Diagnosis and Management of Heart Murmurs in Children

Jerome Liebman
Pediatrics in Review 1982;3;321
DOI: 10.1542/pir.3-10-321

The online version of this article, along with updated information and services, is located on
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http://pedsinreview.aappublications.org/content/3/10/321

Pediatrics in Review is the official journal of the American Academy of Pediatrics. A monthly
publication, it has been published continuously since 1979. Pediatrics in Review is owned, published, and
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Village, Illinois, 60007. Copyright 1982 by the American Academy of Pediatrics. All rights reserved.
Print ISSN: 0191-9601.

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Diagnosis
Murmurs
Jerome

and Management
in Children

Liebman,

MD*

The physician
taking
care of children
must
meet
the problem
of an
easily
audible
heart
murmur
almost
every
day.
In order
to do so, the
physician
must
be able to perform
an excellent
cardiovascular
examination,
should
have an approach
to
making
the differential
between
abnormal
and
normal
murmurs,
and
should
be able to figure
out what the
common
cardiac
lesions
sound
like.

If

one

places

an

intracardiac

phonocatheter
at the root (origin)
of
the pulmonary
artery
or root of the
aorta
in all nondiseased
people,
a
heart
murmur
will be documented
(Figs
1 and 2).13
This
murmur
is
present
for two reasons:
(1 ) because
the origin
of each
great
artery
is
narrower
than
the ventricle
which
ejects
the blood
into it, and (2) because
each great artery
arises
from
the ventricle
at an angle.
A useful
analogue
to explain
the normal
murmur to families
is to suggest
that
they
go to the basement
of their
home,
find a water
pipe,
and listen
with ears close
to the pipe.
As the
water
goes
through
the pipe it can
easily
be heard,
and, of course,
that
is a murmur.
If one finds the input to
that water
pipe and turns the spigot
such that the space
for the entering
stream
is narrowed,
that murmur
will
become
louder;
and if then one finds
an angle
in the pipe
where
turbulence
is likely,
the murmur
is again
recognized
to be louder.
As pediatric radiologists
are constantly
reminding
us, the heart
of the child,
particularly
the
preschool-aged
child,
is very close to the chest wall.
As all nondiseased
people
have
a
murmur
in the root of the aorta
and
pulmonary
artery,
and as the heart
of the child is close to the chest,
it is
easy
to explain
why
these
normal
murmurs
are often
so well heard
on
the surface
of the childs
chest
(Fig
3). (The term
functional
murmur
is not used by this author,
for functional
murmurs
are not normal.
An

*
Department
bow
Babies
land.

of Heart

of Pediatric
and Children

Cardiology,

s Hospital,

RainCleve-

example
is provided
by the hemodynamics
in atrial septal
defect
with
a 4:1
pulmonary
to systemic
flow
ratio.
This is four times
the normal
flow through
normal
pulmonary
and
tricuspid
valves,
causing
abnormal
murmurs
which
are a function
of this
increased
flow.)
The incidence
of congenital
heart
disease
is approximately
.8/i
00
(although
it may approach
1 / 100),
but that number
is small by comparison with
the numbers
of children
with normal
murmurs.
Therefore,
the
pediatrician
s differential
diagnosis
when
he or she hears
a murmur
in
an asymptomatic
child
starts
with
the likelihood
that the murmur
is normal.
As medical
knowledge
has
expanded,
many
physicians
have
come
to believe
that cardiology
is a
black
box
field,
entirely
dependent upon
sophisticated
equipment
and methodology,
both noninvasive
and invasive.
The latter
is certainly
the case for complex
disease,
but it
is far from
true
in the majority
of
situations.
The matrix
of the evaluation is the physical
examination,4
abetted,
as indicated,
by the electrocardiogram89
and the chest
roentgenogram.
Echocardiography
provides
such remarkable
tools for the
cardiologists
and has had such publicity,
that it is often
utilized
unnecessarily.
In the evaluation
of most
children
(after
infancy)
with
heart
murmurs,
it is usually
not necessary.

EDUCATIONAL

22.

OBJECTIVE

Appropriate

recognition

of

the common
functional
heart murmurs, with ability to differentiate
them from organic
murmurs,
with
knowledge
of clinical
features
of
common
cardiac defects,
and with
the ability to make appropriate
referrals for further
evaluation
and
to counsel the child and family appropriately
(Topics 81/82).

femoral
pulses
ora decrease
in one
of the brachial
pulses,
then two upper extremities
and one leg pressure
should
be measured.
One
should
also
estimate
the pulse
pressure,
for,
with
experience,
the examiner
will be able to be more
accurate
in

CARDIOVASCULAR
EXAMINATION

determining

narrow,

anterior-posterior

In Fig 4 is shown
the form used for
the physical
exmination
of a patient
being
evaluated
at the congenital
heart
ambulatory
unit at Rainbow
Babies
and Childrens
Hospital.
This
form
helps
in maintaining
the discipline
required
for
an
appropriate
cardiac
examination.
Some
of the
items in Fig 4 deserve
comment:
1. If the femoral
pulses
(felt
simultaneously
with the right as well
as left brachiai)
are well felt, then
only
a right
arm
blood
pressure
is
necessary.
If there
is a decrease
in
pediatrics

in review

average,

or

wide pulse pressure

than will an ausculted
pressure
in a baby
or a not
very
cooperative
preschool-aged
child.
2. Mild cyanosis
may be difficult
to recognize.
Most
people
find that
the fingernails
are an excellent
place
for observation.
In addition,
proximal
to the nails,
the fingers
will sometimes be red and shiny in many congenital
cardiac
lesions.
Although
this
may indicate
mild cyanosis,
the redness
is usually
an associated
congenital
anomaly
due to congenitally
large capillaries.
3. A left chest
prominence
may
indicate
a large
heart-but
make
note of a pectus
as well as a narrow
Both

chest

of these

diameter.

cause
the heart
to assume
a position
that
is more
toward
the
right
anterior
oblique,
making

the

palpation
the chest
casual

may

heart

appear

and making
roentgenogram

the

large
image
large

to
on
to

observation.

4. Rales in a child are more likely

to indicate
pneumonia
than
pulmonary edema.
5. The liver need not be palpated
in the standard
textbook
way. I have
found
that palpating
from
below
upward
frequently
causes
guarding
#{149}

vol. 3 no. 10 april

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1982

PIR

321

Heart

Murmurs

Fig 1 .
lntracavitary
phonocardiogram
in root of pulmonary
artery
of normal
First sound
(Si) is shortly
after electrocardiogram
(ECG).
There is a sound-free
by diamond-shaped
high frequency
murmur
which
ends well before
pulmonary
(52).

This

is typical

normal

pulmonary

ejection

murmur

which

was

not

audible

9-year-old
child.
period
followed
second
sound
on surface

of

chest.

Fig 2.
Intracavitary
phonocardiogram
in root of aorta of normal
7-year-old
child.
S is shortly
after ECG.
There
is a sound-free
period
followed
by diamond-shaped
high frequency
murmur
which
ends just before
aortic
second
sound.
5, has been
recorded.
This is a typical
normal
aortic
ejection
murmur
which
was audible
on surface
of chest
at fourth
left interspace
at left
sternal
edge.

PIR

322

pediatrics

in review

#{149}

vol. 3 no. 10 april

and loss of the edge,

etc. For me, it
is better
to feel lightly
from
above
downward,
gradually
extending
the
hand
lower
and lower.
The edge
is
then easy to delineate,
after which
the
standard
measurement
below
the right
costal
margin
is made
at
the nipple
line. One should
also try
to specifically
outline
the liver-frequently
making
a drawing
including
the livers
extent
to the left, especially to the left of the midline.
A liver
that is 5 cm down from the rib margin
at the nipple
line, that is not tender,
and does not extend
to the left may
be normal;
whereas
a 3-cm liver with
a large left lobe extending
well to the
left of the midline
may be quite
abnormal.
The livers
left lobe appears
to be preferentially
enlarged
in congestive
heart failure.
6. The
normal
cardiac
impulse
after infancy
is a left ventricular
impulse.
The physician
should
stand
with the childs
head to his left. The
right
hand
is then
extended
outstretched
from
the sternum
toward
the apex. The normal
left ventricular
impulse
rocks
the hand
toward
the
sternum
from the apex.
The abnormal left ventricular
impulse
is an accentuation
of the normal
and often
causes
retraction
of the skin in systole, inside
the nipple
line. The right
ventricular
impulse
(always
abnormal after
early
infancy)
rocks
the
hand toward
the apex from the sternum.
There
will frequently
be systolic
retraction
of the skin
outside
the apex.
At the same
time,
that
hand is also attempting
to determine
whether
the heart
is of normal
size,
moderately
enlarged,
or greatly
enlarged
to the anterior
axillary
line. It
is also determining
whether
the apex
is hyperdynamic,
as in significant
mitral
valve regurgitation,
for exampie. Another
type of hyperdynamic
impulse
is recognized
by putting
the
outstretched
hand
vertically
up the
left sternal
border.
In children
(better
in skinny
infants
than chubby
teenagers,
but
usually
satisfactory
in
prepuberty)
that impulse
represents
the activity
of the right
ventricular
outflow
tract and pulmonary
artery.
The hyperdynamic
upper
left sternal
edge
gives an excellent
clue to significant
left-to-right
shunts.
7. A thrill
in the chest
indicates
only that there is a grade IV or louder

1982

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CARDIOLOGY

murmur
at that
point.
However,
a
thrill
in systole
at the suprasternal
notch
is virtually
diagnostic
of either
pulmonic
stenosis
or aortic
stenosis.
Large
left-to-right
shunts
may cause
faint thrills
at that point,
and coarctation
of the aorta may cause
a very
active
systolic
impulse
at the suprasternal
notch,
but not usually
a thrill.
A systolic
thrill over the carotids
is
diagnostic
of aortic
stenosis.
8. One must be quite compulsive
in listening
to the heart
sounds
to
train oneself
to listen
to one sound
at a time everywhere.
One listens
to
everything
first in order
to evaluate
the timing
of systole
and diastole.
Then
one should
l;sten
to the first
sound
(Si) everywhere,
the second
sound
(52)
everywhere,
the
third
sound
(Se) everywhere,
and then the
various
clicks
everywhere.
Such
sounds
as fourth
sounds
(54)
and
opening
snaps
are so rarely
heard
in
children
that the pediatrician
would
be better
off concentrating
on the
others.
Finally,
the
pediatrician
should
listen
to systole
alone,
and
then diastole
alone.
This type of auscultation
is called
dissection.
If
the physician
tries
to evaluate
by
listening
to everything
at once, there
is no chance!
S1 is made
up mainly
of mitral
and
tricuspid
valve
dosures,
particularly
the former,
unless
right ventricular
pressure
is high. S
is, therefore,
usually
maximal
at the
apex.
A thin
second
component
heard
after the apical
component
at
the lower
left sternal
border,
particularly
in inspiration,
is a normal
tncuspid
closure
sound
following
the
mitral.
52
is made up mainly
of aontic
and pulmonic
valve closures
and is
normally
best heard at the upper
left
sternal
bonder.
With
inspiration,
there is an increase
in negative
intrathonacic
pressure
so that more blood
enters
the right
side
of the heart.
Right
ventricular
systole
is, therefore,
prolonged
with a delayed
pulmonary
closure
on inspiration.
(Aortic closure
is slightly
early.)
Therefore, the normal
split of S2 on inspiration
is wide,
averaging
40 to 50
msec;
whereas
on expiration,
there
may be no split or a narrower
split.
Although
there
is variation
of the
normal,
any variance
from the above
is a likely
concern.
Without
going
through
all the possible
abnormali

Fig 3.
External
phonocarcitogram
of normal
at left sternal
edge.
Murmur
starts
after 5,
is approximately
two-thirds
systolic.
This
vibratory.
There
was no transmission
to
itary phonocardiogram
at root of aorta was
2). This murmur
on chest
is a normal
murmur
aortic
ejection
murmur.

5-year-old
child recorded
at fourth
left interspace
and stops
well before
52,
so that length
of murmur
is a medium
frequency
murmur,
which
sounded
back and excellent
transmission
to neck.
lntracavin all normal
children
studied
(similar
to that of Fig
and is believed
most likely
to represent
a normal

ties of 52,
important
examples
are:
(a) the more
severe
the pulmonic
stenosis,
the more delayed
(and soften) is pulmonary
closure;
(b) in atnial
septal
defect,
the split is widen than
normal
and
is approximately
the
same
on inspiration
and expiration
( fixed
wide split); (c) a narrow
split
on no split may be present
in significant
aontic stenosis
because
pnolongation
of left
ventricular
systole
causes
delayed
aortic
closure;
(d) a
narrow
split on no split may also be
present
because
of pulmonary
anteny hypertension
(for the high diastolic
pressure
closes
the pulmonic
valve
early;
(e) a single
52, of increased
intensity
and loudest
at the
lower
left sternal
bonder,
is chanactenistic
of tetnalogy
of Fallot,
because
the pulmonary
closure
is not
being
heard
at all and a loud aontic
closure
is being
transmitted
down
the descending
aorta.
It should
be
clean
in evaluating
children
with
heart
murmurs
that there
is nothing
more
important
than analysis
of the

pediatrics

in review

second
sound.
S is a low frequency
sound
which
is heard
best with the
bell,
lightly
applied
to the chest.
A
normal
53 is soft, best heard
at the

apex,

and

not easy

for most

people

to hear.
It is associated
with
rapid
filling
of the left ventricle.
A night
ventricular
S (best
heard
at the
lowen
left sternal
borden)
is always
abnormal
after
early
infancy,
as is
an abnormally
loud apical
S, even
in the absence
of heart murmur.
Clicks
are
extremely
important.
Ejection
clicks
are either
pulmonic
or aontic.
The
pulmonary
ejection
click,
associated
with the hemodynamics
that causes
a large
puImonary artery,
is a sharp
sound
heard

close

to (on almost

on top of) the first

sound,
best heard
at the upper
left
sternal
bonder.
The pulmonary
ejection click
is better
heard
on expination than inspiration
(on only heard
on expiration).
Aortic
ejection
clicks
are less sharp and more clearly
separated
from
S1 than are pulmonary
ejection
clicks.
They are best heard
vol. 3 no. 10 april

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1982

PIR

323

Murmurs

Heart

unl(.Ity

Hospitals

Congenital

of

Cleoeland

Heart

EXAM

Asboistory

J,,it

SHEET
Todays
ECG

DIAGNOSIS

teteresi

History

E.ecse
Dypoea

Tests

Ordered

(IIS

X.Ray

Naoe

Deoelopoet

Echo
Echo

Tole,aoce

ii
20

Hatoiogy

Ciasoss
q-tllg

Theapy010.

dgtahs
Dosage
Symptolts

ot

one
DATE

OF

EXAR1NATION:

P,obleos

Signed:
Esambiaftoe

Physical

_________
___________
neai

Wt

BlOOd

pesscte
a,ms
legs

Ht_
ott

Signed:

(esp.drcgs)

RETURN

L.ps

Rales
Loet
Spleen
Fe,nn,al

_________

Exasiner

Staff

Phosiciso,

DATE:

detotetty

Th,,lI
Rettactoos

Toes

let

ate

Edettta

Chest

Fatgets
Cyanosts
Clubbng
Fleonsss

Resptatoy

TREATMENT

__%

got

ale

(SteCostal)

(daphtagttatc

RETURN
EVALUATION
pIses

OF

TODAYS

DATE

TESTS:
Tests

ccc:

Ordered

on

Motors:

Eci
VCG:
vci

Heest

X-Ray:
Cardac
,npolse N V
normal)
DascplOn
of cardtac
OnpUlSe

N V

aboor,nall

LV

eormal)

LV (aboor,nal)

Dftose-Sep

NV

L
Echo

Morsel
leart

Apex

Hyperdyna,,.ic

Apex

Hypsrdyxasic

Upp.r

Left

Soc

Sternal
Edge tJ

Echo
Echo

SouOdsot

Mac

SpitIng

Respjratory

ID)

(11)
Echo

(20)

ller:ato

logy

(21):

change
MET.
Nesatclogy:

Second

Focrth

Chck

Ejection:
Mon-ejection:
Grade

MUrmUrs

Fig

4.

Examination

Phase

sheet

Ocaltyard
Length

for a child

Macma:
Locate

being

Ttansm:ss,oo

seen

at the lower left sternal

edge and/or
the apex-or
even as far lateral
as
the anterior
axillany
line. There
is no
variation
with respiration.
They
are
associated
with the hemodynamics
that cause
a lange ascending
aorta.
Nonejection
clicks
are midsystolic,
usually
one-third
to one-half
way into
systole.
They may or may not initiate

a late systolic
murmur
and are always abnormal,
indicative
of a prolapsed mitral valve (Fig 5). Nonejection clicks
may be multiple.
Murmurs,
systolic
and diastolic,
should
be compulsively
described
the way the physician
hears
them.
Such
a statement
as, there
is a
systolic
murmur
at the apex
may
be
useless.
Attempts
should
be
made
not only to grade
them
in the
standard
way for intensity
(I to VI for
systolic
murmurs;
I to IV for diastolic
murmurs)
but also to describe
the
length
and the pitch
and to try to
delineate
the relationship
of the murmurs
to each
sound.
For example,
the most common
abnormal
munmur
is that of a small
ventricular
septal

defect.
PIR

324

The

murmur

pediatrics

usually
in review

ob#{149}

in Rainbow

Resp
Change

Babies

SOlItARY

and

Children

AiS

s Hospital

PLAN:

Congenital

scunes
the first sound
and is at the
lower
left sternal
edge.
If the defect
is small,
the munmun
may be quite
diamond-shaped.
In nondiseased
hearts
in the preschool-aged
child,
the most
common
nonmal
murmur
heard
in the chest
is a diamondshaped
ejection
systolic
murmur,
also at the lower
left sternal
border.
The
most
important
differentiating
point is that in ventricular
septal
defect, the murmur
obscures
the first
sound.
Most
systolic
murmurs
ane
best heard
with the diaphragm,
although
the normal
ejection
systolic
murmur
at the lower
left sternal
bonder is often
vibratory
(Fig 3) and the
vibnating
nature
is usually
best appreciated
with
the
bell.
Diastolic
murmurs
may be both high and low
fnequency.
A high
frequency
diastolic
decrescendo
murmur
at the
second
through
fifth
interspace
at
the left sternal
edge, even if it is only
grade
I, is abnonmal.
It is easy
to
hear-with
concentration.
In order
to hear
it, the physician
must
now
say I am now going
to listen
for a
high
frequency
diastolic
decres-

vol. 3 no. 10 april

Heart

Ambulatory

Unit.

cendo
murmur.
Low fnequency
diastolic
murmurs
are more difficult
to
hear, needing
not only concentration
but lots of practice.
The direction
often
given
to students
is to tune
yourself
low.
The standard
teaching is that diastolic
murmurs
are always abnormal,
but one type of diastolic
murmur
can
be normal-a
short (up to grade
II), medium
to high
frequency
diastolic
murmur
at the
lower
left sternal
bonder,
heard
with
the child
lying
down
only
(Fig 6).
This
murmur,
although
not rare,
is
rarely
recognized,
but, of course,
if
it is missed
it does not matter.

Diagnosis

of Normal

Murmur

The diagnosis
of a nonmal
murmur
is based
on certain
positive
descriptions of normal
murmurs,
as well as
no evidence
for specific
cardiac
abnormality.
The
readily
recognized
normal
murmurs
in children
after infancy
are:
1. A systolic
ejection
murmur
at
the lower
left sternal
border
may be

1982

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CARDIOLOGY

Fig 5.
External
phonocardiogram
of 6-year-old
girl
with
mitral
valve
prolapse
and
trivial
mitral
regurgitation.
There
are simultaneous
phonocardiographic
registrations
at left upper
sternal
border
(LUSB)
and apex,
as well as respiration
marker
(inspiration
is higher
on trace)
and
lead 2 electrocardiogram.
Intracavitary
recording
had also been
made
in left atrium
where
it was almost
identical
with that seen at apex.
Note
that there
is a sound-free
space
slightly
more
than halfway
into systole,
where
there
is a sharp
sound.
This sharp
sound
(C) is a nonejection
click,
which
does not vary with respiration.
Following
the click is a late systolic
murmur
which
ends at 52. Aortic
ejection
clicks
also do not vary
with respiration,
but are very close
to first sound.
Pulmonary
ejection
clicks
are louder
on expiration
and are usually
closer
to S.

up to grade Ill in intensity,

is usually
not transmitted
to the back, but is
frequently
very well transmitted
to
the neck. Frequently,
this murmur is
very vibratory
in nature,
is particularly common
in the preschool-aged
child, but can be recognized
at any
age.

We

believe

(but

do

not

have

absolute
evidence)
that this is the
normal aortic ejection
murmur.
2. A systolic
ejection
murmur
at
the upper left sternal border may be
up to grade III in intensity,
is usually
not heard
in the back,
but is frequently
very well transmitted
to the
neck.
This murmur
is particularly
common
in adolescents,
but can be
recognized
at any age. We believe
(but do not have absolute
evidence)
that this is the normal
pulmonary
ejection
murmur.
3. A venous
hum at the upper
right chest may be heard while sitting up but rarely when lying down.
The murmur
is of medium-high
frequency and changes
intensity
as the
neck is moved
right and left. The
murmur
is apparently
due to the
sharp angle made by the subclavian
vein as it enters
the superior
vena
cava.
This angle
is much
greaten
when
sitting
up than
when
lying
down.

4. A normal diastolic
murmur
may
be a grade
I or II short,
diamondshaped early diastolic
murmur heard
at the lower left sternal edge, at the
third
or fourth
intercostal
space,
while lying down. When the patient
sits up, the murmur
usually
disappears, although
rarely it may just be
softer.
5.

Various

high

cardiac

muns disappear.
The diagnosis
is
easily
made,
namely,
by ejection
murmurs
at the upper left and upper
right

sternal

to each

borders,

axilla

which

transmit

and back.

PHYSICAL
EXAMINATION
OF
COMMON
CARDIAC
LESIONS
WITH NONINVASIVE
FINDINGS

output

In order

to understand

these

le-

states may cause heart murmurs

in
patients
with nondiseased
hearts.
Thynotoxicosis
and iron deficiency
are excellent
examples.
Hemolytic
anemias,
such as that associated
with sickle cell disease,
are likely to
cause
even
louder
murmurs
than
iron deficiency
because
not only are

sions and to determine

what is happening,
an understanding
of the box
diagram
of the heart is remarkably
useful.
Such diagrams,
because
of
space limitations,
could not be pant
of this review,
but readers
are urged
to study those representing
common
acyanotic
lesions in the literature.46

the cardiac
longstanding

outputs
nature

Patent

may

extra

in the

cause
outflow

greater,
of the

muscle

tracts.

The

but the
disease

to develop
functional

nature of the murmurs

has the same
explanation
as in the various
left to
night

shunts.

6. Newborns
do not commonly
have normal murmurs
except for the
functional
peripheral
pulmonic
stenosis,
present
because
the angulation
between
the main pulmonary artery and each branch may be

sharp.
With increasing
age and size,
the angle becomes
less and the mur-

pediatrics

in review

A 3:1
S) flow

Ductus

Arteriosus

pulmonary
ratio

(PDA)

to systemic

in PDA

will

(P/

be associ-

ated with three times normal pulmonary artery

and pulmonary
venous
blood flow. The left atrium and left
ventricle

contain

three

times

the non-

mal volume. Three times normal flow

traverses
the
mitral
and
aortic
valves,
causing
functional
murmurs. The findings,
therefore,
are:
1 Increased
pulse pressure.
2. Abnormal
left ventricular
impulse with candiomegaly.

#{149}

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1982

PIR

325

Heart

Murmurs

normal
size,

to small
pulmonary

ascending
aorta
arterial
vascular-

ity.
8.

Echocandiogram

demonstrates
and abnormal

(very

useful)

night volume overload

septal motion.

Ventricular

Septal

Defect

(VSD)

The same thought

process
as for
the other two left-to-night
shunt lesions gives us the following
for a 3:1
P/S

flow

ratio:

Diffuse
and left) with
1

2.

Hypendynamic

nal border,
3. Split
Fig 6.
Intracavitary
phonocardiogram
from body
of left ventricle
of 7-year-old
child
(same
child
as Fig 2). Gain has not been changed.
Ejection
murmur
is much
decreased
in intensity
from that in root ofaorta
and is believed
to be transmitted.
Note that there is a diamond-shaped
medium
to high frequency
murmur
in first part of diastole.
Short
grade
II diastolic
medium-tohigh frequency
murmur
was heard
at the fourth
left interspace,
while lying down
only.
This is
a typical
normal
diastolic
murmur.

3. Hyperdynamic
upper left sternal bonder.
4. Continuous
murmur,
usually
maximal
at the upper left sternal bonden; this murmur
peaks in late systole and the systolic
portion
is often
uneven
in character,
like water
going down the Colorado
River rap

ids.
5.

Soft

systolic

ejection

murmur

(grade
Ill on less) at the upper right
sternal
borden
owing
to increased
flow across a normal aontic valve.
6. Mid-diastolic,
low frequency
rumbling
murmur owing to increased
flow traversing
a normal mitral valve.
7. ECG-left
ventricular
hypertrophy (LVH) usually;
if there is pulmonany hypertension,
night ventnicular hypentrophy
(RVH) is additionally present.
8. Chest roentgenogram-cardiomegaly,
I pulmonary
artery size, ?
increased
ascending
aontic size,
pulmonary
artery
vasculanity
(endon

third

Atrial

vessels

well

seen

of the lung

field).

Septal

Defect

in the

outer

326

pediatrics

pulse
2.

Abnormal

with

right

which

ventricular

im-

cardiomegaly.

Hyperdynamic

nal bonder.
3. Widely

upper

split

does

not

left ster-

second
vary

sound,

with

respira-

tions,
even with the patient
sifting
up.
4. Soft systolic
ejection
murmur
(grade
III on less) at the upper
left
sternal
bonder
owing
to increased
flow

traversing

a normal

pulmonary

valve.
5.

flow

(ASD)

in review

which
mally,

medium-to-low

traversing

normal

tricuspid

valve.
6. ECG-RVH,
with terminal
conduction
delay (Fig 7).
7. Chest roentgenognam-cardiomegaly,
pulmonary
artery

vol. 3 no. 10 april

right

size,

upper

especially
of the

varies
with
but
whose

(night

left sten-

high.
second

sound

respirations
analysis

is

nonex-

tremely
important:
the lower the pulmonary
vascular
resistance,
the
wider the split, mainly because
pulmonany
closure
is delayed;
the
higher

the

pulmonary

vascular

ne-

sistance,
the narrower
the split,
mainly
because
the high diastolic
pressure
is associated
with earlier
closure
of the pulmonic
valve.
An
increased
intensity
pulmonary
dosure sound is most useful if there is
an associated
tamboun.
4. Systolic
murmur
maximal
at
the third,
fourth,
or fifth left interspace,
which
obscures
or partially
obscures
the first sound. The reason
for

the

latter

is that

ulan pressure
curve
the right ventricular
therefore,

the

the

left

rises just
pressure

murmur

of the

ventnic-

before
curve;
left

to

right shunt
across
the ventricular
septum
may begin before the rising
left ventricular
pressure
exceeds
left
atnial pressure
to cause mitral valve
closure.
The murmur
of ventricular
septal defect does not have to be full
length
(holosystolic),
often
being
only
two-thirds
to
three-fourths
length.
The intensity
of the murmur
also varies,
depending
upon
how
much flow is going across
the defect.

Mid-diastolic

frequency
murmur
at the lower left
sternal
bonder
owing
to increased

A 3:1 P/S flow ratio in ASD will be

associated
with three times normal
pulmonary
artery and pulmonary
yenous flow, and night atrium and right
PIR

ventricle
contain three times the nonmal volume.
(Because
the atrial defect is likely to be nonrestrictive
and
because
flow through
the ASD is in
systole
and diastole,
the left atnial
flow does not stop there-causing
no enlargement.)
Three
times
normal flow traverses
the tricuspid
and
pulmonic
valves
so that there are
functional
murmurs
through
the
valves. There is no murmur
of blood
traversing
the atrial
opening.
The
findings,
therefore,
are:

cardiac
impulse
cardiomegaly.

If the

flow

is little,

the

murmur

is soft regardless
of the size of the
defect.
Small defects
are often associated
with soft murmurs
because
the very restrictive
defect
does not
allow much flow; and very lange defects can be associated
with soft
murmurs
because
a high pulmonary
vascular
resistance
has developed
and does not allow much flow. If the

1982

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CARDIOLOGY

flow

is great,

regardless

of the defect,
in congestive

of the size

and if the patient

is not
heart failure,
the mun-

3.

Systolic

mur will be at least grade IV. In general, within the context

of the high

on

flow,

the

imal

at the

more

plateau-shaped

murmur

This

click

the

de-

first

sound

the

having
upper

will

be;

larger

the

the

more

opening,

the

the

restrictive

fect, the more diamond-shaped

murmur
will be.
5.

Soft

systolic

ejection

murmur

(grade
Ill on less) at the upper left
sternal
bonder
owing
to increased
flow traversing
the normal pulmonic
valve. This murmur
may be difficult
to

recognize

murmur
across

because

the

of flow going
the ventricular

transmit

so widely

louder

left to night
septum
may

as to obscure

it.

6. Mid-diastolic
low
frequency
rumbling
murmur
at the apex owing
to increased
flow traversing
a normal
mitral valve.
7.

ECG-normal

in small

defects,

LVH with larger flow and frequently

additional
RVH.
If the pulmonary
blood flow is large, then the left yentnicular volume
is also large. In addition, the left ventricle
must propel
the blood through
the VSD. Some of
the VSD flow presumably
goes right
through
into the pulmonary
artery,
aithough

the

night

ventricle

must

do

some
extra
volume
work
as well.
Consequently,
in terms
of volume
work,
the left ventricle
contributes
more

than

However,
higher

and

does

the
the

the

right

more

the

larger

right

ventricle.

the defect,

the

ventricular

pressure,

the

will

ECG

show

RVH.

8. Chest noentgenognam-cardiomegaly,
including
increased
left
atnial size,
pulmonary
artery
size,
normal
ascending
aorta,
an occasional right descending
aorta, I pulmonary arterial vasculanity.

Valvular

Pulmonic

Stenosis

(PS)

Valvular
pulmonic
stenosis
is almost invariably
valvular
as infundibulan pulmonic
stenosis
is almost always associated
with a VSD.
1. Abnormal
right ventricular
impulse with no or little cardiomegaly.
2. Normally
active
left
sternal
border,
but occasionally
hyperdynamic high up.

thrill

nal notch.
4. Pulmonic

ejection

expiration

versing

at the

(as

better

blood

is tra-

in expiration),

upper

may

click,

less

the valve

left

pulse

suprasten-

max-

sternal

be night on top of the

so that

it is recognized

a loud
first
left sternal
border

by
at the

(not

likely)

that is louder
on expiration
than inspiration.
The
apparent
loud
first
sound
is actually
a pulmonary
ejection click.
5. Split
of the second
sound
is
slightly
wide with mild stenosis,
associated
with a normal (to increased

intensity)
pulmonary
closure
sound.
As severity
increases,
the split of the
second sound widens and the intensity

of

pulmonary

closure

dimin-

ishes. In more severe

stenosis,
the
decreased
intensity
second sound is
due to the murmur
obscuring
the
aortic
valve
closed.

6.
left

closure
and
the
being
too distorted

pulmonary
to snap

Ejection
murmur
at the
sternal
bonder (associated

a systolic

grade

thrill

if the

murmur

is short

severe

the

cleanly

and

stenosis,

murmur
and
if the murmur

upper
with

murmur

IV). If the stenosis

is loud-

is mild,

soft.

The

the

longer

the
more

the

the later the peak;

but
cleanly
stops
before
a

audible

aortic

closure

arated
ing

border.

sound,

with

no on little

2. Suprasternal
thrill plus a carotid
3. Aortic
ejection

from

with

candiomegaly.

notch
systolic
click,

systolic
thrill.
well sep-

the first sound,

not vary-

respirations,

best

heard

at

the lower left sternal

border,
apex,
and/or
left axilla.
4. Intensity
of aortic
closure
is
normal
no matter what the severity
(although
it can actually
even be
somewhat
increased).
With increasing severity
and prolongation
of left
ventricular
systole,
aortic
closure
becomes
closer to pulmonic
closure,
narrowing
the split and even becoming

inseparable

from

pulmonic

do-

sure (no split).

On rare occasions,
there is so much prolongation
of aortic closure that aortic closure
occurs
after
pulmonary
closure.
The result
is a single second
sound on inspiration, and a split on expiration
(paradoxical
split). If the second
sound
is normally

split,

the stenosis

is mild.

5. Ejection
systolic
murmur
usually at the upper night sternal border
associated
with a systolic
thrill if the
murmur
is loud (grade
IV). The location of the maximal
intensity
of the
murmur
can
vary
greatly,
even
sometimes
being over the sternum
or the upper
left sternal
border. (Valvar aortic stenosis
can be maximal
at the lower
left sternal
border
in
infancy,

but

later

in

childhood,

on minimal
cardiomegaly
with
a
prominent
pulmonary
artery.
The
proximal
branches
may
also
be
large,
but the outer third of the chest

maximal
murmur
in that area usually
indicates
one of the types of subvalvar aortic stenosis.)
For the stenosis
to be significant,
the murmur
must
be nearly full length.
6. ECG-LVH.
(ST and T abnormality indicates
potentially
grave Severity,
even if the child is asymptomatic.)
In mild cases,
the ECG is
normal.
(Occasionally,
severe aortic
stenosis
is associated
with a normal
standard
ECG, but the vectorcardiognam will usually
be abnormal.)
7. Roentgenogram-normal
size
or minimal
cardiomegaly
with
a
prominent
ascending
aorta.

roentgenogram
will not show
arteries,
so that pulmonary
vascularity
is normal.

Coarctation

the right ventricular

pressure
is expected
to be significantly
less than

systemic
level. Roughly,
when the
murmur
extends
to aortic
closure,
the right ventricular
pressure
is likely
to be in the neighborhood
of systemic

pressure,

extends

and

past aortic

ventricular

pressure

if the

murmur

closure,

the night

is likely

to

be

suprasystemic.
7.

ECG-RVH.

8.

Roentgenogram-normal

Aortic

size

end-on
arterial

Stenosis

Valvar stenosis
is the most
mon form of aortic stenosis.
1. Abnormal
left ventricular
pediatrics

com-

Coarctation
of the aorta is almost
always in descending
arch near ongin of left subclavian
and ligamenturn arteriosus.
1.

im-

In review

of Aorta

pulses

Diminished

in relation

or absent

fernoral

to the right

brachial

#{149}
vol. 3 no. 10 aprIl

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1982

PIR

327

Heart

Murmurs

artery pulse. The left bnachial

pulse
may be the same as the right, but it
is often diminished
(for reasons
not
always clear), on occasion
even diminished
to the level of the femonal
artery
pulse.
The latter
may
be
caused
by the left subclavian
artery
arising
below
the coarctation,
or it
may be that the left subclavian
artery
is hypoplastic
at its origin
at the
coarctation.
Therefore,
as part of the
evaluation,
if only one arm blood
pressure
is measured
along with one
leg pressure,
it should
be the night
arm.
2. Abnormal
left ventricular
pulse with no or little cardiomegaly.

im-

3. Active
pulse, rarely

im-

supnasternal
with a thrill.

4. Aortic
ejection
present
unless
there

despite

severe

notch

click
is not
is associated

disease

and

is

rarely more than grade III. It is ejection in type and starts clearly
after
the first sound. Although
the murmur
can be maximally
heard anteriorly,
anywhere

edge,

along

it may

even

teriorly,
back,

the

left

on near the apex

with
maximal

be

virtually
over

sternal

absent

the

loud

an-

in the

left scapula

or slightly
lower. Occasionally,
particularly
near the spine, the murmur
extends
into diastole
due to continuous flow through
the coarctation
(not due to collateral
vessels).
7. ECG-LVH
(although
the
younger
the child,
the greaten
the
possibility
that RVH may be present). In infants,
RVH is usually
present. The latter is believed
to be a
remnant
of previous
fetal hemodynamics
which
occurred
when
the
night ventricle
may have been sending blood
through
the
the descending
aorta.

coarctation
to
In fetal life, it

can be hypothesized
thathypenpIasia of the ventricular
muscle may be
a response
to increased
workload
as well as hypertrophy,
so that RVH
remains
long after birth.
8. Roentgenogram-nonmal
size
or minimal
cardiomegaly.
The ascending
aorta is normal unless there
PIR

328

pediatrics

in review

past

#{149}

the

stenotic
and
plus the notch
Mitral

arch,

so that

poststenotic
make a 3

the

pne-

dilation

sign.

Regurgitation

Mitral

regurgitation

can

be

con-

genital
or due to rheumatic
heart
disease.
1 Abnormal
left ventricular
impulse with little on no to great candiomegaly
depending
upon the severity of the lesion.
.

Hyperdynamic

apical

impulse

varying
with severity
as described
above.
3. First sound
varies from being
loud if the valve is freely mobile,
to
being very soft if the valve is very
distorted.
4. Normally
on even
slightly
widely
split second
sound
is expected
because
the left atrium is so
compliant

that

it tends

to accept

the

large volume
load without
much increase
in left atrial and thus pulmonary venous
pressure.
However,
the
recognition
that the split is narrow
with
increased
intensity
pulmonary

on left axilla,

the murmur

just

2.

abnormality
of the aortic valve. Bicuspid
aortic valves are very commonly associated.
5. Increased
intensity
of aortic
closure.
6. Murmur
of the coarctation
is
variable.
It may be very soft (grade
II)

is an additional
aontic valve problem.
Occasionally,
an indentation
may be
recognized
in the descending
aorta

closure
is a potentially
grave prognostic sign. Presumably
a high pulmonary venous pressure
with resulting high pulmonary
artery diastolic
pressure
is the cause.
5. Systolic
murmur can be as soft
as grade I or as loud as grade IV.
The frequency
is usually
high. The
murmur

is

characteristically

de-

scnibed
as holosystolic
(throughout
systole)
but this is not necessarily
a
good term, for the murmur
usually
does not obscure
the first sound.
If
the first sound is very soft, however,
obscuring
of that sound by the murmur may be difficult
to distinguish.
Unlike ventricular
septal defect with
left to night shunt,
in which
early
systole
is very important
in making
the diagnosis,
late systole
is more
important.
The murmur
should
be
prominent
in late systole
and may
even peak at that time presumably
because
function.

of

papillary

muscle

dys-

6. If the mitral
regurgitation
is
considerable,
then there must be a
large
forward
flow
(no diastole)
across
the valve.
Consequently,
a

functional
flow through
valve
results.
diastolic
and

murmur
of increased
the nonstenotic
mitral
This
murmur
is middoes
not go into late

diastole
(as would
mitral stenosis).

be expected

in

7. ECG-LVH
(normal,
8. Roentgenognam-varying
diomegaly
depending

if mild).
canupon
the

amount
of regurgitation.
atrium is lange. Pulmonary

The left
vascular-

ity

may

be

cases,
nary

but

increased

this

venous

Mitral

in

increase

severe

is pulmo-

in origin.

Valve

Prolapse3

1 Questionably
abnormal
left
ventricular
impulse.
No candiomegaly.
2. Nonejection
mid-systolic
click
at the apex. The timing is one third
.

to

halfway

into

systole.

Sometimes

clicks

are

multiple

and

the

times they are

to miss.
More

some-

very loud, impossible

often,
they
are very

soft and easy to miss. The clicks

intensity
is not different
lying down
or sitting
up, but it may be earlier
when
the child
is sitting
murmur
is softer
while

the click
in that

3.

may be easier

up.
lying

to recognize

position.

Late

systolic

ning with the

tending
to the

murmur

begin-

midsystolic
click,
exsecond
sound
(Fig 5).

The character
of this
tnemely variable
from

murmur
patient

tient,
tient.

the
and

as well
It may

quency,

like

as within
be soft

classic

the murmur

tiated
sitting

pafre-

regungiharsh
on
virtually
thing is

is late systolic,

by the click
and louder
up than
lying
down.

sionally,
absent

is exto pa-

same
high

mitral

tation,
but it may also be
groaning,
on whooping-on
anything
else. The important

that

As the
down,

miwhen
Occa-

the murmur
may even be
lying down, and be grade I,

II, on Ill while

sitting

up. On occasion,

the murmur
while
lying

may
down

be late systolic
and holosystolic

when

up.

sitting

4. ECG-vaniable.
May
mal (and abnormal
a few
later).
Prominent
abnormally

be norminutes
ante-

nor and
mon as
sionally,

superior
T waves
are comare large
U waves.
Occathe T wave may even be to

the right
gitation.

despite
(These

trivial mitral regurT and U wave ab-

vol. 3 no. 10 aprIl 1982

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CARDIOLOGY

_S:i.i4
*0
-

II

III

AVI

AVL

V4

vs

AVF

.U-ST1

_________
*S

it
Y4R

vi

V2

Fig 7.
Typical
standard
ECO child with atrial Septa/defeCt.
vector
is slightly
abnormally
to right,
but almost
anterior.
rSR in both right chest
leads.
Terminal
vector
was also

normalities
have suggested
papillary
muscle
dysfunction
as being
etiologic in some patients.)
5. Echocandiognam-Pnolapse
of
the posterior
mitral valve leaflet
posteniorly
is characteristic;
however,
this
echocandiognaphic
finding
is
commonly
ovennead
so that the diagnosis
of prolapsed
mitral
valve
should
be
made
mainly
utilizing
physical
examination.
If the auscultatony
examination
is negative,
the
diagnosis
should
generally
not be
made.

EPILOGUE

There
is moderate
Consequently,
vector
shown
to be somewhat

right ventricular
hypertrophy.
crosses
line perpendicular
slowly
inscribed.

oscope
to be heard.
In carrying
on
the analogue
of the water
pipe in the
basement,
the auscultor
is now at
the other
end of the basement.
Furthermone,
parents
love
the
word
normal.
The term innocent
murmur
is satisfactony,
but it is not very specific.
For example,
the munmur
of mild
valvan
pulmonic
stenosis
is surely
innocent
for life expectancy
is believed
to be normal,
but the patient
is definitely
not normal
and many
physicians
believe
that infective
endocarditis
pnophylaxis
should
be
given
Particularly
objectionable,
however,
is the term functional
murmun. For example,
the two murmurs
associated
with atnial septal
defect
are both functional
murmurs,
a function of increased
flow through
normal valves.
They are not normal.
.

Note that the term normal

murmur
is considered
much
more
suitable
than
the terms
functional
murmur
and innocent
murmur.1#{176}Intracardiac
phonocardiographic
studies
have
demonstrated
that all people
have
these
normal
murmurs;
and when
the
normal
murmur
is no longer
heard
on the chest
surface
in adolescence
and adulthood,
the munmun
has not disappeared.
The murmur
is
merely
too fan away from the steth-

REFERENCES
1.

Physiologic
and Murmurs,

Principles

of

Heart

Sounds

American
Heart Association Monograph
No. 46. New York,
American
Heart Association,
1975

pediatrics

in review

V6

Initial
to V4R

QRS is normal
and terminal
and Vi lead axes.
Result
is

2. Liebman J, Sood S: Diastolic

apparently
normal
children.
38:755, 1968
3.

Sreenivasan

VV,

Liebman

murmurs

in

Circulation
J:

Posterior

mitral regurgitation
in girls possibly
due
to posterior
papillary
muscle
dysfunction. Pediatrics
42:276, 1968
4. Nadas AS, Fyler DC: Pediatric
Cardiology.
Philadelphia,
WB Saunders
do,
1972
5. Rudolph AM: Congenital
Diseases
of the
Heart.
Chicago,
Year Book Medical Pubushers,
nc, 1 974
6. Liebman
J, Borkat G, Hirschfeld
S: The
heart,
in Klaus
MH, Fanarotf
AA (eds):
Care

of

The

High-Risk

Neonate.

Phila-

deiphia,
WB Saunders,
do, 1979,
pp
294-323
7. Ongley PA, Sprague
HB, Rappaport
MB,
et al: Heart Sounds
and Murmurs:
A Clinical

and

Phonocardiographic

Study.

New

York, Grune & Stratton,

1960
8. Liebman
J, Plonsey
R: Electrocardiography, in Moss AJ, Adams FH, Emmanovilides
Gd (eds): Heart Disease
in Infants,
Children
and Adolescents,
ed 2.
Baltimore,
Williams
& Wilkins,
1977, pp
18-61
9. Liebman
J, Plonsey
R: Basic principles
for understanding
electrocardiography.
Paediatrician
2:251, 1973
10. daceres
CA, Perry LW: The Innocent
Murmur.
Boston,
Little, Brown and do,
1967

vol. 3 no. 10 april

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1982

PIR

329

Diagnosis and Management of Heart Murmurs in Children

Jerome Liebman
Pediatrics in Review 1982;3;321
DOI: 10.1542/pir.3-10-321

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