

in the lungs.
 Possible result: Small defects have a normal chest X-ray.
 Large defects with a big shunt have cardiomegaly and
pulmonary plethora.
A chest X-ray can also help follow the progression of
congestive heart failure by looking at the size of the
heart and the amount of blood flow to the lungs. This
may be normal at birth and change with time.

Electrocardiogram- ECG shows signs of an enlarged left ventricle.
Possible result: Small defects have a normal ECG. Large defects
with a big shunt have biventricular hypertrophy due to the
pressure load on the RV and the volume load on the LV. If the
shunt decreases due to either the development of pulmonary
stenosis or vascular disease then the volume load decreases on
the LV and the left ventricular hypertrophy resolves to leave
right ventricular hypertrophy.

echocardiogram
 Echocardiogram is used to make a definite diagnosis.
 This shows size & position of VSD (arrow) and thus assists with the

prognosis. It also allows identification of associated defects (ASD,
pulmonary stenosis, aortic, mitral valve & arch lesions) when present.
Colour Doppler is especially useful in identifying small defects.
 The size of the shunt can be numerically estimated by Doppler flow
techniques but the accuracy is poor and the method rarely used
clinically. A reasonable subjective impression can however be made
based on the LA and LV size which will be enlarged in a large shunt.
This may stretch the mitral valve ring causes mitral regurgitation
(LAVVR). 

The pulmonary artery pressure can usually accurately
be assessed using Doppler across the VSD. In the
example the VSD velocity which using the Bernoulli
equation (4V2) is equivalent to a pressure difference
between the LV and RV of 64 mm predicating a
normal PA pressure in most children.

Cardiac catheterization
- (rare) concerns of high blood pressure in the lungs esp. moderatesized defects
Cardiac catheterization is used to study the various functions of the
heart. Using different techniques, the coronary arteries can be viewed
by injecting dye or opened using balloon angioplasty. The oxygen
concentration can be measured across the valves and walls (septa) of
the heart and pressures within each chamber of the heart and across
the valves can be measured. The technique can even be performed in
small, newborn infants.

. Ventricular septal defect
shown intraoperatively

A large piece of pericardium
was used to close the defect

A VSD can be detected by

 cardiac auscultation.
 Classically, a VSD causes a pathognomonic

holo- or pansystolic murmu
 Auscultation is generally considered
sufficient for detecting a significant VSD.
 The murmur depends on the abnormal flow
of blood from the left ventricle, through the
VSD, to the right ventricle

A VSD can be detected by

 pulmonary hypertension
 This effect is more noticeable in

patients with larger defects, who
may present with breathlessness,
poor feeding and failure to thrive in
infancy. Patients with smaller defects
may be asymptomatic

Normal section through the
middle

Normal heart front view

VSD

CHF1 knockout mice have ventricular septal defects but no other organ
pathology

Sakata Y et al. PNAS 2002;99:16197-16202

©2002 by National Academy of Sciences

Membranous VSD

http://www.embryology.ch/
anglais/pcardio/patholcardio03.
html#AtrioventrikularerSeptum
defekt

Muscular VSD

Histological sections of mousehearts

VSD

VSD

Cardiomyopathy

Increased size of both cytoplasm
and nuclease

cardiomyocyte
multinucleation.

Myocyte hypertrophy

Histology of myocyte

Clinical Features
 Small VSDs may be

asymptomatic
 VSDs in the
muscular portion of
the septum may
close spontaneously
during infancy or
childhood
 Large defects cause
severe left to right
shunts
 ASDs often lead to
other cardiac
malformations or
secondary defects

Sequelae of Untreated Large
Ventricular Septal Defects

 Hypertrophied and dilated right ventricle
 Progressive pulmonary hypertension
 Shunt reversal

 Infective endocarditis
 Vascular changes leading to cardiac failure
 High output
 Subvalvular aortic stenosis (aortic valve incompetence)

Hypertrophied and Dilated
Right Ventricle

 Right QRS axis

deviation
 Ejection is
forceful but
ineffective
 Ventricular
arrhythmias
 Myocardial
ischemia leading
to anginal pain

Progressive Pulmonary
Hypertension
 Vascular hyperactivity that leads to




chronic vasoconstriction
Dyspnea on exertion
Cyanosis
Severe respiratory insufficiency
Chest pain
Shunt reversal

Effects of Shunt
Reversal
 Condition similar to the Tetralogy of

Fallot
 Decreased blood flow to the lungs 
Cyanosis
 Increased size of heart
 Increased risk of infective
endocarditis

Infective Endocarditis
 High pressure shunts increase

hemodynamic trauma to the endocardial
surface
 The creation of jet lesions in the right
ventricle
 Infection of the mural surface of the
endocardium
 Formation of vegetation (mass of
thrombotic debris and organism)

Vascular Changes Leading to
Cardiac Failure
 Heart is enlarged, flabby and its

pumps are inefficient
 Development of progressive
congestive heart failure
 Patient experience episodes of
angina pectoris
 As disease progresses, sudden death
is common

TRE ATMENT

 The medical treatment of infants with

ventricular septal defect (VSD) is directed at
the control of congestive heart failure.
 The goals of therapy are:
 To relieve symptoms.
 To minimize frequency and severity of

respiratory infections.
 To facilitate normal growth.

 Ventricular septum defect in infants is initially

treated medically with:
 Cardiac glycosides (e.g. 10-20mcg/kg/day)

- increases the strength of the heart muscle to
deal with the greater blood volume.
 Loop diuretics (e.g. furosemide 1-3 mg/kg/day)

- help remove excess fluid from the body so the
heart doesn't have to work as hard and the
patient feels much better.
 ACE inhibitors (e.g. captopril 0.5-2 mg/kg/day)

- used to decrease the work load on the left
ventricle.

 Patients with small VSDs do not require

treatment because approximately 80%
of such lesions heal spontaneously.
 Restricting the activities of a child with

an isolated VSD is rarely necessary.

 A VSD that either decreases in size or

closes completely during the first year
of life presents no problems to the
practicing physician.
 Older children with VSDs are seldom

symptomatic and require little in the
way of medical therapy.

Surgical Therapy
 Surgical closure is typically done before the

child begins preschool.

Surgery is indicated if medications do not
work in the first few months or years of life,
especially if the child is not growing
adequately even with medications.
 Surgery is more urgent if evidence of
pulmonary hypertension has developed.

 VSD surgery involves making a cut in the

chest so a surgeon can stitch the hole
closed or sew a patch of manmade
surgical material (such as Dacron or
Gore-Tex) over the defect. This prevents
shunting (the movement of oxygenated
blood from the left to the right ventricle).
Eventually, the tissue of the heart heals
over the patch or stitches, and by 6
months after the surgery, the hole will be
completely covered with tissue.

 Surgery is not usually performed in

newborns because small defects will
close spontaneously in 20-25%.
 The surgery also is more risky in the

first few months of life; the risk of
death from the operation is higher in
the first 6 months of life than later.

MANAGEMENT

 Medical management includes endocarditis

antibiotic prophylaxis for all patients with VSDs.
 Respiratory infections require prompt

evaluation and treatment.
 Evaluate children with VSD at least once or

twice yearly to detect changes in the clinical
picture that suggest the development of
pulmonary vascular obliterative changes.

 Patients with VSD and pulmonary

vascular obstructive disease who are
deemed inoperable because of
irreversibly elevated resistance require
more intensive support and
symptomatic therapy as cyanosis
progresses and activity becomes more
limited.

 Improvement in the symptoms

associated with the polycythemia of
Eisenmenger complex (headache,
extreme fatigue, and extreme
dyspnea) may be provided by partial
exchange transfusion for RBC volume
reduction.

-END-

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