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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


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 : group.

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

Gautam K. Singh

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
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.
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.
The pathophysiology of AS depends on the severity of the
Unicuspid Valve: The unicuspid aortic valve has only lesion, age of onset, and associated cardiac abnormalities.
one patent commissure. 4,5 The proximal aortic root The AS in symptomatic young infants is usually critical
circumference is approximately half of the normal and the in nature and is different in pathophysiology from the AS
length of the free edge of the leaflet is less than the in children and adolescents. The LV in young infant is
circumference of the sinus. 3,4 The leaflet is single, limited in its ability to match cardiac output to postnatal
thickened, and rigid. The valve orifice is eccentric (Fig. 2). demand due to a combination of small cavity, myocardial
Therefore, a limited flap-valve type of opening during ischemia, endocardial fibroelastosis, and decreased pre-
ejection of the blood occurs and results in intrinsic load.8 It may be further burdened by increased after-load
obstruction to flow. Unicuspid valve in critical aortic mismatch resulting from removal of the low-resistance
stenosis often has a centrally situated orifice and no placental circulation after birth. Therefore, the systemic
commissure.5 Both types of architectures of unicuspid circulation is very often duct-dependent. With closure of
valve do not lend themselves well to closed valvotomy the ductus arteriosus, systemic cardiac output and
and balloon dilatation. 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 depend upon age of presentation

and severity of the AS.
Fig. 3. Valvular aortic stenosis with a bicuspid valve. The 2-D
echocardiographic parasternal short axis view of a bicuspid Aortic Stenosis in Young Infants
aortic valve in systole is showing an ellipsoidal orifice and
anterior-posterior arrangement of the two leaflets. Most infants usually appear normal at birth. The neonatal
Abbreviations as in Fig. 1. and well-baby check-ups perform poorly as screening

352 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
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 valve area (MVA) ³ 4.75 cm2/m2, a ratio (LAR) of long-
secondary to endocardial fibroelastosis. axis of the LV and the heart ³ 0.8, and LV cross-sectional
Transvalvular Pressure Gradients: The quantitative area ³ 2.0 cm2 . Based on some of the morphometric
assessment of the severity of AS is provided by the parameters, Rhodes et al developed a predictive equation
transvalvular pressure gradient. The systolic blood flow for survival: Discriminating score = 14.0 (BSA) + 0.943
velocity, measured by the Doppler examination, is (Root) + 4.78 (LAR) + 0.157 (MVA) -12.03. 17 A
converted to pressure gradient (DP) by using a simplified discrimination score of less than - 0.35 was predictive of
Bernoulli equation: Dp = 4V2 where V is the maximum poor outcome after two-ventricle repair.
Doppler velocity across the aortic valve measured by a An echocardiographically demonstrated predominant
continuous wave Doppler interrogation. In clinical or total antegrade flow in the ascending and transverse
practice, a catheter measured peak-to-peak pressure aorta has been associated with survival after two-ventricle
gradient has been used as a primary measure of the repair in severe AS.19
severity of AS. A peak-to-peak pressure gradient < 25
mmHg is considered trivial, 25 to 49 mmHg mild, 50 to 74 CARDIAC CATHETERIZATION
mmHg moderate, and = 75 mmHg severe AS. Although, Cardiac catheterization is now usually not performed for
in general, the Doppler derived peak instantaneous establishing the diagnosis. It is indicated to discern the
pressure gradient correlates well with catheter measured severity of AS when multiple levels of obstruction in
peak-to-peak pressure gradient it very often series in LVOT are likely to influence the therapeutic
overestimates the catheter peak-to-peak gradient option, or interventional balloon aortic valvuloplasty is
particularly in children due to down stream pressure planned. The left ventricle can be entered retrogradely via
recovery phenomenon. If recovered pressure (RP) is umbilical artery in newborns and by percutaneous
deducted from the Doppler peak instantaneous pressure femoral artery puncture in children. Alternatively, the LV
gradient, the net pressure accurately predicts the catheter can be entered antegradely via the foramen ovale. In
peak-to-peak gradient.14,15 The RP can be derived by pediatric practice, a peak-to-peak pressure gradient across
echocardiography: RP = 4V2 2×AVA/AOA×(1- AVA/ the LVOT is measured by the withdrawal of an end-hole
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). Fig. 5. Left ventriculogra in valvular aortic stenosis. A long axial
view of a left ventriculogram in systole is showing a doming
Assessment of the Adequacy of the Left Heart Struc-
aortic valve with a small jet of contrast through the eccentric
tures and Functions valve orifice and poststenotic aortic dilation.
In neonates with critical aortic stenosis, variability of the
left heart size and LV function determine whether catheter from the LV to the aorta. The LV and aortic valve
relieving the LVOT obstruction will achieve a two- are demonstrated by left axial oblique and elongated right
ventricle repair or whether a staged single-ventricle repair anterior oblique views (Fig. 5). The LV volume and
(Norwood procedure) should be considered. Many ejection fraction can be measured and cardiac output can
echocardiographic morphometric parameters and be calculated by the Fick technique or an indicator
hemodynamic variables of the left heart can be utilized to dilution method. The aortic valve area can be calculated
help make the decision.17-19 Morphometric measurements from the Gorlin formula.20
of the left heart structures, which favor survival after two- ELECTROCARDIOAGRAM
ventricle repair are: an indexed aortic root at the level of
sinus of Valsalva (Root) ³ 3.5 cm/m2, an indexed mitral The electrocardiographic changes do not predict the

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

Aortic Stenosis

degree of severity of AS uniformly. A normal In the Second Natural History Study, the peak systolic
electrocardiogram does not exclude a severe obstruction. gradient was found to be the most reliable indicator of the
A relatively sensitive indicator of a severe AS is LV clinical course.12 Beyond infancy; patients with catheter
hypertrophy with strain manifested by the widening of measured peak pressure gradient of < 25mmHg have a
the angle between the mean QRS and T-waves in the 21% chance whereas those with a gradient of 25 to 49
frontal plane ³ 90 degrees and ST depression ³ 0.2 mV in mmHg have a 41% chance of requiring a valvotomy.
the left precordial leads. A progressive increase in the LV Patients with a gradient of ³ 50 mmHg have a 71% chance
voltage along with ST-segment and T-wave abnormalities of requiring an intervention for relief of obstruction,
suggest increasing LVOT obstruction. A right ventricle which if unattended, will put them at significant risk for
hypertrophy with a right axis deviation in the frontal serious ventricular arrhythmias, and possibly sudden
plane is the predominant presentation in neonates. death.
There is a higher incidence of serious arrhythmia In pediatric patients with congenital bicuspid aortic
(multiform premature ventricular contraction, ventricular valve, the progression of stenosis begins in the second
couplets, and ventricular tachycardia) and sudden death decade and is faster in the antero-posteriorly located
in pediatric patients with moderate to severe AS than in cusps.24
the normal population. Therefore, ambulatory Bacterial endocarditis is a serious potential
electrocardiographic monitoring should be performed as complication of AS. In the Second Natural History Study,
an important component of diagnostic evaluation of older the incidence rate of endocarditis was 27 per 10,000
children and adolescents. person-years.25 There was an increased incidence after
surgical valvotomy, which was a function of severity of
EXERCISE TESTING the defect and not a function of surgery. Presence of aortic
regurgitation did not increase the risk of developing
Cardio-respiratory dysfunctions discerned by exercise bacterial endocarditis.
testing may be an important indication for therapeutic
intervention or indicator of its success. Older children and MANAGEMENT
adolescents can exercise on a treadmill or bicycle. Most
patients with moderate and severe VAS have blunted Age at presentation, severity of the obstruction, and the
increase in systolic blood pressure, which is inversely associated cardiac abnormalities determine the
related to the transaortic pressure gradient. The presence management approach to the AS. The two current
of serious arrhythmias and the extent of ST- depression, therapeutic interventions to relieve the LVOT obstruction
defined as deviation from the PQ isoelectric line 60 msec are percutaneous balloon aortic valvoplasty (BAV) and
after J point are significantly, inversely related to surgical aortic valvotomy/valve replacement.
transaortic gradients and indicative of myocardial
ischemia. A persistent serious cardiac arrhythmia or ST- BALLOON AORTIC VALVULOPLASTY
depression ³ 1mm is usually an indication for therapeutic
intervention.21 Blunted stroke volume index and cardiac Since its initial report, 26 BAS has developed as an
index are also seen at every level of exercise in patients effective alternative to surgical valvotomy in pediatric
with severe AS.22 patients.
Inflation of a balloon placed across the aortic valve
CHEST RADIOGRAPH exerts radial forces on the stenotic lesion without axial
component and tears the weakest part of the valve
Cardiomegaly is the roentgenographic manifestation of architect. In bicuspid aortic valve AS the fused
LV hypertrophy. However, it is not a sensitive marker of commissures are the weakest links that break with
the severity of AS and is present in 10% and 25% of balloon dilation and result in adequate relief of
patients with pressure gradients between < 65mmHg and obstruction with some valvular insufficiency. However,
> 80 mmHg respectively. Cardiomegaly is present in over balloon dilation in unicuspid valve AS tends to split the
90% of symptomatic neonates with AS who also show leaflet opposite the patent commissure resulting in only
pulmonary venous congestion on chest radiograph. partial relief of obstruction but with significant valvular
Aortic valve calcification is usually not seen in pediatric insufficiency. Balloon Inflation up to the recommended
patients. atmospheric pressure produces valvuloplasty. The
recommended balloon/annulus ratio is 0.8 to 1.0 or at
NATURAL HISTORY least 1mm smaller than the aortic annulus.27
Immediate and Intermediate Results : Immediate
Congenital AS is a progressive disorder. It carries an reduction in peak pressure gradient across the aortic valve
annual mortality rate of up to 2.1% in the first three gradient occurs in the majority of patients. In Pediatric
decades in medically managed patients.23 Fewer than 20% Valvuloplasty Registry on 204 patients, mean
of even mild AS are likely to remain mild after 30 years. transvalvular gradient decreased from 77 to 30 mmHg.28

Indian Journal of Pediatrics, Volume 69—April, 2002 355

Gautam K. Singh

Mortality risk was 2.4 %, and confined mostly to patients ventilation to improve the systemic perfusion.
3 months old or less. Aortic regurgitation increased in Echocardiographic evaluation helps to determine whether
10.2%. Intermediate-term follow-up in a series of neonatal an intervention to achieve a two-ventricle status or a
BAV, mortality was 12% and rate of re-intervention was staged single-ventricle repair should be undertaken.
41%.29 Intermediate-term follow-up in another series Whether the intervention in those patients in whom
mostly consisting of children, showed no restenosis and aortic valvotomy alone is indicated, should consist of
74% of patients had no change in the degree of aortic percutaneous BAV or surgical valvotomy remains
insufficiency.30 Predictors of restenosis are age £ 3 years arguable. The centers that have catheter-interventional
and immediate post-valvuloplasty peak gradient ³ 30 expertise choose to perform expeditious BAV in unstable
mmHg.31 and markedly symptomatic young infants. At our center
Late Results : There are limited data on late follow- anterograde transumbilical venous balloon aortic
up. 32 The late results suggest that progressive aortic valvuloplasty is the preferred initial option.35 However,
regurgitation develops in approximately 25% patients surgical valvotomy remains a satisfactory option. Where
who would need either valve repair or valve replacement valve stenosis is uncorrectable, aortic valve replacement
in the long term.32 with pulmonary autograft is an attractive option.


The surgical valvotomy in neonates and young infants
with critical AS is usually performed by dividing fused Some guidelines for therapeutic intervention are provided
commissures and adequate leaflet attachment to the aortic in Table 1. In patients with severe AS having peak
wall through transverse aortotomy. Division of gradient ³ 80 mmHg or aortic valve area < 0.5 cm2/m2
rudimentary commissures produces aortic regurgitation. body surface area, immediate relief of obstruction is
In patients with essentially uncorrectable valve clearly indicated. 12 Intervention in patients with
(unicuspid or severely dysplastic bicuspid valve), small moderate AS having peak gradients of 50-79 mmHG and
aortic annulus, or recurrent stenosis with significant aortic valve area of > 0.5 cm 2 /m 2 but < 0.7 cm 2 /m 2 is
regurgitation, an aortic valve replacement may be done in controversial because early operation does not delay the
standard fashion. Because of proven viability and potential need for valve replacement whereas unrelieved
potential for growth, pulmonary autograft is now used obstruction poses significant risk for serious
for valve replacement (Ross procedure) in many centers arrhythmias.12 However, LV hypertrophy with strain at
in all age group.33 A prosthetic valve or an aortic valve rest or during exercise testing or symptoms of syncope or
allograft can also be used for valve replacement. angina are indications for intervention even though the
Immediate-and-long-term result: The early mortality gradient may be <50 mmHg. The type of intervention
after surgical valvotomy in neonates and young infants remains arguable. Most centers prefer percutaneous BAV
with critical AS in previous reports has been 13% to 100%, because good results have been obtained in children and
whereas that in children and adolescents has been 2 % or adolescents with this technique that may be comparable
less. 34 Incremental risk factors for mortality were to those obtained surgically.
coexisting severe left-sided cardiac defects and young age
at operation. With recent approach in treating patients TABLE 1. Aortic Stenosis : Indications for Intervention.
with associated left heart defects (particularly AS with A combination of clinical features and any one of the investigations
small LV) by staged single-ventricle repair or transplant, provides clear indication for therapeutic intervention.
surgical mortality in neonates and infants has
1. Clinical Features
significantly reduced. The 25-year survival after the
Presence of dyspnea on exertion, angina, or syncope
primary operation in children and adolescents is up to Arterial pusus parvus et tardus
90% but freedom from re-operation is only 60%.34 In one Configuration of the systolic murmur with late peaking
large series actuarial survival and freedom from an 2. Echocardiographic Features
additional aortic operation after valve replacement with
Left ventricular mass/volume ratio >2:1
prosthesis or aortic valve allograft was 49 ± 8 % at 9 years Peak instantaneous pressure gradient across aortic valve >75 mmHg
whereas those with a pulmonary autograft was 89 ± 4 %.33 Mean pressure gradient > 50 mmHg
Aortic valve orifice <0.5 cm2/m2 body surface area
MANAGEMENT OF INFANTS WITH CRITICAL AS 3. Catheterization Measured Features
Peak-to-peak pressure gradient >75 mmHg
Young infants with critical AS present with congestive Aortic valve orifice <0.5 cm2/m2 body surface area
heart failure and may rapidly deteriorate to shock with 4. Electrocardiographic Features
metabolic acidosis. They should be treated aggressively
Left ventricular hypertrophy with strain
with prostagland E1 infusion, inotrophic support,
ST-segment depression >1 mm at rest or during exercise
diuretic, correction of metabolic acidosis, and mechanical
356 Indian Journal of Pediatrics, Volume 69—April, 2002
Aortic Stenosis

MEDICAL MANAGEMENT 10. Borow KM, Colan SD, Neumann A. Altered left ventricular
mechanics in patients with valvular aortic stenosis and
All patients of AS, regardless of the severity of obstruction coarctation of the aorta: effects on systolic performance and
and intervention, need prophylaxis against bacterial late outcome. Circulation 1985; 72 : 515-522.
endocarditis. Since AS is a progressive disorder and is 11. Report from the Joint Study on the Natural History of
Congenital Heart Defects. Circulation 1977; 56(Suppl-I) : I-1-I-
associated with exercise-induced sudden death in those
with moderate to severe obstruction, the need for careful 12. Keane JF, Driscoll DJ, Gersony WM et al. Second natural
follow-up cannot be over emphasized. history study of congenital heart defects: results of treatment
Patients with peak gradient <25 mmHg should be of patients with aortic valvar stenosis. Circulation 1993;
periodically evaluated at 1 to 2 years interval by 2-D and 87(Suppl I) : I-16-I-27.
13. Schiller NB, Shah PM, Crawford M et al. Recommendations
Doppler echocardiography. They usually do not need
for quantitation of the left ventricle by two-dimensional
restriction in physical activities. Patients with a gradient of echocardiography. J Am Soc Echocardiogr 1989; 2 : 358-367.
25 to 49 mmHg should not participate in competitive 14. Baumgartner H, Stefenelli T, Niederberger J, Schima H,
sports or games but can participate in recreational Maurer G. “Overestimation” of catheter gradients by Doppler
activities if exercise testing is normal. They should be ultrasound in patients with aortic stenosis: a predictable
evaluated at least yearly by 2-D and Doppler manifestation of pressure recovery. J Am Coll Cardiol 1999;
33 : 1655-1661.
echocardiography and exercise testing. 15. Singh GK, Marino CJ, Oliver D, Balfour I, Chen S, Jureidini S
Patients with peak gradient of 50 to 75 mmHg should et al. Importance of pressure recovery in the evaluation of
be restricted to light exercise. Because of an increased risk stenotic outflow tract lesions in pediatric patients: a
of serious ventricular arrhythmias and possibly sudden simultaneous Doppler and catheter correlative study (abstr).
death, delaying intervention in these patients may not be J Am Soc Echocardiogr 2001; 13: 474.
16. Oh JK, Taliercio CP, Holmes DR Jr et al. Prediction of the
severity of aortic stenosis by Doppler aortic valve area
determination: prospective Doppler-catheterization
PROGNOSIS correlation in 100 patients. J Am Coll Cardiol 1988; 11 : 1277-
Patients with AS have a 25-year survival of 85%.12 Forty 17. Rhodes LA, Colan SD, Perry SB, Jonas RA, Sanders SP.
Predictors of survival in neonates with critical aortic stenosis.
percent of medically managed patients subsequently Circulation 1991; 84 : 2325-2335.
require surgical intervention, whereas almost 40% of the 18. Parsons MK, Moreau GA, Graham TP Jr, Johns JA, Boucek RJ
operated patients require a second operation. More than Jr. Echocardiographic estimation of critical left ventricular size
half of the patients are likely to remain in New York Heart in infants with isolated aortic valve stenosis. J Am Coll Cardiol
Association class I in long-term.12 1991; 18 : 1049-1055.
19. Kovalchin JP, Brook MM, Rosenthal GL, Suda K, Hoffman JI,
Silverman NH. Echocardiographic hemodynamic and
REFERENCES morphometric predictors of survival after two-ventricle repair
in infants with critical aortic stenosis. J Am Coll Cardiol 1998;
1. Hoffman, JEI, Christianson R. Congenital heart disease in a 32 : 237.
cohort of 19,502 births with long-term follow-up. Am J Cardiol 20. Gorlin R, Gorlin SG. Hydraulic fromula for calculation of the
1978; 42 : 641-647. valve area of the stenotic mitral valve, other cardiac valves,
2. Braunwald E, Goldblatt A, Augen MM, Rockoff SD, Morroco and central circulatory shunts. Am Heart J 1951; 41 : 1-29.
AG. Congenital aortic stenosis: clinical and hemodynamic 21. Driscoll DJ, Wolfe RR, Gersony WM et al. Cardiorespiratory
findings in 100 patients. Circulation 1963; 27 : 426-462. response to exercise of patients with aortic stenosis,
3. Angelini A, Ho SY, Anderson RH, Devine WA, Zuberbuhler pulmonary stenosis, and ventricular septal defect. Circulation
JR, Becker AE et al. The Morphology of the normal aortic valve 1993; (Suppl I) : I-102-I-109.
as compared with the aortic valve having two leaflets. J Thorac 22. Cyran SE, James FW, Daniels S, Mays W, Shukla R, Kaplan S.
Cardiovasc Surg 1989; 98 : 362-367. Comparision of the cardiac output and stroke volume
4. McKay R, Smith A, Leung MP, Arnold R, Anderson RH. response to upright exercise in children with valvular and
Morphology of the ventriculoaortic junction in critical aortic subvalvular aortic stenosis. J Am Coll Cardiol 1988; 11 : 651-
stenosis: implications for hemodynamic function and clinical 658.
management. J Thorac Cardiovasc Surg 1992; 104 : 434-442. 23. Campbell M. The natural history of congenital aortic stenosis.
5. Edwards JE. Pathology of left ventricular outflow tract Br Heart J 1968; 30 : 514.
obstruction. Circulation 1965; 31 : 586-599. 24. Beppu S, Suzuki S, Matsuda H, Ohmori F, Nagata S, Miyatake
6. Roberts WC. The congenitally bicuspid aortic valve: a study of K. Rapidity of progression of aortic stenosis in patients with
85 autopsy cases. Am J Cardiol 1970; 26 : 72-83. congenital bicuspid aortic valves. Am J Cardiol 1993; 71 : 322-
7. Simpson JM, Sharland GK. Natural history and outcome of 327.
aortic stenosis diagnosed prenatally. Heart 1997; 77 : 205-210. 25. Gersony WM, Hayes CJ, Driscoll DJ et al. Bacterial
8. Lakier JB, Lewis AB, Heymann MA, Stanger P, Hoffman JIE, endocarditis in patients with aortic stenosis, or ventricular
Rudolph AM. Isolated aortic stenosis in the neonate: natural septal defect. Circulation 1993; (Suppl I) : I-121-I-126.
history and hemodynamic considerations. Circulation 1974; 50 26. Labibidi Z, Weinhaus L. Successful balloon valvuloplasty for
: 801-818. neonatal critical aortic stenosis. Am Heart J 1986; 112 : 913-916.
9. Donner R, Carabello BA, Black I, Spann JE. Left ventricular 27. Zeevi B, Keane JF, Castaneda AR, Perry SB, Lock JE. Neonatal
wall stress in compensated aortic stenosis. Am J Cardiol 1983; critical valvar aortic stenosis: A comparison of surgical and
51 : 946-951. balloon dilatation therapy. Circulation 1989; 80 : 831-839.

Indian Journal of Pediatrics, Volume 69—April, 2002 357

Gautam K. Singh

28. Rocchini AP, Beckman RH, Shachar GB et al. Balloon aortic causes of late aortic insufficiency. Am Heat J 1997; 133 : 418-
valvuloplasty: results of valvuloplasty and angioplasty of 827.
congenital anomalies registry. Am J Cardiol 1990; 65 : 784-789. 32. Rao PS. Long-term follow-up results after balloon dilatation of
29 Egito EST, Moore P, O’Sullivan J, Colan S, Perry SB, Lock JE pulmonary stenosis, aortic stenosis and coarctation of the
et al. Transvascular balloon dilatation for neonatal critical aorta: a review. Progress in Cardiovasc Dis 1999; 45 : 59-74 .
aortic stenosis: early and mid-term results. J Am Coll Cardiol 33. Elkins RC. Congenital aortic valve disease: evolving
1997; 29 : 442-447. management. Ann Thorac Surg 1995; 59 : 269-274.
30. O’Connor BK, Beekman RH, Rocchini AP, Rosenthal A. 34. Kirklin JW, Barratt-Boyes BG, eds. Cardiac Surgery 1993; 2 :
Intermediate term effectiveness of balloon valvuloplasty for 1195-1238. New York: Churchill Livington.
congenital aortic stenosis: a prospective follow-up study. 35. Rao PS, Jurideini SB. Transumbilical venous, anterograde,
Circulation 1991; 84 : 732-738. snare-assisted balloon aortic valvuloplasty in a neonate with
31. Galal O, Rao PS, Al-Fadley F et al. Follow-up results of balloon critical aortic stenosis. Cathet Cardiovasc Intervent 1998; 45 :
aortic valvuloplasty in children with special reference to 144-148.

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