Ventricular Septal Defects

Tate Gisslen, MD
Mentor: Bradley S. Marino, MD, MPP,
MSCE
May 6, 2011

Anatomy
4 morphological components of septum
Membranous
Inlet
Outlet/Infundibular
Muscular/Trabecular

Anatomy
Membranous-70-80%
Small
Located at base, between inlet and outlet
Perimembranous - Extends to adjacent septum
Membranous

Membranous

Anatomy
Inlet
Inlet 5-8%,
AV valve to chordae attachments

Inlet

Anatomy
Outlet/Infundibular
5-7%
Separates L and R outflow tracts

Infundibular

Anatomy
Muscular/Trabecular (5-20%)
Anterior/Marginal (anterior to septal band)
Midmuscular/Central (posterior to septal band)
Apical (inferior to moderator band)
Posterior (beneath septal leaflet)

Muscular

Physiology
Blood flow (which way and how

much) dependent on multiple

factors
Small and restrictive
Lesion size

Large and non-restrictive

Balance between pulmonary and
systemic vascular resistance

Lesion Size
Restrictive VSD
< 0.5 cm2 (Smaller than Ao valve orifice area)
Small L to R shunt
Normal RV output
75% spontaneously close < 2yrs
Non-restrictive VSD
> 1.0 cm2 (Equal to or greater than to Ao valve

orifice area)
Equal RV and LV pressures
Large hemodynamically significant L to R shunt
Rarely close spontaneously

Vascular Resistance
Pulmonary resistance may remain high longer in

infants with large VSD

Minimal L to R shunt

Decreasing pulmonary resistance leads to significant L

to R shunt
Clinical symptoms of CHF

Persistent L to R shunt leads to hypertrophy of the

medial smooth muscle layer of the pulmonary arteries

which increases PVR and potential R to L shunting
Long-standing L to R shunting that results in
chronically increased PVR may lead to persistent R to L
shunting described as Eisenmenger Physiology

Clinical Features-Small Lesions

Murmur
4 to 10 days, early with rapid

decrease in PVR

Asymptomatic
normal feeding, growth and

development

Murmurs
Restrictive VSD - Holosystolic

murmur
correlates with continuous
pressure gradient
Non-restrictive large VSD no

murmur (no turbulence if no

gradient)

Clinical Features-Large Lesions

Accentuated precordial activity
More prominent as LV volume increases
Signs/symptoms of CHF
Diaphoresis
Tachypnea
Fatigue with feeding
Hepatomegaly
Rales
Duskiness with crying
May develop as early as 2 weeks
Severity increases as PVR decreases

Evaluation
Chest Radiography
Cardiomegaly
Increased pulmonary

vasculature
Pulmonary edema

CXR of VSD

Evaluation
EKG
Small: normal or LVH
Prominent Q, R, and T waves in II,
III, aVF and V6
Large: Biventricular

hypertrophy
RVH- rsR in V1, S wave in V6

Echocardiography
Assess indication in consultation with

Cardiology
Assess location, size, and multiplicity
RV and PA pressure
Assess for LA and LV dilation
Assess LV function
Note relation to great vessels, AV
valves

Cardiac Catheterization
Able to document
Number of defects
Presence of associated defects
Magnitude of shunt
Estimate PVR
Not used if information apparent by

other means
Most information available through
Echocardiography

Prevalence
Most common congenital heart

lesion
Occurs in 50% of children with
heart lesions
15-20% in isolation
5-50 per 1000 live births
56% female

Associated Defects
Left Heart Defects
Aortic stenosis
Coarctation of the aorta
Right Heart Defects
Tetrology of Fallot
Double Outlet Right Ventricle
Truncus Arteriosus
Some single ventricle (e.g. Tricuspid

atresia, double inlet left ventricle)

Chromosomal Disorders
associated with VSD
Trisomy 21: 40% of T21 will have VSD
Trisomy 13, 18: 18% of T13, 31% of T18 will

have VSD
22q11 deletion:
Tetrology of Fallot is most common anomaly
VSD with or without aortic arch anomaly is second most

common

Holt-Oram (Hand-heart syndrome): TBX5 gene

found on Chromosome 12
Recurrence risk for VSD based on parental VSD
Paternal 2%
Maternal 6-10%

Treatment for Small VSD

No medication or surgery if

asymptomatic
75-80% close by 2 years
Observation
No antibiotic prophylaxis for

procedures

Treating a Moderate to Large

VSD
Treatment of CHF
Determining when to repair

CHF Treatment
High-calorie formula
Medication
Diuretics
Furosemide with or without

spironolactone
Afterload reduction
Enalapril or Captopril
Digoxin (maybe)
Symptoms of CHF improve as L to R

shunt decreases

Indications for Intervention

Decompensated CHF
Compensated CHF with:
Large hemodynamically significant

VSD - L to R shunting with Qp/Qs >

2:1, even if asymptomatic, ideally
before 1 year
Growth failure, unresponsive to
medical therapy is an indication for
surgery

Not Indicated
Small VSDs - 6 months without CHF or

PVR
Small hemodynamically insignificant

VSD L to R shunting with Qp/Qs <

1.5:1
Eisenmenger physiology

Post-Intervention
Most infants have normal growth

and development
Early closure (< 1 year) associated
with better LV function and
regression of hypertrophy
Residual VSD is not common
RBBB is common following surgery
Rare complete heart block

Surgery
Typically dacron patch closure
Sometimes primary closure
Surgical mortality < 1%

Catheter Closure
Location
Muscular
Perimembranous
Complications
Heart block
Valve insufficiency

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