Approach to A Child With A Heart Murmur
Banani Poddar and Srikanta Basu
Department of Pediatrics, Govt. Medical College & Hospital, Chandigarh, India.
Abstract. The vast majority of children with heart murmurs have an 'innocent' murmur. Differentiation of such murmurs from
those due to structural cardiac disease, so called 'pathological' murmurs, is largely clinical. Pediatricians are capable of
differentiating one from the other, provided a detailed evaluation is done. This article outlines the salient features of innocent
murmurs that help us recognize them clinically. [Indian J Pediatr 2004; 71 (1) : 63-66] E-mail : bananip@hotmail.com
Key words : Murmurs; Cardiac; Diagnosis
Pediatricians often encounter children with heart
murmurs, either as a part of 'well-baby' examination or
d u r i n g a s s e s s m e n t of i n t e r c u r r e n t illnesses. 1,2,3 The
d i l e m m a faced at such a time is to decide which child
r e q u i r e s f u r t h e r e v a l u a t i o n a n d w h i c h child has an
'innocent' murmur. In this article, clinical differentiation
b e t w e e n p a t h o l o g i c a n d innocent m u r m u r s has been
outlined so that parents of children with the latter can be
re-assured and saved from further investigations and / o r
referral.
What is a Murmur?
A u d i b l e s o u n d w a v e s in the r a n g e of 20-2000 H z
emanating from the heart and vascular system constitute
a cardiac murmur. 1 In simpler terms, it can be said to be
the a u d i t o r y (auscultatory) consequence of turbulent
blood flow from within the cardiovascular system.
W h y are Murmurs Important?
A large n u m b e r of children (including neonates) are
detected to have a cardiac m u r m u r ; especially prior to
school age2 Some m u r m u r s in neonates, m a n y in infants
a n d m o s t in c h i l d h o o d are ' b e n i g n ' or ' i n n o c e n t ' . 1-5
H o w e v e r , a cardiac m u r m u r m a y be the first sign of a
serious s t r u c t u r a l cardiac disease, e s p e c i a l l y in the
neonate. 6 The latter not only carry a high morbidity (and
if untreated, mortality) but also have enormous financial
and psychological implications for the child and parents.
Hence differentiation of one from the other is mandatory.
W H A T ARE I N N O C E N T MURMURS?
Those m u r m u r s that occur in the absence of structural
cardiac disease are said to be 'innocent'. They have been
variously described as functional, benign, innocuous or
physiologic murmurs.
Reprint requests : Dr. Banani Poddar, Associate Professor, Dept of
Critical Care Medicine, SGPGIMS,Lucknow - 226014.
Indian Journal of Pediatrics, Volume 7 1 ~ a n u a r y , 2004
Innocent Murmurs of Childhood
Systolic m u r m u r s
Vibratory Still's murmur
Pulmonary flow murmur
Peripheral pulmonary arterial stenosis murmur
Supraclavicular or brachiocephalic systolic murmur
Aortic systolic murmur
Continuous m u r m u r s
Venous hum
Mammary arterial souffl6
The Vibratory Still's Murmur
This is the most common innocent m u r m u r in children.
First described by George F. Still in 1909, 7 it presents most
often between the ages of 2 and 6 years, though it m a y be
present at extremes of age (adolescence and infancy). It is
an early systolic, grade 1 to 3 (usually grade 2), low to
m e d i u m pitched m u r m u r , best heard at the lower left
sternal edge and extending to the apex and loudest in the
supine position. It changes on sitting or standing. It has a
distinctive vibratory quality or a twanging sound, which
gives it a musical character.
The origin of the m u r m u r is not clear. This has been
attributed to various causes including vibration of the
p u l m o n a r y valves during systolic ejection, physiologic
n a r r o w i n g of the left v e n t r i c u l a r o u t f l o w tract 8 and
presence of ventricular false tendons?
The Pulmonary Flow Murmur
This m a y be heard in children, adolescents and y o u n g
adults. It is an ejection systolic m u r m u r , crescendo -
decrescendo in character, of low intensity (Grade 2-3) and
is h e a r d at the left s t e r n a l b o r d e r in the 2 nd a n d 3 rd
intercostal spaces. It is r o u g h in character and is thus
distinct from the musical Still's murmur. Best heard in the
s u p i n e p o s i t i o n , it is also e x a g g e r a t e d b y a p e c t u s
excavatum, a straight back or kyphoscoliosis that results
in compression or brings the right ventricular outflow
tract closer to the chest wall. The m u r m u r increases in
63
 Banani Poddar and Srikanta Basu
intensity in expiration while inspiration and u p r i g h t
position decrease it.
The innocent p u l m o n a r y flow m u r m u r s h o u l d be
distinguished from murmurs due to increased flow in an
atrial septal defect and also from p u l m o n a r y valvar
stenosis. The p r e s e n c e of a h y p e r d y n a m i c r i g h t
ventricular impulse; wide, fixed splitting of the $2 and a
mid-diastolic flow m u r m u r help in the distinction of the
former, while in the latter, the presence of a systolic thrill,
a longer and more harsh m u r m u r and an ejection click
would aid in the diagnosis.
Peripheral Pulmonary Arterial Stenosis Murmur
This is commonly heard in infants and neonates. It is of
ejection systolic character, grade I to 2, low pitched and
extends till or just beyond $2. These m u r m u r s become
m o r e p r o m i n e n t w i t h viral u p p e r r e s p i r a t o r y tract
infections, especially in the recovery phase, and reactive
airway disease. They are often best heard in the axillae or
on the back.
The r e l a t i v e d i s p a r i t y in size b e t w e e n the m a i n
pulmonary artery trunk and its small branches at birth is
thought to be the cause for this murmur. Further, the
branches of the pulmonary artery arise at sharp angles,
again resulting in turbulence. In older infants with
respiratory tract infections, regional vascular reactivity
and redistribution of blood flow may cause reappearance
of the murmur. Differentiation from the m u r m u r due to
an a n a t o m i c n a r r o w i n g of the p u l m o n a r y arterial
branches is by the longer duration of the latter, the higher
pitch and the older age group in whom the latter is heard.
The Supraclavicular/Brachiocephalic Systolic Murmur
This murmur, heard in children and y o u n g adults is a
crescendo - decrescendo early systolic murmur best heard
above the clavicles with radiation to the neck. It is low to
medium pitched and brief. The murmur does not change
with supine or sitting position b u t d i m i n i s h e s with
h y p e r e x t e n s i o n of the s h o u l d e r s . TM This m u r m u r is
thought to originate from the brachiocephalic vessels as
they arise from the aorta.
The Aortic Systolic Murmur
This murmur is an innocent systolic flow murmur arising
from the left ventricular outflow tract and associated with
i n c r e a s e d s y s t e m i c cardiac o u t p u t . It is ejection in
character, systolic and is best heard in the aortic area.
Common situations in children when these murmurs are
heard include fever, anemia, anxiety, hyperthyroidism
etc. Similar murmurs may be heard in trained athletes
with slower heart rates and greater stroke volume.
The m a i n d i f f e r e n t i a t i o n is f r o m h y p e r t r o p h i c
obstructive cardiomyopathy (HOCM) and left ventricular
outflow tract obstruction. In HOCM, increased venous
return (as in rapid squatting) diminishes the murmur,
while the Valsalva maneuver causes it to be louder. Also,
64
the presence of a positive family history should alert the
clinician in favour of HOCM.
The Venous Hum
This is the most common continuous murmur in children.
Initially described by Potain in 1867,11it is most audible in
the neck, anteriorly, just lateral to the sternocleidomastoid
muscle often extending to the infraclavicular region of the
chest wall. It is usually louder on the right side, better
heard sitting than lying and is best elicited with the
patient sitting up and looking away from the side of
examination. It is w i d e l y variable in character a n d
intensity, from faint to grade 6. Gentle compression over
the jugular vein or turning the head towards the side of
the murmur diminishes the murmur.
Turbulence at the confluence of flow from the internal
jugular and subclavian veins as they enter the superior
vena cava, or angulation of the internal jugular vein as it
courses over the transverse process of the atlas, is thought
to cause this venous hum&
The Mammary Artery Souffl6
This m u r m u r , well recognised in late p r e g n a n c y and
lactation, can rarely occur in adolescence. This starts in
systole but may extend into diastole and is heard on the
anterior chest wall over the breast. It is high pitched, has
a superficial c h a r a c t e r and firm p r e s s u r e w i t h the
s t e t h o s c o p e m a y s o m e t i m e s abolish the m u r m u r .
T h o u g h t to be arterial in origin, it occurs due to the
enlarged vessels of the chest wall. Differentiation from a
murmur due to patent ductus arteriosus or arterio-venous
fistula is essential.
APPROACH TO A CHILD WITH A MURMUR
Having thus described the various innocent murmurs in
childhood, let us now examine the approach to a child
with a heart murmur.
History
The history should elicit the presence or absence of the
cardinal cardiac symptoms. The symptoms of congestive
heart failure in infants are very subtle and may be missed,
if not specifically asked for. Past h i s t o r y to suggest
rheumatic fever must be excluded. Family history of
hypertrophic cardiomyopathy, congenital heart disease or
u n e x p l a i n e d c h i l d h o o d / e a r l y a d u l t h o o d death is of
importance. A perinatal history of p r e m a t u r e birth,
m a t e r n a l diabetes, d r u g or toxin i n g e s t i o n a n d
intrauterine infection is relevant.
Examination
Apart from examination of the cardiovascular system,
perhaps, the most important assessment is the evaluation
for dysmorphism and the presence of other congenital
anomalies. Presence of anomalies of other organ systems
Indian Journal of Pediatrics, Volume 71~anuary, 2004
 Approach to a Child with A Heart Murmur
is associated with congenital heart disease in as m a n y as
25% of cases. The assessment of the child's growth and
development is vital and m a y inform us about "failure to
thive'. The child's play capacity & / o r ability to exercise
s h o u l d b e s o u g h t for. A s y s t e m a t i c c a r d i o v a s c u l a r
examination including assessment of the arterial pulses
a n d p e r f u s i o n , m e a s u r e m e n t of b l o o d p r e s s u r e , the
systemic venous assessment, precordial inspection,
palpation and auscultation in the four different areas, are
done. Step-by-step auscultation firstly for heart sounds
a n d s u b s e q u e n t l y for m u r m u r s , a n d for a d d i t i o n a l
sounds, as also clicks is required. A crucial auscultatory
assessment in children is to characterise the second heart
sound and its components. Auscultation should also be
c a r r i e d out o v e r the back, the axillae a n d the neck.
Variations of m u r m u r s and heart sounds with position
(supine, sitting, s t a n d i n g ) a n d v a r i o u s m a n o u v r e s
(respiration, Valsalva, exercise) further characterise the
nature of these.
Investigations
The chest X-ray (CXR), electrocardiogram (ECG) and
e c h o c a r d i o g r a m are invaluable tools to diagnose and
assess the severity of a cardiac disease. However, they
add little or no information in a child clinically assessed to
have an 'innocent murmur'. Hence, these investigations
are not routinely recommended. The pitfalls associated
with such investigations have been dealt with later.
IS IT POSSIBLE TO DIAGNOSE AN ' I N N O C E N T
M U R M U R ' CLINICALLY?
The d i a g n o s i s of an i n n o c e n t m u r m u r r e q u i r e s the
absence of s y m p t o m s referable to the c a r d i o v a s c u l a r
system, the absence of d y s m o r p h i s m or characteristic
c a r d i o v a s c u l a r signs a n d the r e c o g n i t i o n of the
characteristic features of'innocent' murmurs. 3To aid us to
this end, several 'guidelines' have been published.
We are aware of the 'Nadas' criteria' for the diagnosis
of congenital heart disease/3 In addition, several studies
have a d d r e s s e d this issue as to whether clinicians can
accurately classify m u r m u r s as 'innocent' or 'pathologic'
and several characteristic attributes of each m u r m u r were
found. 1~-17In a study of 222 children being evaluated for a
heart m u r m u r b y pediatric cardiologists, the following
clinical signs w e r e identified, TM the presence of any of
which negates a diagnosis of an 'innocent murmur'. These
include:
> Pansystolic m u r m u r
> M u r m u r intensity > grade 3.
> Maximal intensity of m u r m u r at left u p p e r sternal
border.
> Harsh quality of a murmur.
> Presence of an eafly/midsystolic click.
> Presence of an abnormal $2.
In another such study, 15 the characteristics of pathologic
Indian Journal of Pediatrics, Volume 71--January, 2004
m u r m u r s were found to be
> A systolic m u r m u r with intensity of a grade 3
> A diastolic m u r m u r
> An increase in m u r m u r intensity with the patient
standing.
Hence, the Nadas' criteria, in addition to these above
attributes of m u r m u r s , clearly define ' r e d flags' in the
diagnosis of innocent murmurs.
The sensitivity a n d specificity of differentiating a
pathologic m u r m u r was 92% and 94% respectively, in the
above study, with physical examination alone. 14 Similar
results with high sensitivity and specificity have been
shown in other studies. 17-19Interestingly, the pathologic
m u r m u r s , which were wrongly classified as 'innocent',
were due to lesions that were trivial or insignificant.
In a s t u d y c o n d u c t e d on the a c c u r a c y of clinical
a s s e s s m e n t of h e a r t m u r m u r s b y office- b a s e d
pediatricians, z~ the sensitivity was 82% and specificity
72% and this was considered suboptimal. However, in
this s t u d y , e x a m i n a t i o n w a s c o n f i n e d to c a r d i a c
auscultation alone. In another study comparing academic
pediatricians with pediatric cardiologists/6 it was found
that the accuracy of clinical diagnosis of heart m u r m u r s
w a s g o o d a m o n g b o t h a n d e i t h e r w a s u n l i k e l y to
misclassify a pathologic m u r m u r as 'innocent'. Hence, a
diagnosis of an innocent m u r m u r should be clinical and
not one of exclusion.
Pediatricians can easily take a history, examine the
pulses, and measure the blood pressure and have been
shown to be as good as pediatric cardiologists in correctly
classifying a m u r m u r , provided a detailed evaluation is
done. 3 Also, it is possible to identify a pathologic m u r m u r
clinically with a reasonable degree of accuracy, provided
the 'red flags' are looked for carefully.
PITFALLS OF D I A G N O S T I C TESTS IN THE
EVALUATION OF A C A R D I A C M U R M U R
While an enlarged t h y m u s on a CXR causes confusion
about heart size in the infant, interpretation of an ECG
requires a t h o r o u g h k n o w l e d g e of n o r m a l age-related
changes in infancy and childhood.
In a study of asymptomatic infants and children with a
cardiac murmur, 21a low reproducibility and accuracy was
found of radiologic assessment of chest radiography by
pediatric radiologists with respect to presence or absence
of heart disease. Similarly, in a study on 120 infants and
children being evaluated for a heart m u r m u r , z2 the ECG
was found to be of no help in discrimination b e t w e e n
h e a r t d i s e a s e a n d no h e a r t d i s e a s e . In this s t u d y
mentioned above, 16 revision of the diagnosis after ECG
a n d CXR w a s m o r e o f t e n m i s l e a d i n g t h a n h e l p f u l .
H o w e v e r , t h e s e m o d a l i t i e s c e r t a i n l y e n h a n c e the
diagnostic accuracy when heart lesions are present. This
has been well d e m o n s t r a t e d in a s t u d y c o n d u c t e d b y
Danford et a123where 149 patients < 21 years age were
evaluated for a heart murmur. While the ECG enhanced
65
 Banani Poddar and Srikanta Basu
d e t e c t i o n of a n atrial s e p t a l defect a n d to a certain extent
p u l m o n a r y s t e n o s i s , t h e X - r a y e n h a n c e d d e t e c t i o n of a
h e m o d y n a m i c a l l y s i g n i f i c a n t v e n t r i c u l a r s e p t a l defect.
Hence, there are limitations of diagnostic utility of routine
CXR a n d ECG, e s p e c i a l l y w h e r e the clinical d i a g n o s i s is
that of an ' i n n o c e n t m u r m u r ' . TM
T h e g o l d s t a n d a r d for t h e d i a g n o s i s of a s t r u c t u r a l
c a r d i a c d i s e a s e is a n e c h o c a r d i o g r a p h i c e v a l u a t i o n .
H o w e v e r , t h e r e c a n b e p r o b l e m s w h e n p e r f o r m e d in
l a b o r a t o r i e s w i t h o u t expertise in p e d i a t r i c h e a r t disease. 1
N o r m a l p h y s i o l o g i c a l v a r i a t i o n s s u c h as p r e s e n c e of a
p a t e n t f o r a m e n o v a l e o r P D A in a n e o n a t e m a y b e
m i s i n t e r p r e t e d as p r e s e n c e of h e a r t disease. G e v a et al TM
have amply demonstrated that a routine echocardio-
g r a p h y is n o t r e q u i r e d in all cases w i t h a heart m u r m u r .
M o r e o v e r , these b a b i e s often r e q u i r e conscious s e d a t i o n
for the p r o c e d u r e , w h i c h m a y l e a d to p r o b l e m s .
Even in t o d a y ' s hi-tech w o r l d , the best w a y to correctly
c l a s s i f y a m u r m u r as ' i n n o c e n t ' or ' p a t h o l o g i c a l ' is b y
clinical evaluation with special emphasis on a careful
a u s c u l t a t i o n . D a h l et al h a v e r e c e n t l y d e s c r i b e d a
t e c h n i q u e f o r s e n d i n g a r e c o r d i n g of h e a r t s o u n d s
(electronic s t e t h o s c o p e ) v i a e - m a i l to a specialist, w i t h a
h i g h sensitivity (89.7%) a n d specificity (98.2%) of correctly
c l a s s i f y i n g the m u r m u r . 24 S i m i l a r l y , u s i n g a n electronic
stethoscope, h e a r t s o u n d s w e r e r e c o r d e d a n d fed into an
artificial n e u r a l n e t w o r k ( A N N ) after p r o c e s s i n g . 25 The
accuracy of classification into 'innocent' and
' p a t h o l o g i c a l ' w a s 100% u s i n g this A N N . In the absence
of such s o p h i s t i c a t i o n a v a i l a b l e r o u t i n e l y , p e d i a t r i c i a n s
c a n p l a y a p i v o t a l r o l e in c o r r e c t l y c l a s s i f y i n g a h e a r t
m u r m u r as ' i n n o c e n t ' or ' p a t h o l o g i c a l ' . T h u s all f u r t h e r
i n v e s t i g a t i o n s a n d / o r referral in children w i t h ' i n n o c e n t '
m u r m u r s m a y b e a v o i d e d a n d the c h i l d k e p t o n follow-
up. I n c a s e of a c l i n i c a l i m p r e s s i o n of a ' p a t h o l o g i c a l '
m u r m u r or the p r e s e n c e of the ' r e d flags' as h i g h l i g h t e d
above, the c h i l d m a y b e r e f e r r e d for f u r t h e r e v a l u a t i o n .
T h e u r g e n c y w i t h w h i c h s u c h a n e v a l u a t i o n is d o n e
d e p e n d s on t h e a g e of t h e p a t i e n t . It is u s u a l l y w i t h i n
h o u r s in neonates, d a y s in infants a n d w e e k s in children. 1
Hence, to c o n c l u d e the o n l y w a y to m a k e a d i a g n o s i s
of a n i n n o c e n t m u r m u r is b y a u s c u l t a t i o n a n d r o u t i n e
echocardiography is n o t i n d i c a t e d . T h e d e c i s i o n
r e g a r d i n g r e f e r r a l f o r e c h o c a r d i o g r a p h y a n d / o r to a
p e d i a t r i c c a r d i o l o g i s t d e p e n d s o n (a) t h e c l i n i c a l
i m p r e s s i o n ( ' i n n o c e n t ' o r p a t h o l o g i c m u r m u r ) , (b) t h e
' c o m f o r t ' factor i.e. the a n x i e t y level of the parents, (c) the
c o n f i d e n c e of t h e e x a m i n i n g p e d i a t r i c i a n a n d (d) t h e
a v a i l a b i l i t y of the child for f o l l o w - u p .
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Indian Journal of Pediatrics, Volume 71~January, 2004