VENTRICULAR SEPTAL

DEFECT
Dolly mathew

Development of IVS
Muscular septum
primordial IV septum

Closure of
interventricular
foramen& membranous
septum formationRt & Lt bulbar ridges
endocardial cushions

Anatomy

 A VSD is a defect in the ventricular septum

The ventricular septum consists of an inferior muscular
and superior membranous portion
The membranous portion -most commonly affected in
adults and older children
most common congenital cardiac anomalies.
3-3.8 per 1000 live births
30-60% of all newborns with a CHD
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

Location of VSDs

outlet
perimembranous
Muscular

Inlet

Swiss cheese

Classification

soto et al

Perimembranous(membranous/

infracristal )-70-80%
Muscular- 5-20%
Central- mid muscular
Apical
Marginal- along RV septal junction
Swiss cheese septum multiple
defects
Inlet/ AV canal type-5-8%
Supracrital/ subaortic- 5-7%

Types of VSD (kirklin)

1
2

Hemodynamic classification
Restrictive- resistance that limits the shunt at the site of
vsd
LVSP > RVSP
pulm /aortic systolic pressure ratio < 0.3
Qp / Qs<1.4/1
Moderately restrictive - RVSP high, but less than LVSP
- Qp/Qs 1.4/2.2

Non restrictive -Shunt not limited at the site of defect

RVSP , LVSP, PA , Aortic systolic
pressures
equal
Qp/Qs >2.2
Flow determined by PVR

Small VSD in infancy

<1/3rd size of aortic root
shunt limited by size of the defect
Shunt entirely during ventricular
systole
L R shunt <50% LV output
Pulmonary:systemic flow ratio < 2:1

Medium sized VSD

VSD size about

half equal to the
size of the aortic
orifice
When PA & RVSP
are
> 50% of
systemic arterial
pressure
mod-large L R
shunt develops

p218

Large VSD
Size equal to the
aortic root
Equalization of
pressures in RV& LV
Increased LA
pressure opening of
foramen ovale

Pathophysiology

 During systole, blood is shunted from LV to RV

passes through the lungs and re enters the LV
via the pulmonary veins and LA
causes volume overload on the LV
Shunt into the RV elevates RV pressure and
volume, leading to pulmonary hypertension.
More noticeable in patients with larger defects

pathophysiology
Magnitude of shunt: size, PVR
Small defect: large resistance occurs
at the defect
Larger defect: resistance offered by
the defect minimum
: Shunt depends
largely on PVR
Lower the PVR, greater the LR
Shunt

 Enlargement of LA,
LV,PA
Shunt mainly in
systole, when the
RV also contracts
Shunted blood
goes directly to PA

Natural history

Natural history

Spontaneous closure :75-85 % all VSDs

:35% perimemb ( 1st 6/12)
More frequent in small defects
Decrease in size with age
Inlet & outlet defects donot become smaller /close
spont
Large & nonrestrictive defects : 10- 15%

endocarditis risk of endocarditis 4-10% for the

first 30 years of life
High velocity turbulent jet into RV

 CHF
Large VSDs
Mod sized VSDs survive into
adulthood
Increased rt sided flow pulmonary
vascular disease Eisenmengers
physiology if left untreated

 Risk factors for decreased survival

Shortness of breath, fatigue, DOE,progressive
AR
Cardiomegaly
PASP >60mm Hg/ >1/2 of systemic pressure
Good prognosticators
Lack of symptoms
normal LV size & function
small LR shunt
normal pulmonary pressures / resistance
Intact vasodilator response in pulmonary
vasculature

genetic factors
Affected father- 2%
Affected mother 6%
25 yr survival for all pts with a VSD
87%
Mortality increases with the size of
VSD

Mechanisms of closure
Growth & hypertrophy of septum around the
defect
By development of subacute bacterial
endocarditis
adherence of STL tissue to the margins
(Negative pressure effect exerted by a high
velocity stream flowing through the defect )
Ventricular septal aneurysm
prolapse of aortic cusp
intrusion of a sinus of valsalva aneurysm

History & clinical

features

History
Incidence unrelated to maternal age,
sex, birth order
3.3% 1st degree relatives of index
patients
Among 1st degree relatives with CHD,
1/3rd have vsd
30-60% siblings of index patients
have vsd
Parents with spontaneously closed
vsd can have offspring with vsd

Small VSD - infancy

Normal wt gain & development

2-8 wks tachycardia & tachypnea
especially with infection
2-4/6 systolic mr, medium frequency

Large VSD - infancy

Infant well in the immediate postnatal period

Systolic mr LLSB after 1-7 days
develop respiratory distress , in 2-8 wks
Cardiomegaly
Systolic thrill , along LSB
S1 normal/ soft: s2loud narrow split
Systolic mr , 2-3/6 intensity at birth, louder &
harsh as shunt increases
S3 & MDM at apex
If the infant survives - subsequent course with
persistent dyspnea, sweating, poor feeding, failure to
thrive, LRTI

Beyond infancy
Arterial pulse- brisk ( vigorous ejection from a
volume overloaded ventricle)
N pulse in eisenmengers - systemic stroke
volume maintained
Cyanosis & clubbing : eisenmengers
JVP N in small defects
elevated - Mod restr & nonrestrictive vsd
with ccf
Precordial bulge ( large shunt 5-6 months)
Harrisons sulcus

Cardiomegaly
RV heave in pts with RV vol overload
Features of PAH
Grade 2-5/6 systolic regurgitant
mrLLSB
MDM preceeded by S3
Infundibular vsd: early diastolic
decrescendo mr of AR

Improvement of symptoms
Closing defect
findings : soft s2
high frequency & shorter murmur
Increasing PVR
findings : increased RV pulsations
s2 loud, narrow split
Infundibular hypertrophy
decreased LR shunt,
findings : s2 decreases in intensity ,
crescendo-decrescendo systolic murmur in the
ULSB,
cyanosis (shunt reversal )

 Eisenmengers

apex by RV

Palpable dilated hypertensive pulmonary

trunk

Loud pulmonary closure sound

Very short or no systolic mr of vsd

Short pulmonary ejection mr ULSB

EDM of pulmonary regurgitation

Loud harsh s1 coincident holosystolic

mr of TR

ECG
small defects unremarkable
LA enlargement - Mod restrictive, large
LR shunts
left axis deviation
Inlet vsd /AV septal defect
5% moderately restrictive vsds
Ventricular septal aneurysms
multiple vsds

 LV enlargement in larger defects

RVH - Mild or moderate elevation of
RV
pressure (rsR in V4R or V1)
- Large VSD, equal ventricular
pressures , elevated PVR
RVH , RAD - Eisenmengers
RBBB - Surgical repair

Chest x ray
Small defects that were mod restrictive at birth increased LV size, dilated
pulmonary trunk & its branches
Large shunts hyperinflated lungs with flat hemi
diaphragms
LA enlargement best appreciated in the lateral position

Increased PVR, decreases LR shunt, decreases heart size, enlargement of

pulmonary trunk& its branches persists
Nonrestrictive vsd with elevated but variable PVR- enlargement of all 4 chambers
Eisenmengers syndrome- oligemic lung fields, RA,LA, LV normal, RV occupies
the apex

Echocardiography

Common locations of vsd -2d echo

Echocardiography- doppler
CFM-Direction, timing of flow
IVG (mmHg) = 4v
PG = LVSP - RVSP

LVSP - PG jet = RVsp Pasp

RVSP = cuff systolic BP 4v
PVR = TRV / TVI in RVOT x 10 + 0.16
High PA pressure, TRV/TVI rvot < 0.2 ; indicates low PVR, elevated pressure
secondary to the flow

Cardiac catheterization
Hemodynamic assessments
cardiac index
oximetry
quantification of shunt
To assess pulmonary vascular resistance
Pts with increased PVR, with mod or large LR
shunt
If PVR is increased, response to 100% oxygen,NO
tested

cineangiography
Defect best imaged in
LAO(70)cranial (25)
Inlet defect hepatoclavicular view
( 40LAO,cranial
angulation)
Anterior muscular VSD- RAO view
Aortography - r/o PDA ,coarctation

Other imaging modalities

Cardiac CT- assess VSD anatomy in
suboptimal echo imges
No information about
shunt fraction
MRI
delineate vsd location& shunt
fraction in complex associated
lesions

Management

 Observation & follow up

Small VSDs
Medical management
Medium sized vsd
CCF- treat with diuretics & digitalis,
ACEI
failure ppted by LRTI- Treat both
2-3 months follow up
RV & PA pressures assessed
Failure to thrive
Surgical
Large vsd

 drugs
digoxin 10-20mcg/kg per day
furosemide 13mg/kg per day
captopril 0.52mg/kg per day
enalapril 0.1mg/kg per day

Indications of surgical intervention

Large VSD with pulmonary
hypertension
VSD with aortic regurgitation
VSD with associated defects
Failure of congestive cardiac failure
to respond to medications

Timing of surgery in VSD

<3months - if symptomatic
3-6 months - symptomatic, growth
failure, increasing PAH
>6 months primarily based on
PAH
Wait till 1 yr , if no PAH

 ACC/AHA guidelines 2008 for

management of adults with CHD

Surgical VSD closure

I IIa IIb III

Surgeons with training & expertise

should perform VSD closure surgeries

Closure of vsd indicated when Qp/Qs

2 or more & clinical e/o LV volume overload

I IIa IIb III

When pt has a history of IE

Surgical VSD closure

I IIa
IIa IIb III Closure of vsd is reasonable when LR shunt is present

at a Qp/Qs >1.5, with a PA pressure <2/3rd of systemic

pressure & pulse volume recording < 2/3 rd of SVR

I IIa IIb III Closure of vsd is reasonable when LR shunt is present

B
I IIa IIb III

at a Qp/Qs >1.5, in the presence of LV systolic or

diastolic failure

Vsd closure not recommended in pts with severe

irreversible PAH

Interventional Catheterization for

VSD

I IIa IIb III

Device closure of a muscular vsd may be
considered,especially if its remote from tricuspid
valve & aorta, if the vsd is associated with severe
Lt sided chamber enlargement, or if PAH

VSD closure
Direct closure of the defect
Surgical mortality <1%
Complications RBBB- direct injury
to rt bundle, disruption of purkinje
fibers
Residual shunt (<5% )
Injuries to tricuspid valve & aortic
valve

PA banding
PA banding- palliative procedure , when
additional lesions make repair difficult
Done in multiple VSDs
30-50% of original diameter is narrowed
Systolic pressure of 25-30 mmHg beyond
the constriction
RV/PA pressure gradient > 45 associated
with hypoxemia

Post op follow up
Every 1-2 yrs
VSD & mild PAH& repair after 3 yrs of
age- watch for progressive
pulmonary vascular disease
long term follow up needed

Special
situations

VSD with AR

Peri membranous VSD with AR - 5-8%

Subarterial VSDs 30%
Sagging or herniation of RCC or RCC+ NCC
May cause RVOT obstruction
Due to morphological abnormality of valve
LV volume regurgitant volume & shunt
volume
VSD murmur dates from infancy
AR murmur appears (5-9 yrs)

LV RA shunt

Gerbode defect
Shunt begins inutero
Usually restrictive
Rightward thoracic
position of murmur
X ray RA
enlargement
disproportionate to
the size of
pulmonary trunk

Thank you