Symposium on Pediatric Cardiology – I

Aortic Stenosis
Gautam K. Singh

Department of Pediatrics, Division of Pediatric Cardiology, Saint Louis University School of Medicine/Cardinal
Glennon Children’s Hospital, St. Louis, USA

Abstract. Valvular aortic stenosis in pediatric age group is mostly congenital in origin. The aortic valve may be unicuspid,
bicuspid, tricuspid or rarely quadricuspid. Left ventricle undergoes concentric hypertrophy secondary to obstruction to its
outflow tract. In neonatal aortic stenosis, left ventricle may be hypoplastic. The pathophysiology and clinical presentation vary
with the age of onset and severity of obstruction. Echocardiography and Doppler are indispensible for the diagnosis of aortic
stenosis and its severity. Cardiac catheterization is not necessary for the diagnosis, however it is performed as a part of
balloon aortic valvuloplasty in severe cases. Balloon valvuloplasty is an effective alternative to surgery in pediatric age group.
Some of these patients require surgical valve repair or replacement on follow-up. Neonates and young infants with critical
aortic stenosis present with cardiogenic shock and need aggressive treatment with prostaglandin E1 infusion along with
inotropic support. In experienced hands, balloon valvuloplasty is a safe procedure in neonates and infants with critical
stenosis. Patients with mild and moderate aortic stenosis may be left on medical follow-up.
[Indian J Pediatr 2002; 69 (4) : 351-358]

Key words : Aortic stenosis; Echocardiography; Balloon valvuloplasty

Aortic stenosis (AS) is a fixed form of obstruction to the

egress of blood through left ventricular outflow tract
(LVOT) at the aortic valve level. In pediatric age, it is
mostly congenital in nature especially when isolated and
accounts for 60% to 75% of fixed LVOT obstructive
lesions.1 AS occurs more frequently in males than females
by a ratio of 3 to 5:1. Associated congenital heart defects
(CHDs) are present in 15% to 20% of the patients with AS,
the most common being patent ductus arteriosus,
coarctation of aorta, and ventricular septal defects.2

ANATOMY AND PATHOLOGY

Sinuses of Valsalva form the functional unit of a normal
aortic root. Three expansions of the aortic wall and the
semi-lunar attachment of its corresponding leaflets create
three pocket-like spaces which are separated by
commissural spaces and inter-leaflet triangles.3,4 The
length of the free margin of a leaflet is longer than its
attachment to the supporting sinus and the sum of the
areas of the leaflets is greater than the cross-sectional area
of the aortic root. Combination of such architecture Fig. 1. Normal aortic valve. The 2-D echocardiographic parasternal
allows for a competent closure during diastole the short axis view of a normal aortic valve in systole is showing
the three leaflets and the circular orifice. AO, aortic valve; LA,
reversal of curvatures of the leaflets with resultant non-
left atrium; RA, right atrium; RV, right ventricle.
obstructed circular valve orifice during systole (Fig. 1).
The congenital AS displays several morphological
types of abnormal valves: unicuspid, bicuspid, tricuspid, Configuration of the aortic valve usually can be predicted
and quadricuspid.5 In general, valves with fewer cusps by age. The unicuspid valve is more frequently seen in
and commissures are more likely to be stenotic. neonates with critical aortic stenosis. Aortic stenosis,
which becomes apparent in children and adolescents,
Reprint requests : Dr. Gautam K. Singh, Associate Professor,
Division of Pediatric Cardiology, Saint Louis University School of
usually exhibits bicuspid valve. Aortic stenosis with truly
Medicine, 465 South Grand Boulevard, St. Louis, MO 63104-1095, tricuspid valve is mostly seen beyond the pediatric age
USA. Fax : (314) 268-6411, E-mail : singhgk@slu.edu group.

Indian Journal of Pediatrics, Volume 69—April, 2002 351

 Gautam K. Singh
Fig. 2. Valvular aortic stenosis with a unicuspid valve. The 2-D
echocardiographic parasternal short axis view of a unicuspid
aortic valve in systole is showing eccentric small circular
orifice. Abbreviations as in Fig. 1.
Unicuspid Valve: The unicuspid aortic valve has only
one patent commissure. 4,5 The proximal aortic root
circumference is approximately half of the normal and the
length of the free edge of the leaflet is less than the
circumference of the sinus. 3,4 The leaflet is single,
thickened, and rigid. The valve orifice is eccentric (Fig. 2).
Therefore, a limited flap-valve type of opening during
ejection of the blood occurs and results in intrinsic
obstruction to flow. Unicuspid valve in critical aortic
stenosis often has a centrally situated orifice and no
commissure.5 Both types of architectures of unicuspid
valve do not lend themselves well to closed valvotomy
and balloon dilatation.
Fig. 3. Valvular aortic stenosis with a bicuspid valve. The 2-D
echocardiographic parasternal short axis view of a bicuspid
aortic valve in systole is showing an ellipsoidal orifice and
anterior-posterior arrangement of the two leaflets.
Abbreviations as in Fig. 1.
352
Bicuspid Valve: The bicuspid aortic valve is the most
frequent congenital malformation of the aortic valve.1 The
two leaflets are arranged in an anterior-posterior
orientation with right-left patent commissures in
approximately 60% of the cases (Fig. 3) and a right-left
orientation with anterior-posterior commissures in the
other 40%. 3,6 A fibrous ridge or raphe represents an
abortive commissure. The coronary arteries always arise
from the anterior sinus in the anterior-posterior type and
from the anterior aspect of each sinus in the right-left
type.
Concentric hypertrophy of the left ventricle (LV) due
to pressure overload and poststenotic dilatation of the
ascending aorta due to turbulent flow develop in due
course. Neonates with critical AS may have endocardial
fibroelastosis and varying degrees of LV hypoplasia. Long
standing moderate-to-severe AS in children may develop
marked myocardial fibrosis limited to the LV.
PATHOPHYSIOLOGY
The pathophysiology of AS depends on the severity of the
lesion, age of onset, and associated cardiac abnormalities.
The AS in symptomatic young infants is usually critical
in nature and is different in pathophysiology from the AS
in children and adolescents. The LV in young infant is
limited in its ability to match cardiac output to postnatal
demand due to a combination of small cavity, myocardial
ischemia, endocardial fibroelastosis, and decreased pre-
load.8 It may be further burdened by increased after-load
mismatch resulting from removal of the low-resistance
placental circulation after birth. Therefore, the systemic
circulation is very often duct-dependent. With closure of
the ductus arteriosus, systemic cardiac output and
peripheral perfusion diminish. Severe congestive heart
failure and metabolic acidosis then develop.
The AS in children is due to relatively unchanged
effective valve orifice with growth. The increased LV
mass induced by elevated wall stress over compensates
for pressure overload, which is characterized by a
subnormal wall stress and a supra-normal ejection
fraction. 9,10 The cardiac output in such groups is well
maintained both at rest and during exercise. However,
inadequate LV hypertrophy, not commensurate with
increasing wall-stress and altered contractility develop
and result in depressed cardiac pump function, low
cardiac output, decline in LV-aortic pressure gradient and
congestive heart failure.
CLINICAL PRESENTATIONS
Clinical presentations depend upon age of presentation
and severity of the AS.
Aortic Stenosis in Young Infants
Most infants usually appear normal at birth. The neonatal
and well-baby check-ups perform poorly as screening
Indian Journal of Pediatrics, Volume 69—April, 2002
 Aortic Stenosis

tests for severe AS unless the examiner has a high index of such as squatting and isotonic exercise, but is decreased
suspicion for CHDs.9 Some infants with critical AS die by maneuvers, which lower transvalvular flow such as
unrecognized. The majority of infants present with vasopressors, isometric exercise, or standing.
progressive congestive heart failure by 3 months of age.
Symptomatic infants are pale, mottled, hypotensive, and DIAGNOSTIC TECHNIQUES
dyspneac. A normal first heart sound, an intermittent soft Clinical symptoms and signs only suggest but do not
ejection click, and a gallop are present in approximately confirm the diagnosis of the AS in pediatric patients. Tests
one-half of the infants. An ejection systolic murmur of are usually needed to establish the morphologic diagnosis
variable intensity is present along the mid left and right of AS, severity of the obstruction, and adequacy of left
upper sternal border propagating to the carotid arteries. heart structures and function to support the systemic
Heptomeagaly is variable. The arterial blood gas has circulation to help formulate the nature and timing of the
reduced pao2 (30-40 torr) and significant metabolic intervention. For these purpose echocardiography is the
acidosis. If the LVOT obstruction is not relieved, the infant diagnostic tool of choice in pediatric patients. Other
with critical AS will have a progressive downhill course. modalities for diagnostic evaluation and monitoring will
Aortic Stenosis in Children and Adolescents be discussed briefly.
Children and adolescents with AS are usually
asymptomatic. Dyspnea, angina, or syncope, particularly
on exercise, is present in £ 10% of older children with
moderate and severe AS.11 However, sudden death has
been noted in 1 to 10% of children between 5 to 15 years
of age 12 with moderate-to-severe AS. Therefore,
symptoms of syncope and chest pain on exertion in this
age group deserve thorough evaluation.
The characteristic physical findings of adults with AS
are not uniformly present in children. The arterial pulsus
parvus et tardus (slowly rising, small but sustained pulse),
carotid shudder (anacrotic notch with systolic vibration),
and prominent a wave due to a “booster pump” action of
hypertrophied atria are not well appreciated in younger
children. A displaced and sustained apical impulse with
presystolic tap of left atrial contraction indicates
significant LV hypertrophy. A systolic thrill over the right
sternal border is present in over two-thirds of patients
with peak transaortic gradient exceeding 25 mmHg. Fig. 4. Valvular aortic stenosis with a doming valve. The 2-D
The first heart sound is usually normal but the second echocardiographic parasternal long axis view of a bicuspid
heart sound may be single or narrowly split because of aortic valve in systole is showing diminished cusps
prolonged LV systole (peak gradient exceeding 75 mmHg separation with eccentric orifice. Abbreviations as in Figure
1.
across LVOT). Paradoxical splitting of the second heart
sound suggests LV dysfunction but is rare in children. A
fourth heart sound is generally associated with severe ECHOCARDIOGRAPHY
obstruction. An ejection click, best heard just after the first
heart sound at the apex and along the left lower sternal Morphology of the Aortic Valve
border is due to a halting upward movement of a pliable The two-dimensional (2-D) echocardiography
doming aortic valve (absent in immobile valve). It is demonstrates cusp morphology namely number,
unaffected by respiration and absent in severe AS. The thickening and impaired mobility of the cusp with
characteristic systolic murmur of AS, which starts just characteristic valve doming, which are well seen in the
after ejection click is crescendo-decrescendo in shape, low parasternal views (Figs. 2-4). The suprasternal view shows
pitched, and best heard at the base of the heart with poststenotic aortic dilatation.
propagation to the carotid arteries. The configuration, but
Assessment of the Severity of the Valve Stenosis
not the length of the murmur, indicates the severity of the
obstruction. It tends to peak during the last two-thirds of LV Hypertrophy : The extent of concentric hypertrophy
the ventricular systole if the pressure gradient exceeds 75 indirectly reflects the severity of AS. Using a 2-D
mmHg. A high-pitched, short, and early diastolic murmur echocardiographic technique,13 the ratio of LV mass and
of aortic regurgitation is heard in approximately one- end-diastolic volume can assess the appropriateness of LV
fourth of the patients with AS. The murmur of AS is hypertrophy and the risk of myocardial ischemia. In
augmented by maneuvers which increase stroke volume neonates with critical AS, LV may be both hypertrophied

Indian Journal of Pediatrics, Volume 69—April, 2002 353

 Gautam K. Singh
and dilated with bright echogenic endocardium
secondary to endocardial fibroelastosis.
Transvalvular Pressure Gradients: The quantitative
assessment of the severity of AS is provided by the
transvalvular pressure gradient. The systolic blood flow
velocity, measured by the Doppler examination, is
converted to pressure gradient (DP) by using a simplified
Bernoulli equation: Dp = 4V2 where V is the maximum
Doppler velocity across the aortic valve measured by a
continuous wave Doppler interrogation. In clinical
practice, a catheter measured peak-to-peak pressure
gradient has been used as a primary measure of the
severity of AS. A peak-to-peak pressure gradient < 25
mmHg is considered trivial, 25 to 49 mmHg mild, 50 to 74
mmHg moderate, and = 75 mmHg severe AS. Although,
in general, the Doppler derived peak instantaneous
pressure gradient correlates well with catheter measured
peak-to-peak pressure gradient it very often
overestimates the catheter peak-to-peak gradient
particularly in children due to down stream pressure
recovery phenomenon. If recovered pressure (RP) is
deducted from the Doppler peak instantaneous pressure
gradient, the net pressure accurately predicts the catheter
peak-to-peak gradient.14,15 The RP can be derived by
echocardiography: RP = 4V2 2×AVA/AOA×(1- AVA/
AOA) where V is Doppler peak instantaneous velocity,
AVA is aortic valve area and AOA is ascending aorta
cross sectional area.14
Aortic Valve Area : AVA can be calculated by
continuity equation: AVA = ALVOT x VLVOT/VAV
where ALVOT is the LVOT area, VLVOT is the velocity
proximal to the stenotic aortic valve measured by the
pulsed Doppler, and VAV is the velocity across the
stenotic aortic valve obtained by the continuous wave
Doppler from multiple windows.16 Because the pressure
gradient, calculated from the measured velocity may not
be correct in low flow states and may not adequately
predict the severity of aortic stenosis, AVA calculated by
the continuity equation may be a more accurate measure
of the severity of AS. An effective valve area < 0.5 cm2/
m2 body surface area is considered as critical obstruction
whereas that > 0.7 cm2 / m2 body surface area indicates
mild obstruction (normal 1-2 cm2/m2).
Assessment of the Adequacy of the Left Heart Struc-
tures and Functions
In neonates with critical aortic stenosis, variability of the
left heart size and LV function determine whether
relieving the LVOT obstruction will achieve a two-
ventricle repair or whether a staged single-ventricle repair
(Norwood procedure) should be considered. Many
echocardiographic morphometric parameters and
hemodynamic variables of the left heart can be utilized to
help make the decision.17-19 Morphometric measurements
of the left heart structures, which favor survival after two-
ventricle repair are: an indexed aortic root at the level of
sinus of Valsalva (Root) ³ 3.5 cm/m2, an indexed mitral
354
valve area (MVA) ³ 4.75 cm2/m2, a ratio (LAR) of long-
axis of the LV and the heart ³ 0.8, and LV cross-sectional
area ³ 2.0 cm2 . Based on some of the morphometric
parameters, Rhodes et al developed a predictive equation
for survival: Discriminating score = 14.0 (BSA) + 0.943
(Root) + 4.78 (LAR) + 0.157 (MVA) -12.03. 17 A
discrimination score of less than - 0.35 was predictive of
poor outcome after two-ventricle repair.
An echocardiographically demonstrated predominant
or total antegrade flow in the ascending and transverse
aorta has been associated with survival after two-ventricle
repair in severe AS.19
CARDIAC CATHETERIZATION
Cardiac catheterization is now usually not performed for
establishing the diagnosis. It is indicated to discern the
severity of AS when multiple levels of obstruction in
series in LVOT are likely to influence the therapeutic
option, or interventional balloon aortic valvuloplasty is
planned. The left ventricle can be entered retrogradely via
umbilical artery in newborns and by percutaneous
femoral artery puncture in children. Alternatively, the LV
can be entered antegradely via the foramen ovale. In
pediatric practice, a peak-to-peak pressure gradient across
the LVOT is measured by the withdrawal of an end-hole
Fig. 5. Left ventriculogra in valvular aortic stenosis. A long axial
view of a left ventriculogram in systole is showing a doming
aortic valve with a small jet of contrast through the eccentric
valve orifice and poststenotic aortic dilation.
catheter from the LV to the aorta. The LV and aortic valve
are demonstrated by left axial oblique and elongated right
anterior oblique views (Fig. 5). The LV volume and
ejection fraction can be measured and cardiac output can
be calculated by the Fick technique or an indicator
dilution method. The aortic valve area can be calculated
from the Gorlin formula.20
ELECTROCARDIOAGRAM
The electrocardiographic changes do not predict the
Indian Journal of Pediatrics, Volume 69—April, 2002
 Aortic Stenosis
degree of severity of AS uniformly. A normal
electrocardiogram does not exclude a severe obstruction.
A relatively sensitive indicator of a severe AS is LV
hypertrophy with strain manifested by the widening of
the angle between the mean QRS and T-waves in the
frontal plane ³ 90 degrees and ST depression ³ 0.2 mV in
the left precordial leads. A progressive increase in the LV
voltage along with ST-segment and T-wave abnormalities
suggest increasing LVOT obstruction. A right ventricle
hypertrophy with a right axis deviation in the frontal
plane is the predominant presentation in neonates.
There is a higher incidence of serious arrhythmia
(multiform premature ventricular contraction, ventricular
couplets, and ventricular tachycardia) and sudden death
in pediatric patients with moderate to severe AS than in
the normal population. Therefore, ambulatory
electrocardiographic monitoring should be performed as
an important component of diagnostic evaluation of older
children and adolescents.
EXERCISE TESTING
Cardio-respiratory dysfunctions discerned by exercise
testing may be an important indication for therapeutic
intervention or indicator of its success. Older children and
adolescents can exercise on a treadmill or bicycle. Most
patients with moderate and severe VAS have blunted
increase in systolic blood pressure, which is inversely
related to the transaortic pressure gradient. The presence
of serious arrhythmias and the extent of ST- depression,
defined as deviation from the PQ isoelectric line 60 msec
after J point are significantly, inversely related to
transaortic gradients and indicative of myocardial
ischemia. A persistent serious cardiac arrhythmia or ST-
depression ³ 1mm is usually an indication for therapeutic
intervention.21 Blunted stroke volume index and cardiac
index are also seen at every level of exercise in patients
with severe AS.22
CHEST RADIOGRAPH
Cardiomegaly is the roentgenographic manifestation of
LV hypertrophy. However, it is not a sensitive marker of
the severity of AS and is present in 10% and 25% of
patients with pressure gradients between < 65mmHg and
> 80 mmHg respectively. Cardiomegaly is present in over
90% of symptomatic neonates with AS who also show
pulmonary venous congestion on chest radiograph.
Aortic valve calcification is usually not seen in pediatric
patients.
NATURAL HISTORY
Congenital AS is a progressive disorder. It carries an
annual mortality rate of up to 2.1% in the first three
decades in medically managed patients.23 Fewer than 20%
of even mild AS are likely to remain mild after 30 years.
Indian Journal of Pediatrics, Volume 69—April, 2002
In the Second Natural History Study, the peak systolic
gradient was found to be the most reliable indicator of the
clinical course.12 Beyond infancy; patients with catheter
measured peak pressure gradient of < 25mmHg have a
21% chance whereas those with a gradient of 25 to 49
mmHg have a 41% chance of requiring a valvotomy.
Patients with a gradient of ³ 50 mmHg have a 71% chance
of requiring an intervention for relief of obstruction,
which if unattended, will put them at significant risk for
serious ventricular arrhythmias, and possibly sudden
death.
In pediatric patients with congenital bicuspid aortic
valve, the progression of stenosis begins in the second
decade and is faster in the antero-posteriorly located
cusps.24
Bacterial endocarditis is a serious potential
complication of AS. In the Second Natural History Study,
the incidence rate of endocarditis was 27 per 10,000
person-years.25 There was an increased incidence after
surgical valvotomy, which was a function of severity of
the defect and not a function of surgery. Presence of aortic
regurgitation did not increase the risk of developing
bacterial endocarditis.
MANAGEMENT
Age at presentation, severity of the obstruction, and the
associated cardiac abnormalities determine the
management approach to the AS. The two current
therapeutic interventions to relieve the LVOT obstruction
are percutaneous balloon aortic valvoplasty (BAV) and
surgical aortic valvotomy/valve replacement.
BALLOON AORTIC VALVULOPLASTY
Since its initial report, 26 BAS has developed as an
effective alternative to surgical valvotomy in pediatric
patients.
Inflation of a balloon placed across the aortic valve
exerts radial forces on the stenotic lesion without axial
component and tears the weakest part of the valve
architect. In bicuspid aortic valve AS the fused
commissures are the weakest links that break with
balloon dilation and result in adequate relief of
obstruction with some valvular insufficiency. However,
balloon dilation in unicuspid valve AS tends to split the
leaflet opposite the patent commissure resulting in only
partial relief of obstruction but with significant valvular
insufficiency. Balloon Inflation up to the recommended
atmospheric pressure produces valvuloplasty. The
recommended balloon/annulus ratio is 0.8 to 1.0 or at
least 1mm smaller than the aortic annulus.27
Immediate and Intermediate Results : Immediate
reduction in peak pressure gradient across the aortic valve
gradient occurs in the majority of patients. In Pediatric
Valvuloplasty Registry on 204 patients, mean
transvalvular gradient decreased from 77 to 30 mmHg.28
355
 Gautam K. Singh
Mortality risk was 2.4 %, and confined mostly to patients
3 months old or less. Aortic regurgitation increased in
10.2%. Intermediate-term follow-up in a series of neonatal
BAV, mortality was 12% and rate of re-intervention was
41%.29 Intermediate-term follow-up in another series
mostly consisting of children, showed no restenosis and
74% of patients had no change in the degree of aortic
insufficiency.30 Predictors of restenosis are age £ 3 years
and immediate post-valvuloplasty peak gradient ³ 30
mmHg.31
Late Results : There are limited data on late follow-
up. 32 The late results suggest that progressive aortic
regurgitation develops in approximately 25% patients
who would need either valve repair or valve replacement
in the long term.32
SURGERY
The surgical valvotomy in neonates and young infants
with critical AS is usually performed by dividing fused
commissures and adequate leaflet attachment to the aortic
wall through transverse aortotomy. Division of
rudimentary commissures produces aortic regurgitation.
In patients with essentially uncorrectable valve
(unicuspid or severely dysplastic bicuspid valve), small
aortic annulus, or recurrent stenosis with significant aortic
regurgitation, an aortic valve replacement may be done in
standard fashion. Because of proven viability and
potential for growth, pulmonary autograft is now used
for valve replacement (Ross procedure) in many centers
in all age group.33 A prosthetic valve or an aortic valve
allograft can also be used for valve replacement.
Immediate-and-long-term result: The early mortality
after surgical valvotomy in neonates and young infants
with critical AS in previous reports has been 13% to 100%,
whereas that in children and adolescents has been 2 % or
less. 34 Incremental risk factors for mortality were
coexisting severe left-sided cardiac defects and young age
at operation. With recent approach in treating patients
with associated left heart defects (particularly AS with
small LV) by staged single-ventricle repair or transplant,
surgical mortality in neonates and infants has
significantly reduced. The 25-year survival after the
primary operation in children and adolescents is up to
90% but freedom from re-operation is only 60%.34 In one
large series actuarial survival and freedom from an
additional aortic operation after valve replacement with
prosthesis or aortic valve allograft was 49 ± 8 % at 9 years
whereas those with a pulmonary autograft was 89 ± 4 %.33
MANAGEMENT OF INFANTS WITH CRITICAL AS
Young infants with critical AS present with congestive
heart failure and may rapidly deteriorate to shock with
metabolic acidosis. They should be treated aggressively
with prostagland E1 infusion, inotrophic support,
diuretic, correction of metabolic acidosis, and mechanical
356
ventilation to improve the systemic perfusion.
Echocardiographic evaluation helps to determine whether
an intervention to achieve a two-ventricle status or a
staged single-ventricle repair should be undertaken.
Whether the intervention in those patients in whom
aortic valvotomy alone is indicated, should consist of
percutaneous BAV or surgical valvotomy remains
arguable. The centers that have catheter-interventional
expertise choose to perform expeditious BAV in unstable
and markedly symptomatic young infants. At our center
anterograde transumbilical venous balloon aortic
valvuloplasty is the preferred initial option.35 However,
surgical valvotomy remains a satisfactory option. Where
valve stenosis is uncorrectable, aortic valve replacement
with pulmonary autograft is an attractive option.
MANAGEMENT OF CHILDREN AND
ADOLESCENTS WITH AS
Some guidelines for therapeutic intervention are provided
in Table 1. In patients with severe AS having peak
gradient ³ 80 mmHg or aortic valve area < 0.5 cm2/m2
body surface area, immediate relief of obstruction is
clearly indicated. 12 Intervention in patients with
moderate AS having peak gradients of 50-79 mmHG and
valve area of > 0.5 cm 2 /m 2 but < 0.7 cm 2 /m 2 is
controversial because early operation does not delay the
potential need for valve replacement whereas unrelieved
obstruction poses significant risk for serious
arrhythmias.12 However, LV hypertrophy with strain at
rest or during exercise testing or symptoms of syncope or
angina are indications for intervention even though the
gradient may be <50 mmHg. The type of intervention
remains arguable. Most centers prefer percutaneous BAV
because good results have been obtained in children and
adolescents with this technique that may be comparable
to those obtained surgically.
TABLE 1. Aortic Stenosis : Indications for Intervention.
A combination of clinical features and any one of the investigations
provides clear indication for therapeutic intervention.
1. Clinical Features
Presence of dyspnea on exertion, angina, or syncope
Arterial pusus parvus et tardus
Configuration of the systolic murmur with late peaking
2. Echocardiographic Features
Left ventricular mass/volume ratio >2:1
Peak instantaneous pressure gradient across aortic valve >75 mmHg
Mean pressure gradient > 50 mmHg
Aortic valve orifice <0.5 cm2/m2 body surface area
3. Catheterization Measured Features
Peak-to-peak pressure gradient >75 mmHg
Aortic valve orifice <0.5 cm2/m2 body surface area
4. Electrocardiographic Features
Left ventricular hypertrophy with strain
ST-segment depression >1 mm at rest or during exercise
Indian Journal of Pediatrics, Volume 69—April, 2002
 Aortic Stenosis
MEDICAL MANAGEMENT
All patients of AS, regardless of the severity of obstruction
and intervention, need prophylaxis against bacterial
endocarditis. Since AS is a progressive disorder and is
associated with exercise-induced sudden death in those
with moderate to severe obstruction, the need for careful
follow-up cannot be over emphasized.
Patients with peak gradient <25 mmHg should be
periodically evaluated at 1 to 2 years interval by 2-D and
Doppler echocardiography. They usually do not need
restriction in physical activities. Patients with a gradient of
25 to 49 mmHg should not participate in competitive
sports or games but can participate in recreational
activities if exercise testing is normal. They should be
evaluated at least yearly by 2-D and Doppler
echocardiography and exercise testing.
Patients with peak gradient of 50 to 75 mmHg should
be restricted to light exercise. Because of an increased risk
of serious ventricular arrhythmias and possibly sudden
death, delaying intervention in these patients may not be
advantageous.12
PROGNOSIS
Patients with AS have a 25-year survival of 85%.12 Forty
percent of medically managed patients subsequently
require surgical intervention, whereas almost 40% of the
operated patients require a second operation. More than
half of the patients are likely to remain in New York Heart
Association class I in long-term.12
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Indian Journal of Pediatrics, Volume 69—April, 2002