Ventricular Septal Defects

Interventricular Septum:
The ventricular septum develops
before the 8th week of fetal life.
Embryonic ventricular septum:
is derived from the ventricular
wall which is made of cardiac
muscles. It only separates the
ventricles partially, it is not a
complete septum.
Aorticopulmonary (Spiral)
septum: is derived from the
neural crest and is in the middle
of the truncus arteriosus and
divides it into two equal halves
which are the aorta on the left side
and the pulmonary trunk on the
right side.
 Interventricular Septum:
• This aortic-pulmonary septum doesn’t end at
the bottom of the truncus arteriosus; it
actually continues on going into the ventricle,
and because it is in the center of the trunk and
because the embryonic ventricular septum is
right in the center, those two septa end up
meeting end to end and fusing thereby
completing the ventricular septum.
There are two parts of the interventricular
septum:
Muscular IV septum which is made of
cardiac muscle
Membranous IV septum because there is no
muscle in it; it is a neural crest-derived
structure.
 Ventricular Septal Defect
 Represents a hole in
the interventricular
septum that allow a
haemodynamic
communication between
the right and left ventricles.
It typically results in a left-
to-right shunt. The defect
varies in size from 3- 4 mm
to more than 3 cm.
 The shunt may reverse to
Right-to-Left shunt!!!
Epidemiology
Henri Roger was the first man to
describe ventricular septal defect in
1879.
the most common congenital
cardiac abnormality diagnosed in
children (30%) .
and the second most common
diagnosed in adults.
The estimated incidence is at ~1 in
400 births.
Etiology:
30-35% of cases are isolated VSDs .
For the vast majority of children with a defect, there is no evident
reason why a VSD develops.
Risk Factors:
In some cases, the tendency to develop a VSD has genetic basis:
 Chromosomal disorders associated with an increased incidence of
VSD: Down syndrome, Di George syndrome, Turner syndrome.
 Familial forms: TBX5, GATA4, and NKX2.5 mutations.
 Children from an adult with a VSD that is not associated with a
genetic disorder may have risk of VSD as high as 3% if father is
affected, and 6% if mother is affected.
Associated with:-
Atrial Septal Defects (35%).
Patent Ductus Arteriosus (22%).
Coarctation of Aorta (17%).
Subvalvular Aortic Stenosis (4%).
Subpulmonic Stenosis, usually associated with
progressive aortic regurgitation caused by prolapse of
the aortic cusp through the defect.
Multiple VSDs are more prevalent among patients
with tetralogy of Fallot and double outlet right
ventricular defects.
 Classification
Type 1:
 Outlet, subarterial, supracristal, conal septal
defect and infundibular.
 Found in 5-7% of those affected
 More in Asians
 Defect within the conal septum.
 They are located just beneath the pulmonary
valve, and surrounded by the muscle of the
infundibular septum.
Type 2:
 Perimembranous, paramembranous,
conoventricular and subaortic
 Most common; found in 80%.
 Lies in the outflow tract of the left ventricle
immediately beneath the aortic valve.
 Include malalignment defects seen in tetralogy
of fallot.
 Classification
Type 3:
 Inlet and AV canal type.
 Commonly associated with
atrioventricular septal defect.
 Found in about 5%.
 The VSD lies beneath the tricuspid
valve, and limited uptsream by the
tricuspid annulus without
intervening muscle (they are
located posteriorly and inferior to
the perimembranous portion of the
ventricular septum.
 Associated with trisomy 21
(Down Syndrome).
 Classification:
Type 4:
 Muscular.
 They are surrounded by
muscles and can occur
anywhere along the trabecular
portion of the septum.
 found in 5-20% of all VSDs.
 Can be sub classified again
based on the location into:
anterior, apical, posterior and
midventricular.
 Spontaneous closure is
common.
Classification
 The “Swiss Cheese” type of muscular VSD consists of
multiple communications between the right and left
ventricles looking like the holes in Swiss cheese; they
complicate operative repair.
 Classification
Type Gerbode:
 Gerbode also known as left
ventricular to right atrial
communication
 Membranous part has two parts:
(a) atrioventricular part; (b)
ventricular part.
 Defects in the artioventricular
part leads to shunting of blood
from left ventricle to right atrium
directly (true gerbode shunt).
Classification
Defects in the ventricular part leads to shunting of
blood from left ventricle to right ventricle and then
through perforation in the leaflet goes to the right
atrium. It is indirect LV to RA shunt (false gerbode
shunt).
 Classification
Lesion Size:
1) Small Restrictive VSDs (80-90%):
 Smaller than the aortic valve
diameter, perhaps up to 3 mm.
 Small Left to Right Shunt.
 Normal RV output.
2) Large Nonrestrictive VSDs (10-
20%):
 the same size or bigger than the
aortic valve.
 Large hemodynamically
significant Left to Right shunt.
 Equal RV and LV pressures.
 Pathophysiology
During systole, blood leaks from the
left ventricle to the right ventricle
because: 1) pressure in left ventricle is
higher than pressure in right ventricle
(Left to right shunt). 2) the size of the
defect. 3) Pulmonary Vascular
Resistance (PVR) and systolic
resistance. (the shunt depends on (the
size of the defect,compliance of both
ventricles and PVR and systolic
resistance).
The blood which is coming from the
left to the right creates two problems:
Volume overload, and Pressure
overload; this leads to RVH.
Pathophysiology
Consequently, there is large increase in pulmonary
blood flow (volume overflow) as well as excessive
pressure in the pulmonary circulation (it is more
dangerous than ASD because in ASD there was only
volume overflow to the pulmonary circulation).
This will produce irreversible changes that will
eventually lead to Pulmonary HTN and Pulmonary
Vascular Resistance (PVR).
If significant Obstructive Pulmonary Vascular Disease
develops, then surgical correction of the lesion may not
benefit the patient, because this is irreversible.
 Pathophysiology
If right ventricle becomes
significantly hypertrophied
especially secondary to pulmonary
HTN and Obstructive Pulmonary
Vascular Disease, the pulmonary
arterial pressure can reach levels
that equal the systemic pressure,
this reverses the left to right shunt,
so that blood then flows from the
right ventricle into the left
ventricle, and we say that patient
has developed Eisenmenger’s
syndrome.
 Pathophysiology
Reversal of the shunt is a very bad sign! It means there is
significant pulmonary HTN and significant obstructive pulmonary
vascular disease.
The reversal of the shunt depends on many factors:
1) The original size of the defect.
2) How much volume overload and pressure overload in the right heart
and pulmonary circulation and how fast the patient will develop
pumonary HTN and obstructive pulmonary vascular disease.
3) Other factors: for example; patients living at high altitude develop
eisenmenger’s syndrome earlier as compared to patients living at sea
level, because there is less oxygen going to their lungs and the low
oxygen itself keeps the vessels semiconstricted, at the top there is
pressure and volume overload, so they will develop eisenmenger’s
syndrome very rapidly.
 Pathophysiology
Left ventricle gets hypertrophied, because when left
ventricle produces contraction, a lot of its cardiac output
is reversed on the right side and to maintain the forward
cardiac output left ventricle has to work more, so in this
disease at advanced stages there is RVH as well as LVH.
Hypertrophied heart demands more oxygen, and
because blood is shunting here initially and there is less
pressure in the root of the aorta and less blood is going to
the coronary system that increases the risk of ischemic
heart disease, and when reversal of the shunt occurs then
blood which is going to the coronary system is not
oxygenated well so that leads to chest pain.
 Pathophysiology
Complications of Eisenmenger’s Syndrome:
1) Cyanosis (because deoxygenated blood is coming to the left ventricle and this mixing of
the deoxygenated blood to the left ventricular and so the body will get less oxygen).This
cyanosis develops late in the disease and is called “tardive cyanosis” or “frank cyanosis”.
2) Erythrocytosis or Polycythemia (because chronic low oxygen supply to the kidneys will
produce to the excessive production of erythropoietin).
3) Clubbing (due to hypoxia to the ends of the fingers and toes).
4) Severe chest pain.
5) Exertional dyspnea.
6) Syncope.
7) Hemoptysis.
8) Cholilthiasis.
9) Renal dysfunction.
10) Hyperuricemia and gout.
11) Cerebrovascular accidents.
12) Hemostatic abnormalities. Clubbing in eisemenger’s
syndrome1; also called hippocratic
fingers.
Pathophysiology
Increased Risk of Infective Endocarditis: Another
important thing about the ventricular septal defect is
that the jet of the flow may produce damage to the
endocardium and platelets will stick there and those
platelets and fibrin may act as a good hiding place for
microbes increasing the risk of infective endocarditis
(smaller shunt >>increase blood turbulance >>
increase risk of endocarditis).
 Clinical Manifestations
*Symptoms:
 Small VSDs:
Asymptomatic.
 Large VSDs:
1) Heart failure with breathlessness and failure to thrive (flatering
growth) manifests after 1 week old.
2) Recurrent chest infections.

*Physical signs:
 Small VSDs:
1) Loud pansystolic murmur at lower left sternal edge (loud murmur
implies smaller defect>> the smaller the defect the louder the
murmur); and palpable thrill.
2) Quiet pulmonary second sound (P2).
Clinical Manifestations:
 Large VSDs:
1) Tachypnea, tachycardia, and enlarged liver from heart
failure.
2) Heave on the left lower sternal border.
3) Soft pansystolic murmur or no murmur (implying large
defect).
4) Apical mid-systolic murmur (from increased flow across
the mitral valve after the blood has circulated through
the lungs.
5) Loud pulmonary second sound (P2) from raised
pulmonary arterial pressure.
Important Note
When the murmur disappears this could be either good
sign meaning that the defect has closed, or a bad sign
meaning that the patient has developed eisenmenger’s
syndrome.
We can differentiate by a simple test which is pulse
oximeter and we see the O2 saturation, if it is normal
then it is a good sign, if it is abnormal then there is
cyanosis and the patient has developed eisenmenger’s
syndrome.
Investigations
* CXR:
 Small VSDs:
Normal
 Large VSDs:
1) Cardiomegaly
2) Enlarged pulmonary arteries
3) Increased pulmonary vascular markings
4) Pulmonary edema
Chest x-ray demonstrates prominent pulmonary vasculature
(active congestion) without pleural effusions or convincing
consolidation. The heart is prominent. 
This is a large VSD.
Investigations
* ECG:
 Small VSDs:
Normal
 Large VSDs:
Will show signs of left ventricular or biventricular
hypertrophy.
Investigations
* Echocardiography:
It provides definitive diagnosis.
 Small VSDs:
Demonstrates the precise anatomy of the defect. It is
possible to assess its hemodynamic effects using
doppler echocardiography. There is No pulmonary
hypertension.
 Large VSDs:
Demonstrates the anatomy of the defect, hemodynamic
effects and pulmonary hypertension (due to high
flow).
Investigations
Investigations:
* Cardiac Catherization:
Has largely been supplanted by echocardiography,
except in older children in which measurement of
pulmonary resistance is necessary prior to
recommending closure of the defect.
Management:
Small VSDs:
1) Lesions will close spontaneously as the heart grows;
this is ascertained by disappearance of the murmur
with normal ECG and Echocardiography.
2) Prevention of bacterial endocarditis is by
maintaining dental hygiene as long as the VSD is
present.
 Management:
Large VSDs:
1) VSDs may close or narrow spontaneously; the probability of
closure is inversely related to the age at which the defect is
observed:
 1 month have 80% incidence of spontaneous closure.
 6 months >> 50%.
 12 months >>> only 25% chance of closure.
This has an important impact on operative decision making, because
small or moderate sized VSDs may be observed for a period of
time in the absence of symptoms. Large defects and those in
severely symptomatic neonates should be repaired during infancy
to relieve symptoms and because irreversible changes in pulmonary
vascular resistance may develop during the first year of life.
 Management
2) Ventricular septum defect in infants is initially treated medically with:
 Loop diuretics; e.g: furosemide 1–3 mg/kg per day; to relieve pulmonary
congestion. Long-term furosemide treatment results in hypercalciuria,
renal damage, and electrolyte disturbances.
 ACE inhibitors; e.g enalapril and captopril 0.5–2 mg/kg per day. These
medications reduce both the systemic and pulmonary pressures (the former
to a greater degree), thereby reducing the left-to-right shunt
 Cardiac glycosides; e.g: digoxin 10-20 µg/kg per day. This may be
indicated if diuresis and afterload reduction do not relieve adequately
symptoms, although the data regarding efficacy of this drug in this
particular situation are controversial
 Increased caloric density of feedings to ensure adequate weight gain -
Occasionally, oral feeds must be supplemented with nasogastric tube
feedings, because a baby in CHF may be unable to consume adequate
calories for appropriate weight gain.
Management
3) Some cases may necessitate surgical intervention.
Indications for surgical intervention:
 Failure of congestive cardiac failure to respond to medications.
 VSD with pulmonic stenosis (this is considered TOF if
malalignment of VSD).
 Large VSD with pulmonary hypertension.
 VSD with aortic regurgitation and prolapse of aortic valvular cusp
into the VSD (this happens in the two types of VSD which are close
to the aortic valve which are the membranous and outlet types,
because the jet sucks the aortic cusp causing AR).
 After endocarditis.
 Clear volumetric overload of left atrium and ventricle in
echocardiography.
Management:
Contraindications for surgery:
 Small and moderate VSDs with normal PVR have a
natural tendency to become smaller and eventually close.
Surgery is not indicated for these defects.
 Fixed pulmonary vascular obstructive disease resulting
in diminution of left-to-right shunting or even right-to-
left shunting is an absolute contraindication to ventricular
septal defect (VSD) closure. In this situation, the VSD acts
as a "pop-off valve," allowing right-to-left flow to bypass
the lungs and maintain systemic cardiac output. In this
situation, closure of this defect results in right-sided
ventricular failure and low cardiac output.
Management
 Timing of surgery:
 For symptomatic patients or patients with larger ventricular
septal defects (VSDs) or elevated PVR, surgical closure is
indicated. However, the timing of surgery varies. The ideal
time to intervene is when the likelihood of spontaneous
VSD closure is lowest and the risk of irreversible
pulmonary vascular disease and ventricular dysfunction
will be minimized.
 In subarterial VSDs, the risk of irreversible aortic valve
damage caused by cusp prolapse leads to earlier
intervention. With perimembranous and muscular defects,
surgery may be reasonably delayed up to 1 year or more if
the infant is thriving and the pulmonary artery pressure is
known to be near normal.
Management - Surgery
Types of surgery:
• Intra-cardiac approach:
 Most common technique,
and patient is under
cardiopulmonary bypass
(heart lung machine), and
is an open heart
operation.
 moderate hypothermia
and cardioplegic arrest.
Management - Surgery
Approach
The interventricular septum is usually well reached
through the tricuspid valve ( Rt Atriotomy).

Ventriculotomy should be avoided as possible because

of the technical difficulties and the possibility of
developing arrhythmia foci across the ventriculotomy
scar.

However two types of VSD might need a different

approach,
Outlet type
Apical muscular VSDs
 Management
 Right atrial approch (used regardless of the defect to inspect the
anatomy, and is preferable for most defects), left ventriculotomy
( for adequate exposure in apical muscular defects), longitudinal
incision in the pulmonary artery and a transverse incision in the
right ventricle below the pulmonary valve (for supracristal defects).
- A patch repair is employed
taking care to avoid
conduction system.
- Routine use of intraoperative
transesophageal
echocardiography should be
used to assess for any
residual defects.
 Management
• Transcatheter technique:
 A device, known as the Amplatzer
muscular VSD occluder, may be
used to close certain VSDs.
 The cost is also lower than having
open heart surgery.
 The device has demonstrated
100% closure rate in a small series
of patients with isolated or
residual VSDs, or as a
collaborative treatment strategy
for the VSD component in more
complex congenital lesions.
Management
The device is placed through a small incision in the
groin. It is shown to have full closure of the ventricular
defect within the 24 hours of placement.
Proponents of device closure argue thar their use can:
 Decrease complexity of surgical repair.
 Avoid operation for a small residual lesion
 Avoid the need for venticulotomy.
Management
Palliative:
 Used for Multiple or Swiss Cheese
VSDs, because definitive VSD closure
cannot be accomplished.
 Temporary placement of a pulmonary
artery band can be employed to
control pulmonary flow.
 This allows time for spontaneous
closure of many of the smaller defects,
thus simplifying surgical repair.
 Cyanosis increases as in TOF, but the
patient thrives well and mortality
decreases.
Results
Closure of VSDs is safely performed even in very
small infants.
Hospital mortality near zero percent.
The main risk factor: the presence of associated
leasions, especially when present in symptomatic
neonates with large VSD.