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Management of
asymptomatic heart murmurs
Gordon Gladman
Abstract
Asymptomatic cardiac murmurs are a common finding in paediatric
patients with some estimates suggesting that 90% of children have
a murmur detected at some stage. Most are benign ‘innocent’ noises or
reflect minor structural heart disease of no haemodynamic significance.
The degree of parental anxiety invoked by the detection of a murmur in
their child is considerable and although in virtually all cases their
concerns of significant underlying cardiac disease are unjustified,
providing adequate reassurance is a challenge for the health profes-
sionals involved. The aim of this article is to guide practitioners in deter-
mining which babies or children need urgent referral for a specialist
opinion and to provide management suggestions for when a murmur is
noted coincidentally during a routine examination.
Keywords children; congenital heart disease; heart murmurs; infant
Introduction
There remains a spectrum of varying practice in the management
of asymptomatic heart murmurs detected in neonates, infants
and older children with no nationally agreed guidelines. The NHS
Newborn and Infant Physical Examination Programme (NIPE) is
presently reviewing standards on the management of heart
murmurs detected in newborns and infants, necessary as a recent
evaluation of the policies followed locally in 116 neonatal units
throughout the UK revealed wide discrepancies in practice.
The past 15e20 years has witnessed a change in who is likely
to detect an asymptomatic heart murmur. Newborn and infant
screening examinations are now frequently undertaken by nurse
practitioners, often with a neonatal or midwifery background but
initial concerns that their lack of conventional medical training
would result in a failure to identify congenital heart disease
appear unfounded. The ‘gold standard’ in determining if a heart
murmur is pathological (reflecting underlying cardiac disease) or
benign (innocent in nature) remains echocardiography and the
emergence of paediatricians and neonatologists with expertise in
cardiology to support the limited number of paediatric cardiolo-
gists has greatly increased access to such investigations. None-
theless, with up to 80e90% of neonates, infants and children
having a heart murmur detected at some stage in life it is unre-
alistic to offer ‘screening echocardiography’ to all, thus some
structured approach to the management of heart murmurs is
essential.
Overview of paediatric heart murmurs
Commonly encountered in clinical practice, heart murmurs are
created by turbulent blood flow within the heart and circulatory
system. They are typically classified according to anatomic
location (where murmur is best heard); timing within the
cardiac cycle (systolic, diastolic or continuous); intensity
(barely audible to being heard with the stethoscope off the
chest); quality (soft, harsh, blowing, musical, vibratory) and
site of radiation (most distant anatomic location where the
murmur can still be heard).
The sub-classification of murmurs into systolic, diastolic,
continuous and then again into early, pan, mid or late within
each cardiac cycle timing period has always proved challenging
for many practitioners. The advent of the digital acoustic
stethoscope not only provided a high quality ‘digitally remas-
tered’ version of the murmur to the listener’s ear but was also
capable of audio recording and playback and by connecting the
‘stethophone’ to an off-instrument computer loaded with
auscultation software, the audio signal could be analyzed and
differentiation between pathological and innocent murmurs
theoretically be made. Even in a technology age where tele-
medicine, wireless technology and Bluetooth connections allow
heart murmurs to be captured within the electronic stethoscope,
transmitted wirelessly to a remote PC for analysis and ‘re-
listening’ by clinicians, such advances are rarely seen in clinical
practice and have not proved the diagnostic panacea once
envisaged.
The parental anxiety induced following the detection of
a cardiac murmur in their infant or child should not be under-
estimated, every family assuming the murmur signifies signifi-
cant underlying heart disease even though the reality is that in
less than 1e2% will this be the case. The responsibility of the
health professional who first detects the heart murmur is to try
and identify which murmurs suggest likely cardiac disease from
the much more common physiological or innocent noises and
decide on any potential follow up arrangements, organizing
appropriate referrals as necessary.
Access to additional investigations depends upon the
circumstances in which the murmur is first heard. In a hospital
setting it is easier to obtain upper and lower limb blood pressure
assessments, oxygen saturation levels, chest X-rays and 12 lead
ECGs than in a General Practitioner’s (GPs) surgery or commu-
nity clinic. The relative merits of these investigations will be
discussed later.
As most major or complex congenital cardiac lesions present
with significant symptoms and signs before 6 weeks of age,
a higher threshold of suspicion should be maintained for
a murmur heard within the neonatal period (even if the baby is
asymptomatic) than when the murmur is detected for the first
time in an older infant or child. The management of asymp-
tomatic murmurs within these two age ranges will be considered
separately.
Asymptomatic murmurs in neonates and young infants
Early studies suggested murmurs are present in up to 60% of
healthy newborn babies, however, more recent reviews quote an
incidence of less than 10%. The major circulatory adaptations
that occur following delivery can produce a series of transient
Gordon Gladman MBChB MRCP (UK) is Consultant Paediatric Cardiologist
at Royal Liverpool Children’s Hospital, Eaton Road, Liverpool, UK.
SYMPOSIUM: CARDIOVASCULAR
PAEDIATRICS AND CHILD HEALTH 23:2 64 Ó 2012 Published by Elsevier Ltd.
murmurs but can also mask even significant cardiac disease. The
left to right shunt through a large ventricular septal defect may be
minimal in the first few days of life due to the high pulmonary
vascular resistance (PVR) observed in many neonates, the typical
murmur and symptoms (breathlessness and poor feeding) only
becoming apparent as the PVR naturally reduces over the first
few weeks of life. Elevated PVR can also produce transient
tricuspid regurgitant murmurs, which can be harsh and raise
concerns of a major cardiac defect but as the PVR falls, so the
murmur disappears leaving an anatomically and haemody-
namically normal heart.
A common murmur heard in many term babies is that of
physiological branch pulmonary artery stenosis. Producing a less
harsh systolic murmur than true valvar stenosis, the noise is
created by turbulent blood flow through slightly small peripheral
pulmonary arteries, the murmur is easily heard at the back over
the lung fields, produces no symptoms and is accompanied by no
other clinical signs. Typically with growth the murmur disap-
pears in the first year as the vessels develop and turbulent flow is
no longer created.
In the neonatal unit, many of the preterm population will have
clinical and (if looked for) echocardiographic evidence of
a patent ductus arteriosus (PDA) but in only a few will the duct
be creating significant haemodynamic effects necessitating
intervention. Many PDAs will close spontaneously in the first 12
months following discharge from the neonatal unit and again few
cause symptoms.
In determining which neonates to refer for further investi-
gation, history and examination remain important. Routine
antenatal scanning will detect at best 30e40% of major
congenital heart disease (lesions likely to require surgical
intervention) and will commonly miss septal defects and
conditions that affect the outflow tracts like transposition of the
great arteries. There may be clues in the maternal obstetric
history (sodium valproate use, heavy alcohol ingestion) that
predispose the baby to congenital cardiac disease or there may
be accompanying abnormalities in other organs/systems
consistent with a syndrome or genetic condition. A ‘difficult
delivery’ requiring a degree of neonatal resuscitation may
result in significant elevation of pulmonary vascular resistance
predisposing the baby to transient ductal or tricuspid regur-
gitant murmurs as discussed earlier.
The presence of central cyanosis in the absence of symptoms
should raise suspicions of underlying congenital cardiac disease
(transposition, obstructed pulmonary blood flow, pulmonary
venous return anomalies) and several studies have indicated an
important role for oxygen saturation monitor screening around
24 hours of age in all apparently healthy babies. Careful assess-
ment of the perfusion by determining the blood pressure and the
presence (or absence) of upper and lower limb pulses can help
detect left sided cardiac defects (arch anomalies, hypoplastic left
heart). The relative merits of measuring right arm and a lower
limb blood pressure to determine a pressure gradient in potential
coarctation of the aorta have long been discussed. In practice,
few babies tolerate having their blood pressure measured
without becoming upset and the readings are prone to error and
inaccuracy.
As many of the newborns will be in a hospital setting,
they can potentially undergo chest X-ray and 12 lead electro
cardiograms (ECGs). In a truly asymptomatic baby where the
heart murmur is the only clinical sign, few studies have shown
these investigations to be useful and many paediatricians
and paediatric cardiologists no longer undertake them.
For those neonates who have accompanying cardiac symp-
toms or signs, the decision to refer for a specialist opinion is
straightforward. Traditional teaching in many paediatric depart-
ments for the truly asymptomatic ‘isolated’ heart murmur
detected during the newborn examination, is a second exami-
nation prior to discharge by a more experienced clinician and if
no other signs are detected and the baby is well, discharge is
allowed with an early (1e2 week) review. The family is usually
given advice to return if they have any concerns over the baby
(poor feeding, breathlessness) and many clinicians adopt the
policy of then referring the baby to a specialist if the murmur
persists at the first review appointment.
Any clinician who has been working for a reasonable length of
time has experienced sending home an apparently ‘healthy baby’
with or without an isolated murmur, only for the baby to
represent within hours or days in ‘extremis’ with a duct-
dependent cardiac lesion that has become manifest as a result
of closure of the duct following discharge. Such cases add
support to those who advocate that any neonate with a murmur
should undergo echocardiography prior to discharge. The
emergence of neonatologists and paediatricians with expertise in
cardiology means for some hospitals this is now more realistic
than 10 years ago, but the relative ‘limitations’ of the normal
scan in this age group need to be highlighted. Assessing
pulmonary venous anatomy, detecting septal defects and
screening for arch anomalies can be very difficult in neonates
(even for experienced paediatric cardiologists) due to echocar-
diographic challenges created by the physiological changes and
PVR adaptations that occur following delivery. Paediatricians
still need to rely on basic clinical experience and ‘gut instinct’
and even if the local scan is reported as normal, if there are
sufficient clinical concerns, an early specialist referral is
indicated.
The older infant and child
Outside of the neonatal period most heart murmurs are heard
during the course of routine examinations (well baby check) or
during the course of a consultation for a non-cardiac concern,
where as part of the general examination a murmur is noted for
the first time. The vast majority of murmurs in this setting will be
benign (innocent) and it is extremely unlikely that a haemody-
namically significant heart lesion will present for the first time in
this manner. The challenge for the health professional is to try
and identify the more minor heart lesion which might require
regular review and possibly an intervention in the medium to
longterm, for instance small to medium sized ventricular septal
defects, atrial septal defects or mild degrees of semi-lunar (aortic
and pulmonary) valve stenosis.
As with the neonate, a careful history and examination is
essential. Is the child feeding well, thriving, active and gener-
ally healthy? Do the characteristics of the murmur seem
consistent with those typical of an innocent murmur (Table 1)?
Are there other clinical signs: evidence of cyanosis (oxygen
saturation monitoring is useful if available, especially in ethnic
SYMPOSIUM: CARDIOVASCULAR
PAEDIATRICS AND CHILD HEALTH 23:2 65 Ó 2012 Published by Elsevier Ltd.
minority groups where central cyanosis is often hard to detect);
is there a palpable thrill; are all pulses (upper and lower limb)
easily felt as coarctation of the aorta can present at any age. As
before, chest X-ray and ECG are unlikely to be helpful and if
serious consideration is being given to performing these, it is
often more useful to arrange a cardiology opinion and an
echocardiogram.
The ultimate decision as to whether to refer for a specialist
opinion depends upon the GP’s or paediatrician’s confidence in
their abilities to exclude underlying heart disease and reassure
the family over the benign nature of the murmur. Over the years
many papers have demonstrated that paediatricians and paedi-
atric cardiologists are good at differentiating physiological
(benign) murmurs from pathological ones clinically, yet requests
for echocardiograms have risen exponentially over the past two
decades and many paediatric cardiologists find it more beneficial
(in terms of time and parental reassurance) to undertake an
echocardiogram in patients even when they are convinced clin-
ically the murmur is innocent: it often takes longer to explain
why an echocardiogram is not needed than to simply perform
one!
Parental expectations founded on Internet information and
media advice often leads to a situation where simple reassurance
following a thorough clinical examination is inadequate, neces-
sitating a specialist referral and an echocardiogram. A significant
amount of paediatric practice involves dealing with family
anxiety around ‘suspected disease’ or relatively minor ailments
and if a ‘cardiac neurosis’ is developing within a family over
what is clearly an ‘innocent murmur’ in their child, this probably
does constitute reasonable grounds for a specialist referral for
reassurance.
Increasing media coverage of sudden death in young, appar-
ently healthy children and adults has again lowered the ‘referral
bar’ if an asymptomatic murmur is detected in a young person
who regularly undertakes strenuous competitive sports. Despite
intense coverage of high profile cases (footballers, young
athletes) that result in campaigns advocating screening of all
young sportsmen and women (and potentially all children), the
evidence that screening can protect against sudden death is
incomplete and it is unlikely that universal screening will ever be
adopted.
The influence of any relevant family history, presence of an
associated genetic condition (trisomy 21) or syndrome (Noon-
ans, Williams) should again be considered in the referral decision
making process on detecting a heart murmur. The factors that
might influence the decision to formally refer an asymptomatic
murmur detected in an older child for a paediatric cardiology
opinion are outlined in Table 2.
Cardiology networks/national safe & sustainable review
The ‘safe and sustainable’ national review of paediatric cardi-
ology and cardiothoracic services is due to announce its findings
in July, 2012. In addition to proposing a reduction in the number
of paediatric cardiac surgical centres, the review outlined
a vision for regional paediatric cardiology networks that would
provide ‘cradle to grave’ cover from antenatal detection of
congenital cardiac disease to care of the adult born with
congenital cardiac lesions. The five ‘interlinking’ services would
include GPs and Community Paediatric services; local district
hospitals without a paediatrician with expertise in cardiology;
district children’s cardiology services (local hospitals that have
a consultant paediatrician with expertise in cardiology); Chil-
dren’s Cardiology Centres (based in a tertiary level hospital and
employing paediatric cardiologists) and Specialist Paediatric
Cardiology and Cardiothoracic Centres (tertiary level hospitals
employing both paediatric cardiologists and paediatric cardio-
thoracic surgeons). Referral patterns and exactly who assesses
an infant or child with a murmur depends upon local
geographical arrangements and what facilities and services are
available.
The increasing availability of access to echocardiography,
coupled with shortening of the training time to become
a consultant (and presumably reduction in clinical experience
gained) makes it likely that the paediatricians of tomorrow are
going to resort to echocardiography more often than the
consultants of 20 years ago. In principle there is little to fault with
this approach, certainly a policy where an asymptomatic murmur
is reviewed a few weeks after it is first heard and if still present is
referred for a more definitive opinion (which will usually involve
an echocardiogram) is preferable to the old system many of us
witnessed during training, where the child is frequently reviewed
6 monthly for many years with no clear decision ever being
made.
Factors that should prompt a specialist referral when
a murmur is detected
C Any accompanying symptoms
C Any accompanying signs (murmur not consistent with signs
outlined in Figure 1)
C First degree relative born with congenital heart disease
C Presence of underlying syndrome or genetic condition
C History of rheumatic fever (often presents as Sydenham’s chorea
in UK) or Kawasaki disease
C Prenatal factors: maternal medications; alcohol ingestion; in
utero infections; infant of diabetic mother (significantly
increased risk of structural cardiac lesions in offspring)
C Preterm delivery: many very low birth weight survivors will have
evidence of a persistent ductus arteriosus
C Extreme family anxiety
Table 2
Characteristics of an innocent murmur
C Otherwise healthy individual
C Usually systolic in timing and short duration (never diastolic
alone)
C Soft in quality and of low intensity (never harsh and loud)
C Often positional (heard when supine, disappears on standing)
C Comes and goes
C No palpable thrill
C Normal 1st and 2nd heart sounds
Table 1
SYMPOSIUM: CARDIOVASCULAR
PAEDIATRICS AND CHILD HEALTH 23:2 66 Ó 2012 Published by Elsevier Ltd.
Conclusion
Asymptomatic heart murmurs are common throughout the stages
of childhood and indeed some authors suggest will be heard in
virtually all children at some point. Thankfully nearly all are
‘innocent’ in nature but many families will require considerable
reassurance that their child does not have significant cardiac
disease that is likely to restrict them in life. Internet advice and
media articles make it increasingly difficult for such reassurance to
be given with conviction in the absence of a ‘defining echocar-
diogram’. It is probably reasonable for a truly asymptomatic
murmur that is detected coincidentally during the course of
a clinical examination, to be reviewed a few weeks later and if still
present, for the child to be referred for a specialist opinion. A
flowchart suggesting a potential management strategy following
the detection of an asymptomatic murmur is outlined in Figure 1.
Chest X-rays and electrocardiograms appear to provide a low
predictive value in differentiating benign from pathological
murmurs and appear generally unhelpful. Ultimately the decision
whether to refer or not depends on the practitioner’s confidence
in their abilities, whether the murmur is accompanied by any
other signs or symptoms and the extent of family anxiety. A
FURTHER READING
Ainsworth SB, Wyllie JP, Wren C.Prevalence andclinicalsignificance of cardiac
murmurs in neonates. Arch Dis Child Fetal Neonatal Ed 1999; 80: F43e5.
Arlettaz R, Archer N, Wilkinson AR. Natural history of innocent heart
murmurs in newborn babies: controlled echocardiographic study. Arch
Dis Child Fetal Neonatal Ed 1998; 78: F166e70.
Azhar AS, Habib HS. Accuracy of the initial evaluation of heart murmurs in
neonates: do we need an echocardiogram? Pediatr Cardiol 2006; 27:
234e7.
Ewer AK, Middleton LJ, Furmston AT, et al. Pulse oximetry screening for
congenital heart defects in newborn infants (PulseOx): a test accuracy
study. Lancet 27 Aug 2011; 378: 785e94.
Gardiner S. Are routine chest x-ray and ECG examinations helpful in the
evaluation of asymptomatic heart murmurs? Arch Dis Child 2003; 88:
638e42.
Gokmen Z, Tunaoglu FS, Kula S, Ergenekon E, Ozkiraz S, Olgunturk R.
Comparison of initial evaluation of neonatal heart murmurs by pedi-
atrician and pediatric cardiologist. J Matern Fetal Neonatal Med 2009
Nov; 22: 1086e91.
Gregory J, Emslie A, Wyllie J, Wren C. Examination for cardiac malforma-
tions at six weeks of age. Arch Dis Child Fetal Neonatal Ed 1999; 80:
F46e8.
Johnson R, Holzer R. Evaluation of asymptomatic heart murmurs. Curr
Pediatr 2005; 15: 532e8.
Manning D, Paweletz A, Robertson JL. Management of asymptomatic heart
murmurs in infants and children. Pediatr Child Health 2009; 19: 25e9.
Morris G, Wilson DG. Management of asymptomatic heart murmurs. Curr
Pediatr 2000; 10: 242e7.
Norman M. Detecting heart defects in newborn infants e innocent
murmurs mixed with silent dangers. Acta Paediatr 2006; 95: 391e3.
Oeppen RS, Fairhurst JJ, Argent JD. Diagnostic value of the chest radio-
graph in asymptomatic neonates with a cardiac murmur. Clin Radiol
2002; 57: 736e40.
Patton C, Hey E. How effectively can clinical examination pick up
congenital heart disease at birth? Arch Dis Child Fetal Neonatal Ed
2006; 91: F263e7.
Shenvi A, Kapur J, Rasiah SV. Current practice of the management of asymp-
tomatic heart murmurs in neonates in UK. Arch Dis Child Fetal Neonatal Ed
2011; 96: Fa32. http://dx.doi.org/10.1136/archdischild.2011.300164.52.
Wren C, Richmond S, Donaldson L. Presentation of congenital heart
disease in infancy: implications for routine examination. Arch Dis Child
Fetal Neonatal Ed 1999; 80: F49e53.
No additional
clinical findings or
concerns (table 2)
Additional clinical
signs (especially
Sa02 <92%) or
concerns (table 2)
No additional
clinical findings or
concerns (table 2)
If murmur no longer audible and infant
healthy, discharge. If murmur still present
+/- symptoms arrange referral
If murmur no longer audible, discharge.
If still present arrange referral
Additional clinical
signs or concerns
(table 2)
Review in
1–2 weeks
Seek urgent
specialist advice
Review in 6 weeks
Organise
specialist referral
Age < 6 weeks Age > 6 weeks
Murmur detected
(asymptomatic)
Figure 1 Flowchart suggesting management strategy following identification of an asymptomatic murmur.
SYMPOSIUM: CARDIOVASCULAR
PAEDIATRICS AND CHILD HEALTH 23:2 67 Ó 2012 Published by Elsevier Ltd.
Practice points
C A heart murmur will be heard in most children at some stage,
but few will have cardiac pathology.
C Murmurs noted in the first weeks/months of life are more likely
to be pathological than those first detected in an older child.
C Central cyanosis is difficult to detect in children (especially
ethnic minorities).
C Clinical examination combined with oxygen saturation monitor
assessment (where possible) allows effective ‘triaging’ of who
needs to be referred urgently.
C Chest X-rays and electrocardiograms are of little value in
differentiating benign from pathological murmurs.
SYMPOSIUM: CARDIOVASCULAR
PAEDIATRICS AND CHILD HEALTH 23:2 68 Ó 2012 Published by Elsevier Ltd.

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Management of asymptomatic heart murmurs in children

  • 1. Management of asymptomatic heart murmurs Gordon Gladman Abstract Asymptomatic cardiac murmurs are a common finding in paediatric patients with some estimates suggesting that 90% of children have a murmur detected at some stage. Most are benign ‘innocent’ noises or reflect minor structural heart disease of no haemodynamic significance. The degree of parental anxiety invoked by the detection of a murmur in their child is considerable and although in virtually all cases their concerns of significant underlying cardiac disease are unjustified, providing adequate reassurance is a challenge for the health profes- sionals involved. The aim of this article is to guide practitioners in deter- mining which babies or children need urgent referral for a specialist opinion and to provide management suggestions for when a murmur is noted coincidentally during a routine examination. Keywords children; congenital heart disease; heart murmurs; infant Introduction There remains a spectrum of varying practice in the management of asymptomatic heart murmurs detected in neonates, infants and older children with no nationally agreed guidelines. The NHS Newborn and Infant Physical Examination Programme (NIPE) is presently reviewing standards on the management of heart murmurs detected in newborns and infants, necessary as a recent evaluation of the policies followed locally in 116 neonatal units throughout the UK revealed wide discrepancies in practice. The past 15e20 years has witnessed a change in who is likely to detect an asymptomatic heart murmur. Newborn and infant screening examinations are now frequently undertaken by nurse practitioners, often with a neonatal or midwifery background but initial concerns that their lack of conventional medical training would result in a failure to identify congenital heart disease appear unfounded. The ‘gold standard’ in determining if a heart murmur is pathological (reflecting underlying cardiac disease) or benign (innocent in nature) remains echocardiography and the emergence of paediatricians and neonatologists with expertise in cardiology to support the limited number of paediatric cardiolo- gists has greatly increased access to such investigations. None- theless, with up to 80e90% of neonates, infants and children having a heart murmur detected at some stage in life it is unre- alistic to offer ‘screening echocardiography’ to all, thus some structured approach to the management of heart murmurs is essential. Overview of paediatric heart murmurs Commonly encountered in clinical practice, heart murmurs are created by turbulent blood flow within the heart and circulatory system. They are typically classified according to anatomic location (where murmur is best heard); timing within the cardiac cycle (systolic, diastolic or continuous); intensity (barely audible to being heard with the stethoscope off the chest); quality (soft, harsh, blowing, musical, vibratory) and site of radiation (most distant anatomic location where the murmur can still be heard). The sub-classification of murmurs into systolic, diastolic, continuous and then again into early, pan, mid or late within each cardiac cycle timing period has always proved challenging for many practitioners. The advent of the digital acoustic stethoscope not only provided a high quality ‘digitally remas- tered’ version of the murmur to the listener’s ear but was also capable of audio recording and playback and by connecting the ‘stethophone’ to an off-instrument computer loaded with auscultation software, the audio signal could be analyzed and differentiation between pathological and innocent murmurs theoretically be made. Even in a technology age where tele- medicine, wireless technology and Bluetooth connections allow heart murmurs to be captured within the electronic stethoscope, transmitted wirelessly to a remote PC for analysis and ‘re- listening’ by clinicians, such advances are rarely seen in clinical practice and have not proved the diagnostic panacea once envisaged. The parental anxiety induced following the detection of a cardiac murmur in their infant or child should not be under- estimated, every family assuming the murmur signifies signifi- cant underlying heart disease even though the reality is that in less than 1e2% will this be the case. The responsibility of the health professional who first detects the heart murmur is to try and identify which murmurs suggest likely cardiac disease from the much more common physiological or innocent noises and decide on any potential follow up arrangements, organizing appropriate referrals as necessary. Access to additional investigations depends upon the circumstances in which the murmur is first heard. In a hospital setting it is easier to obtain upper and lower limb blood pressure assessments, oxygen saturation levels, chest X-rays and 12 lead ECGs than in a General Practitioner’s (GPs) surgery or commu- nity clinic. The relative merits of these investigations will be discussed later. As most major or complex congenital cardiac lesions present with significant symptoms and signs before 6 weeks of age, a higher threshold of suspicion should be maintained for a murmur heard within the neonatal period (even if the baby is asymptomatic) than when the murmur is detected for the first time in an older infant or child. The management of asymp- tomatic murmurs within these two age ranges will be considered separately. Asymptomatic murmurs in neonates and young infants Early studies suggested murmurs are present in up to 60% of healthy newborn babies, however, more recent reviews quote an incidence of less than 10%. The major circulatory adaptations that occur following delivery can produce a series of transient Gordon Gladman MBChB MRCP (UK) is Consultant Paediatric Cardiologist at Royal Liverpool Children’s Hospital, Eaton Road, Liverpool, UK. SYMPOSIUM: CARDIOVASCULAR PAEDIATRICS AND CHILD HEALTH 23:2 64 Ó 2012 Published by Elsevier Ltd.
  • 2. murmurs but can also mask even significant cardiac disease. The left to right shunt through a large ventricular septal defect may be minimal in the first few days of life due to the high pulmonary vascular resistance (PVR) observed in many neonates, the typical murmur and symptoms (breathlessness and poor feeding) only becoming apparent as the PVR naturally reduces over the first few weeks of life. Elevated PVR can also produce transient tricuspid regurgitant murmurs, which can be harsh and raise concerns of a major cardiac defect but as the PVR falls, so the murmur disappears leaving an anatomically and haemody- namically normal heart. A common murmur heard in many term babies is that of physiological branch pulmonary artery stenosis. Producing a less harsh systolic murmur than true valvar stenosis, the noise is created by turbulent blood flow through slightly small peripheral pulmonary arteries, the murmur is easily heard at the back over the lung fields, produces no symptoms and is accompanied by no other clinical signs. Typically with growth the murmur disap- pears in the first year as the vessels develop and turbulent flow is no longer created. In the neonatal unit, many of the preterm population will have clinical and (if looked for) echocardiographic evidence of a patent ductus arteriosus (PDA) but in only a few will the duct be creating significant haemodynamic effects necessitating intervention. Many PDAs will close spontaneously in the first 12 months following discharge from the neonatal unit and again few cause symptoms. In determining which neonates to refer for further investi- gation, history and examination remain important. Routine antenatal scanning will detect at best 30e40% of major congenital heart disease (lesions likely to require surgical intervention) and will commonly miss septal defects and conditions that affect the outflow tracts like transposition of the great arteries. There may be clues in the maternal obstetric history (sodium valproate use, heavy alcohol ingestion) that predispose the baby to congenital cardiac disease or there may be accompanying abnormalities in other organs/systems consistent with a syndrome or genetic condition. A ‘difficult delivery’ requiring a degree of neonatal resuscitation may result in significant elevation of pulmonary vascular resistance predisposing the baby to transient ductal or tricuspid regur- gitant murmurs as discussed earlier. The presence of central cyanosis in the absence of symptoms should raise suspicions of underlying congenital cardiac disease (transposition, obstructed pulmonary blood flow, pulmonary venous return anomalies) and several studies have indicated an important role for oxygen saturation monitor screening around 24 hours of age in all apparently healthy babies. Careful assess- ment of the perfusion by determining the blood pressure and the presence (or absence) of upper and lower limb pulses can help detect left sided cardiac defects (arch anomalies, hypoplastic left heart). The relative merits of measuring right arm and a lower limb blood pressure to determine a pressure gradient in potential coarctation of the aorta have long been discussed. In practice, few babies tolerate having their blood pressure measured without becoming upset and the readings are prone to error and inaccuracy. As many of the newborns will be in a hospital setting, they can potentially undergo chest X-ray and 12 lead electro cardiograms (ECGs). In a truly asymptomatic baby where the heart murmur is the only clinical sign, few studies have shown these investigations to be useful and many paediatricians and paediatric cardiologists no longer undertake them. For those neonates who have accompanying cardiac symp- toms or signs, the decision to refer for a specialist opinion is straightforward. Traditional teaching in many paediatric depart- ments for the truly asymptomatic ‘isolated’ heart murmur detected during the newborn examination, is a second exami- nation prior to discharge by a more experienced clinician and if no other signs are detected and the baby is well, discharge is allowed with an early (1e2 week) review. The family is usually given advice to return if they have any concerns over the baby (poor feeding, breathlessness) and many clinicians adopt the policy of then referring the baby to a specialist if the murmur persists at the first review appointment. Any clinician who has been working for a reasonable length of time has experienced sending home an apparently ‘healthy baby’ with or without an isolated murmur, only for the baby to represent within hours or days in ‘extremis’ with a duct- dependent cardiac lesion that has become manifest as a result of closure of the duct following discharge. Such cases add support to those who advocate that any neonate with a murmur should undergo echocardiography prior to discharge. The emergence of neonatologists and paediatricians with expertise in cardiology means for some hospitals this is now more realistic than 10 years ago, but the relative ‘limitations’ of the normal scan in this age group need to be highlighted. Assessing pulmonary venous anatomy, detecting septal defects and screening for arch anomalies can be very difficult in neonates (even for experienced paediatric cardiologists) due to echocar- diographic challenges created by the physiological changes and PVR adaptations that occur following delivery. Paediatricians still need to rely on basic clinical experience and ‘gut instinct’ and even if the local scan is reported as normal, if there are sufficient clinical concerns, an early specialist referral is indicated. The older infant and child Outside of the neonatal period most heart murmurs are heard during the course of routine examinations (well baby check) or during the course of a consultation for a non-cardiac concern, where as part of the general examination a murmur is noted for the first time. The vast majority of murmurs in this setting will be benign (innocent) and it is extremely unlikely that a haemody- namically significant heart lesion will present for the first time in this manner. The challenge for the health professional is to try and identify the more minor heart lesion which might require regular review and possibly an intervention in the medium to longterm, for instance small to medium sized ventricular septal defects, atrial septal defects or mild degrees of semi-lunar (aortic and pulmonary) valve stenosis. As with the neonate, a careful history and examination is essential. Is the child feeding well, thriving, active and gener- ally healthy? Do the characteristics of the murmur seem consistent with those typical of an innocent murmur (Table 1)? Are there other clinical signs: evidence of cyanosis (oxygen saturation monitoring is useful if available, especially in ethnic SYMPOSIUM: CARDIOVASCULAR PAEDIATRICS AND CHILD HEALTH 23:2 65 Ó 2012 Published by Elsevier Ltd.
  • 3. minority groups where central cyanosis is often hard to detect); is there a palpable thrill; are all pulses (upper and lower limb) easily felt as coarctation of the aorta can present at any age. As before, chest X-ray and ECG are unlikely to be helpful and if serious consideration is being given to performing these, it is often more useful to arrange a cardiology opinion and an echocardiogram. The ultimate decision as to whether to refer for a specialist opinion depends upon the GP’s or paediatrician’s confidence in their abilities to exclude underlying heart disease and reassure the family over the benign nature of the murmur. Over the years many papers have demonstrated that paediatricians and paedi- atric cardiologists are good at differentiating physiological (benign) murmurs from pathological ones clinically, yet requests for echocardiograms have risen exponentially over the past two decades and many paediatric cardiologists find it more beneficial (in terms of time and parental reassurance) to undertake an echocardiogram in patients even when they are convinced clin- ically the murmur is innocent: it often takes longer to explain why an echocardiogram is not needed than to simply perform one! Parental expectations founded on Internet information and media advice often leads to a situation where simple reassurance following a thorough clinical examination is inadequate, neces- sitating a specialist referral and an echocardiogram. A significant amount of paediatric practice involves dealing with family anxiety around ‘suspected disease’ or relatively minor ailments and if a ‘cardiac neurosis’ is developing within a family over what is clearly an ‘innocent murmur’ in their child, this probably does constitute reasonable grounds for a specialist referral for reassurance. Increasing media coverage of sudden death in young, appar- ently healthy children and adults has again lowered the ‘referral bar’ if an asymptomatic murmur is detected in a young person who regularly undertakes strenuous competitive sports. Despite intense coverage of high profile cases (footballers, young athletes) that result in campaigns advocating screening of all young sportsmen and women (and potentially all children), the evidence that screening can protect against sudden death is incomplete and it is unlikely that universal screening will ever be adopted. The influence of any relevant family history, presence of an associated genetic condition (trisomy 21) or syndrome (Noon- ans, Williams) should again be considered in the referral decision making process on detecting a heart murmur. The factors that might influence the decision to formally refer an asymptomatic murmur detected in an older child for a paediatric cardiology opinion are outlined in Table 2. Cardiology networks/national safe & sustainable review The ‘safe and sustainable’ national review of paediatric cardi- ology and cardiothoracic services is due to announce its findings in July, 2012. In addition to proposing a reduction in the number of paediatric cardiac surgical centres, the review outlined a vision for regional paediatric cardiology networks that would provide ‘cradle to grave’ cover from antenatal detection of congenital cardiac disease to care of the adult born with congenital cardiac lesions. The five ‘interlinking’ services would include GPs and Community Paediatric services; local district hospitals without a paediatrician with expertise in cardiology; district children’s cardiology services (local hospitals that have a consultant paediatrician with expertise in cardiology); Chil- dren’s Cardiology Centres (based in a tertiary level hospital and employing paediatric cardiologists) and Specialist Paediatric Cardiology and Cardiothoracic Centres (tertiary level hospitals employing both paediatric cardiologists and paediatric cardio- thoracic surgeons). Referral patterns and exactly who assesses an infant or child with a murmur depends upon local geographical arrangements and what facilities and services are available. The increasing availability of access to echocardiography, coupled with shortening of the training time to become a consultant (and presumably reduction in clinical experience gained) makes it likely that the paediatricians of tomorrow are going to resort to echocardiography more often than the consultants of 20 years ago. In principle there is little to fault with this approach, certainly a policy where an asymptomatic murmur is reviewed a few weeks after it is first heard and if still present is referred for a more definitive opinion (which will usually involve an echocardiogram) is preferable to the old system many of us witnessed during training, where the child is frequently reviewed 6 monthly for many years with no clear decision ever being made. Factors that should prompt a specialist referral when a murmur is detected C Any accompanying symptoms C Any accompanying signs (murmur not consistent with signs outlined in Figure 1) C First degree relative born with congenital heart disease C Presence of underlying syndrome or genetic condition C History of rheumatic fever (often presents as Sydenham’s chorea in UK) or Kawasaki disease C Prenatal factors: maternal medications; alcohol ingestion; in utero infections; infant of diabetic mother (significantly increased risk of structural cardiac lesions in offspring) C Preterm delivery: many very low birth weight survivors will have evidence of a persistent ductus arteriosus C Extreme family anxiety Table 2 Characteristics of an innocent murmur C Otherwise healthy individual C Usually systolic in timing and short duration (never diastolic alone) C Soft in quality and of low intensity (never harsh and loud) C Often positional (heard when supine, disappears on standing) C Comes and goes C No palpable thrill C Normal 1st and 2nd heart sounds Table 1 SYMPOSIUM: CARDIOVASCULAR PAEDIATRICS AND CHILD HEALTH 23:2 66 Ó 2012 Published by Elsevier Ltd.
  • 4. Conclusion Asymptomatic heart murmurs are common throughout the stages of childhood and indeed some authors suggest will be heard in virtually all children at some point. Thankfully nearly all are ‘innocent’ in nature but many families will require considerable reassurance that their child does not have significant cardiac disease that is likely to restrict them in life. Internet advice and media articles make it increasingly difficult for such reassurance to be given with conviction in the absence of a ‘defining echocar- diogram’. It is probably reasonable for a truly asymptomatic murmur that is detected coincidentally during the course of a clinical examination, to be reviewed a few weeks later and if still present, for the child to be referred for a specialist opinion. A flowchart suggesting a potential management strategy following the detection of an asymptomatic murmur is outlined in Figure 1. Chest X-rays and electrocardiograms appear to provide a low predictive value in differentiating benign from pathological murmurs and appear generally unhelpful. Ultimately the decision whether to refer or not depends on the practitioner’s confidence in their abilities, whether the murmur is accompanied by any other signs or symptoms and the extent of family anxiety. A FURTHER READING Ainsworth SB, Wyllie JP, Wren C.Prevalence andclinicalsignificance of cardiac murmurs in neonates. Arch Dis Child Fetal Neonatal Ed 1999; 80: F43e5. Arlettaz R, Archer N, Wilkinson AR. Natural history of innocent heart murmurs in newborn babies: controlled echocardiographic study. Arch Dis Child Fetal Neonatal Ed 1998; 78: F166e70. Azhar AS, Habib HS. Accuracy of the initial evaluation of heart murmurs in neonates: do we need an echocardiogram? Pediatr Cardiol 2006; 27: 234e7. Ewer AK, Middleton LJ, Furmston AT, et al. Pulse oximetry screening for congenital heart defects in newborn infants (PulseOx): a test accuracy study. Lancet 27 Aug 2011; 378: 785e94. Gardiner S. Are routine chest x-ray and ECG examinations helpful in the evaluation of asymptomatic heart murmurs? Arch Dis Child 2003; 88: 638e42. Gokmen Z, Tunaoglu FS, Kula S, Ergenekon E, Ozkiraz S, Olgunturk R. Comparison of initial evaluation of neonatal heart murmurs by pedi- atrician and pediatric cardiologist. J Matern Fetal Neonatal Med 2009 Nov; 22: 1086e91. Gregory J, Emslie A, Wyllie J, Wren C. Examination for cardiac malforma- tions at six weeks of age. Arch Dis Child Fetal Neonatal Ed 1999; 80: F46e8. Johnson R, Holzer R. Evaluation of asymptomatic heart murmurs. Curr Pediatr 2005; 15: 532e8. Manning D, Paweletz A, Robertson JL. Management of asymptomatic heart murmurs in infants and children. Pediatr Child Health 2009; 19: 25e9. Morris G, Wilson DG. Management of asymptomatic heart murmurs. Curr Pediatr 2000; 10: 242e7. Norman M. Detecting heart defects in newborn infants e innocent murmurs mixed with silent dangers. Acta Paediatr 2006; 95: 391e3. Oeppen RS, Fairhurst JJ, Argent JD. Diagnostic value of the chest radio- graph in asymptomatic neonates with a cardiac murmur. Clin Radiol 2002; 57: 736e40. Patton C, Hey E. How effectively can clinical examination pick up congenital heart disease at birth? Arch Dis Child Fetal Neonatal Ed 2006; 91: F263e7. Shenvi A, Kapur J, Rasiah SV. Current practice of the management of asymp- tomatic heart murmurs in neonates in UK. Arch Dis Child Fetal Neonatal Ed 2011; 96: Fa32. http://dx.doi.org/10.1136/archdischild.2011.300164.52. Wren C, Richmond S, Donaldson L. Presentation of congenital heart disease in infancy: implications for routine examination. Arch Dis Child Fetal Neonatal Ed 1999; 80: F49e53. No additional clinical findings or concerns (table 2) Additional clinical signs (especially Sa02 <92%) or concerns (table 2) No additional clinical findings or concerns (table 2) If murmur no longer audible and infant healthy, discharge. If murmur still present +/- symptoms arrange referral If murmur no longer audible, discharge. If still present arrange referral Additional clinical signs or concerns (table 2) Review in 1–2 weeks Seek urgent specialist advice Review in 6 weeks Organise specialist referral Age < 6 weeks Age > 6 weeks Murmur detected (asymptomatic) Figure 1 Flowchart suggesting management strategy following identification of an asymptomatic murmur. SYMPOSIUM: CARDIOVASCULAR PAEDIATRICS AND CHILD HEALTH 23:2 67 Ó 2012 Published by Elsevier Ltd.
  • 5. Practice points C A heart murmur will be heard in most children at some stage, but few will have cardiac pathology. C Murmurs noted in the first weeks/months of life are more likely to be pathological than those first detected in an older child. C Central cyanosis is difficult to detect in children (especially ethnic minorities). C Clinical examination combined with oxygen saturation monitor assessment (where possible) allows effective ‘triaging’ of who needs to be referred urgently. C Chest X-rays and electrocardiograms are of little value in differentiating benign from pathological murmurs. SYMPOSIUM: CARDIOVASCULAR PAEDIATRICS AND CHILD HEALTH 23:2 68 Ó 2012 Published by Elsevier Ltd.