Part I

Fetal Circulation, ASD, VSD

Ventricular Septal Defect

Henri Roger was the first man to
describe a ventricular septal defect,
in 1879 he wrote:
A developmental defect of the heart
occurs from which cyanosis does not ensue
in spite of the fact that a communication
exists between the cavities of the two
ventricles and in spite of the fact that the
admixture of venous blood and arterial
blood occurs. This congenital defect, which
is even compatible with long life, is a
simple one. It comprises a defect in the
interventricular septum

Ventricular Septal Defect

Most common CHD in children (25%)
Isolated VSD found in only 10% of
adults with CHD
75-80% of small VSDs close
spontaneously by late childhood
10-15% of large VSDs close
spontaneously
60% of defects close before age 3,
and 90% before age 8
Risk factors for decreased survival for
unoperated patients include:
Cardiomegaly on CXR, Elevated PASP
(>50 mmHg), and CV symptoms

Ventricular Septum
Partitioning begins as a
muscular ridge near the
apex
Ridge undergoes active
growth which forms the
muscular septum (inlet,
trabecular, and outlet)
Concomitantly
endocardial cushions
fuse and the two regions
meet

Outlet

Inlet
Trabecular

Types of VSDs
Perimembranous defect (70-80%)
Less likely to be associated with other defects
Highest rate of spontaneous closure

Muscular or apical defects (5-20%)

Typically occur in isolation
High spontaneous closure rates unless multiple

AV-Canal type (5-8%)

Rarely close spontaneously, commonly seen in Trisomy 21
Usually large & associated with abnormal AV valve

Supracristal or subaortic defects (5-7%)

Often small but need closure due to associated AR

VSD
Arterial pulse is often normal
There may be a systolic thrill on palpation of
the precordium (maximal in 3rd or 4th ICS)
Holosystolic, high frequency murmur (grade 46/6) with small VSD and normal PAP
Once PAP increases above the systemic
pressures the holosystolic murmur disappears
Increase flow across pulmonary valve causes a
SEM
A loud P2 component is heard in this setting

ECG in VSD
May be normal but often shows LVH and LAE
Presence of RAD represents elevated RVP and PAP
Postoperative RBBB is common

CXR in VSD
Cardiomegaly with LAE and LVE will be seen with large L to R shunts
A large defect associated with a small heart and oligemic lung fields should
raise the suspicion of pulmonary vascular disease

VSD
Hemodynamic severity grading of isolated VSDs in
adults:
Small: Qp:Qs <1.4, and pulmonary to aortic systolic
pressure <0.3
Moderate: Qp:Qs = 1.4-2.2, and systolic pressure ratio
>0.3
Large: Qp:Qs >2.2, and systolic pressure ratio >0.3
Eisenmenger: Qp:Qs <1.5 and systolic pressure ratio >0.9

Physiologic classification:
Restrictive: RV pressure < LV pressure in absence of
RVOTO
Nonrestrictive: RV pressure = LV pressure in absence of
RVOTO

VSD
Clinical severity grading:
Small: Causes negligible hemodynamic changes. LV size
normal w/o PHTN
Moderate: Causes LV and LA enlargment, and usually some
PHTN (reversible)
Large: Results in pulmonary vascular obstructive disease and
Eisenmenger physiology unless there is coexistent RVOTO

Pathologic and surgical classification:

Perimembranous: bordered by fibrous continuity of an AV
valve and an arterial valve, usually with inlet or outlet
extension
Muscular: bordered by muscular rim, usually trabecular
Doubly committed: bordered by fibrous continuity of both the
aortic and pulmonary valves

VSD Repair
When repair is performed in the first two years
of life, asymptomatic adult survival with normal
growth and development can be anticipated
When surgery is undertaken in older children, a
late postopeartive increase in LV chamber size,
together with decreased systolic function is seen
Development of late postoperative PHTN is
largely determined by the age at surgery and
preoperative PVR
Risk of SBE persists and requires prophylaxis

 The initial workup at a minimum

should include:

A through clinical assessment

ECG
CXR
TTE/Doppler evaluation

 The diagnostic workup may require:

Oxymetry
Right heart Cath (PAP and PVR determination, to assess
pulmonary vascular reactivity)
Coronary angio (in high risk pts or in pts >40 y if surgical
repair is planned)
MRI to prove existence of VSD or to assess for other
anomalie if doubt remains after other imaging modalities.
Also can calculate Qp:Qs
Oxygen saturation with exercise if there is any suggestion
of PHTN. Do not exercise if there is severe PHTN or resting
oxygen Sat is <85%
Open lung Bx should be considered when the reversibility
of PHTN is uncertain from hemodynamic data

 Indications for intervention: Geade C, Level IV

Presence of a significant VSD (symptomatic QP/QS = 2/1,
PASP > 50 mmHg), deteriorating ventricular fx due to
volume (LV) or pressure (RV) overload
Significant RVOTO (pk to pk gradient of > 50 mmHg, or
instantaneous gradient >70 mmHg)
Perimembranous or doubly committed VSD with more
than mild AR
In presence of severe PHTN (PAP >2.3 SABP, or PVR >2/3
SVR), there must be a net L to R shunt of >1.5 or
evidence of PA reactivity when challenged with pulmonary
vasodilator, or lung Bx evidence of PA changes are
potentially reversible (Heath Edwards grade II-III or less)
Hx of endocarditis especially if recurrent

 Transvenous pacing should be avoided where

possible in all patients with VSDs because
paradoxical emboli may occur.
Patients with isolated VSD with or without
associated lesions RVOTO, AV prolapse, subaortic
stenosis, or infective endocarditis) should be
repaired by congenital heart surgeons.
Device closure of VSDs may be performed in the
setting of isolated trabecular muscular VSDs but
are still considered experimental for
perimembranous VSDs

 Successful closure is associated with excellent

survival if ventricular fx is normal. Elevated PAP
preop may progress, regress, or remain the same
postop
A. fib may occur, especially if there has been
longstanding volume overload of the left heart.
Late VT and sudden death are potential risks,
especially in patients repaired late in life. CHB
may also occur after surgical repair
Pregnancy is well tolerated in women with small
or moderate VSD and in women with repaired VSD
Pregnancy is contraindicated in women with
Eisenmenger syndrome due to both high maternal
(>50%) and fetal (~60%) mortality

 Follow-up:

Patients with following problems benefit from

periodic evaluation by cardiologist
Patch leaks or residual VSDs (which seldom require
reoperation)
Elevated PVR at time of surgery
Aortic valve surgery
Late repair of moderate or large defects
Significant atrial or ventricular arrhythmias
Associated cardiac lesions (eg RVOTO, AR)

Endocarditis prophylaxis is recommended for

6/12 following VSD closure or for life if residual
defect persists