1. VENTRICULAR SEPTAL DEFECT
Dolly mathew

2. Development of IVS Muscular septum – primordial IV septum
Closure of interventricular foramen& membranous septum formation-
Rt & Lt bulbar ridges
endocardial cushions

3. Anatomy

4. 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.
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

5. Location of VSDs
outlet
perimembranous
Muscular
Swiss cheese
Inlet

6. Classification

7. 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%

8. Types of VSD (kirklin) 1 2 3 4

9. 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

10. <1/3rd size of aortic root shunt limited by size of the defect
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

11. VSD size about half – equal to the size of the aortic orifice
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

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

13. Pathophysiology

14. 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

15. 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 LR Shunt

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

17. Natural history

18. 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 : %
endocarditis – risk of endocarditis 4-10% for the first 30 years of life
High velocity turbulent jet into RV

19. CHF
Large VSDs
Mod sized VSDs survive into adulthood
Increased rt sided flow  pulmonary vascular disease  Eisenmenger’s physiology if left untreated

20. 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 LR shunt
normal pulmonary pressures / resistance
Intact vasodilator response in pulmonary vasculature

21. 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

22. 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

23. History & clinical features

24. 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

25. Normal wt gain & development
Small VSD - infancy
Normal wt gain & development
2-8 wks – tachycardia & tachypnea especially with infection
2-4/6 systolic mr, medium frequency

26. 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

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

28. 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

29. 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 LR shunt,
findings : s2 decreases in intensity ,
crescendo-decrescendo systolic murmur in the ULSB,
cyanosis (shunt reversal )

30. Eisenmenger’s
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

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

32. 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 - Eisenmenger’s
RBBB - Surgical repair

33. 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 LR shunt, decreases heart size, enlargement of pulmonary trunk& its branches persists
Nonrestrictive vsd with elevated but variable PVR- enlargement of all 4 chambers
Eisenmenger’s syndrome- oligemic lung fields, RA,LA, LV normal, RV occupies the apex

34. Echocardiography

35. Common locations of vsd -2d echo

36. Echocardiography- doppler
CFM-Direction, timing of flow
IVG (mmHg) = 4v²
PG = LVSP - RVSP
LVSP - PG jet = RVsp ≈ Pasp
RVSP = cuff systolic BP – 4v²
PVR = TRV / TVI in RVOT x
High PA pressure, TRV/TVI rvot < 0.2 ; indicates low PVR, elevated pressure secondary to the flow

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

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

39. 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

40. Management

41. 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

42. drugs
digoxin 10-20mcg/kg per day
furosemide 1–3 mg/kg per day
captopril 0.5–2 mg/kg per day
enalapril 0.1mg/kg per day

43. 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

44. 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

45. ACC/AHA guidelines 2008 for management of adults with CHD

46. Surgical VSD closure When pt has a history of IE
Surgeons with training & expertise should perform VSD closure surgeries
Closure of vsd indicated when Qp/Qs or more & clinical e/o LV volume overload
When pt has a history of IE

47. Surgical VSD closure B B
IIa
IIb
III B
Closure of vsd is reasonable when LR shunt is present at a Qp/Qs >1.5, with a PA pressure <2/3rd of systemic pressure & pulse volume recording < 2/3rd of SVR
Closure of vsd is reasonable when LR shunt is present 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 I
IIa
IIb
III B I
IIa
IIb
III

48. Interventional Catheterization for VSD
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

49. 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

50. 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 mmHg beyond the constriction
RV/PA pressure gradient > 45 associated with hypoxemia

51. 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

52. Special situations

53. 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)

54. 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

55. Thank you