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Volume 84, Number 7 www.aafp.org/afp American Family Physician  793
Evaluation and Management of Heart
Murmurs in Children
JENNIFER E. FRANK, MD, and KATHRYN M. JACOBE, MD, University of Wisconsin Fox Valley Family Medicine
Residency Program, Appleton, Wisconsin
H
eart murmurs are common
in asymptomatic, otherwise
healthy children. These mur-
murs are often innocent and
result from the normal patterns of blood
flow through the heart and vessels.1
How-
ever, a heart murmur may be the sole find-
ing in children with structural heart disease;
therefore, a thorough evaluation is necessary.
Incidence and Prevalence
Congenital heart disease (CHD) may occur
in the presence or absence of a heart mur-
mur. The incidence of CHD varies between
four and 50 per 1,000 live births.2
One review
found an incidence of 75 cases per 1,000 live
births; of these, six cases per 1,000 were
moderate or severe.3
History
Certain historical features suggest possible
structural heart disease (Table 1).1,2,4-11
Car-
diovascular signs and symptoms can be non-
specific (e.g., poor feeding, failure to thrive)
or specific (e.g., chest pain, palpitations),
and can help identify children who are likely
to have structural heart disease (Table 2).4,7,10
In infants, feeding difficulties may be the
first sign of congestive heart failure, which is
present in approximately one-third of infants
and children with CHD.4
The most common
symptoms in a series of children presenting
to the emergency department with acute
heart failure included dyspnea (74 percent),
nausea and vomiting (60 percent), fatigue (56
percent), and cough (40 percent).12
Exercise tolerance should be assessed in an
age-appropriate fashion. Parents of infants
should be asked about their child’s ability to
play and the duration and vigor of feeding;
parents of older children should compare
their child’s ability to participate in team
sports with that of peers.4
Chest pain is rarely
a presenting symptom of cardiac disease in
children.13,14
In a pediatric cardiology clinic,
chest pain or syncope prompted consulta-
tion in approximately 10 percent of children;
only 11 percent of those with chest pain and
5 percent of those with syncope had cardiac
disease.14
A high degree of suspicion is
Heart murmurs are common in healthy infants, children, and adolescents. Although most are not pathologic, a mur-
mur may be the sole manifestation of serious heart disease. Historical elements that suggest pathology include fam-
ily history of sudden cardiac death or congenital heart disease, in utero exposure to certain medications or alcohol,
maternal diabetes mellitus, history of rheumatic fever or Kawasaki disease, and certain genetic disorders. Physical
examination should focus on vital signs; age-appropriate exercise capacity; respiratory or gastrointestinal manifes-
tations of congestive heart failure; and a thorough cardiovascular examination, including features of the murmur,
assessment of peripheral perfusion, and auscultation over the heart valves. Red flags that increase the likelihood of a
pathologic murmur include a holosystolic or diastolic murmur, grade 3 or higher murmur, harsh quality, an abnormal
S2
, maximal murmur intensity at the upper left sternal border, a systolic click, or increased intensity when the patient
stands. Electrocardiography and chest radiography rarely assist in the diagnosis. Referral to a pediatric cardiologist
is recommended for patients with any other abnormal physical examination findings, a history of conditions that
increase the likelihood of structural heart disease, symptoms suggesting underlying cardiac disease, or when a specific
innocent murmur cannot be identified by the family physician. Echocardiography provides a definitive diagnosis and
is recommended for evaluation of any potentially pathologic murmur, and for evaluation of neonatal heart murmurs
because these are more likely to be manifestations of structural heart disease. (Am Fam Physician. 2011;84(7):793-
800. Copyright © 2011 American Academy of Family Physicians.)
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Heart Murmurs in Children
794  American Family Physician www.aafp.org/afp	 Volume 84, Number 7 ◆
October 1, 2011
necessary to detect underlying cardiac disease in children
who report exertional syncope or chest pain, or who have
a family history of hypertrophic cardiomyopathy.1,13,14
Physical Examination
The patient’s vital signs should be compared with age-
established norms (available at http://www.cc.nih.gov/
ccc/pedweb/pedsstaff/age.html), and a focused exami-
nation of the respiratory, cardiovascular, and gastroin-
testinal systems should be performed5
(Table 32,5-7,10,15,16
).
Congenital anomalies of other organ systems may be
associated with CHD in up to 25 percent of patients.6
The
child’s appearance, activity level, color, and respiratory
effort should be assessed, and the neck should be exam-
ined for prominent vessels, abnormal pulsations, and
bruits.1
Jugular venous distension is rare in children.4
The chest wall should be inspected for abnormalities of
the sternum, which can be associated with CHD,15
and
for abnormal cardiac impulses or thrills.1
The lungs
should be auscultated for abnormal breath sounds such as
crackles, which may indicate pulmonary congestion, or
wheezing, which may indicate cardiac asthma. Abdom-
inal examination should focus on the liver location
(seeking abdominal situs) and evaluation for liver
Table 1. Historical Findings Suggesting Structural Heart Disease in Children with Heart Murmurs
Historical finding Significance
Family history
CHD More common in children with a first-degree relative who has CHD (three- to 10-fold increased
risk7
); high penetrance with ventricular septal defect and mitral valve prolapse
Sudden cardiac death or hypertrophic
cardiomyopathy
Increased risk of hypertrophic cardiomyopathy (autosomal dominant pattern)
Sudden infant death syndrome Can be secondary to undiagnosed CHD lesions8
Personal history
Conditions that may coexist with CHD:
Aneuploidy (e.g., trisomy 21, Turner
syndrome)
Connective tissue disorder (e.g.,
Marfan syndrome)
Inborn error of metabolism
Major congenital defects of other
organ systems
Syndrome with dysmorphic features
Certain genetic disorders (e.g., DiGeorge syndrome, velo-cardio-facial syndrome) are associated
with cardiac malformations
Trisomy 21 is associated with an increased risk of atrioventricular septal defects, atrial septal
defects, ventricular septal defects, patent ductus arteriosus, and tetralogy of Fallot
Turner syndrome is associated with increased risk of coarctation of the aorta, aortic valve
stenosis, and left ventricular hypertrophy
Marfan syndrome is associated with mitral valve prolapse, aortic root dilation, and aortic
insufficiency
Frequent respiratory infections Respiratory symptoms may be attributable to heart disease (i.e., congestive heart failure);
enlarged vessels may lead to atelectasis or difficulty clearing respiratory secretions, thereby
promoting infection
Kawasaki disease Leading cause of acquired cardiac disease in children; can cause coronary artery aneurysm and
stenosis9
Rheumatic fever Associated with development of rheumatic heart disease
Prenatal or perinatal history
In utero exposure to alcohol or other
toxins
Fetal alcohol syndrome is associated with an increased risk of atrial and ventricular septal
defects, and tetralogy of Fallot10
In utero exposure to selective
serotonin reuptake inhibitors
or other potentially teratogenic
medications
Selective serotonin reuptake inhibitor exposure is associated with a small but statistically
significant increased risk of mild heart lesions, including ventricular septal defects and bicuspid
aortic valve (although not all studies found an increased risk11
)
Lithium exposure is associated with Ebstein anomaly of the tricuspid valve
Valproate (Depacon) exposure is associated with coarctation of the aorta and hypoplastic left
heart syndrome
Intrauterine infection Maternal infections may increase risk of structural heart lesions (e.g., maternal rubella infection is
associated with patent ductus arteriosus and peripheral pulmonary stenosis)
Maternal diabetes mellitus Increased risk of CHD, including transient hypertrophic cardiomyopathy, tetralogy of Fallot,
truncus arteriosus, and double-outlet right ventricle
Preterm delivery CHD is associated with other conditions (e.g., genetic disorders, in utero exposure to toxins) that
can result in preterm birth; 50 percent of newborns weighing less than 3 lb, 5 oz (1,500 g) at
birth have CHD (most commonly patent ductus arteriosus)7
CHD = congenital heart disease.
Information from references 1, 2, and 4 through 11.
Heart Murmurs in Children
October 1, 2011 ◆
Volume 84, Number 7 www.aafp.org/afp American Family Physician  795
enlargement or ascites, which may signal congestive heart
failure.5
The peripheral pulses should be examined for rate,
rhythm, volume, and character, and capillary refill time
should be less than three seconds.4
The heart should be
auscultated over the tricuspid, pulmonary, mitral, and
aortic areas with the bell and diaphragm of the stetho-
scope while the patient is supine, sitting, and standing17
(Figure 118
).Innocent murmurs are produced by the nor-
mal flow of blood through the heart. Changing the flow by
changing the patient’s position (for example, decreasing
flow to the heart with the Valsalva maneuver) will change
the intensity of the murmur. Young children should be
prompted to push out their abdomen against the exam-
iner’s hand.1
The physician should listen for normal S1
and
S2
; a wide fixed split S2
is characteristic of an atrial septal
defect.19
Gallops can be a normal finding in adolescents.1
Table 2. Symptoms Suggesting Cardiac Disease
Symptom or sign Significance
Cardiovascular
Chest pain May be related to aortic stenosis or
hypertrophic cardiomyopathy
Cyanosis Structural heart lesion with restricted
pulmonary blood flow
Dizziness Multiple potential causes, including
hypoxia and CHF
Near-syncope or
syncope
May be related to aortic stenosis or
hypertrophic cardiomyopathy
Palpitations May be related to arrhythmias secondary
to structural heart lesions
Constitutional
Developmental
delay
Congenital heart lesions are more common
in children with certain genetic disorders
and syndromes
Diaphoresis May indicate CHF or poor cardiac fitness
Easily fatigued May indicate CHF, hypoxia, or poor cardiac
fitness
Poor exercise
tolerance or
capacity for play
May indicate CHF, hypoxia, or poor cardiac
fitness
Poor growth or
failure to thrive
May indicate CHF, poor cardiac fitness, or
a genetic disorder or syndrome; poor
weight gain most commonly reflects
decreased cardiac output or left-to-right
shunts with pulmonary hypertension
Respiratory
Asthma-like
symptoms
Cardiac asthma resulting from pulmonary
congestion
Chronic cough Atelectasis or difficulty clearing secretions
because of pulmonary vascular congestion
Dyspnea on
exertion
May indicate CHF, hypoxia, or poor cardiac
fitness
CHF = congestive heart failure.
Information from references 4, 7, and 10.
Table 3. Physical Examination of Children  
with Heart Murmurs
Finding Significance
Abnormal growth
(height and weight
plotted on growth
chart)
Feeding difficulties may be a sign of
cardiac disease in newborns and
infants (decreased exercise capacity)
Certain genetic disorders may
increase risk of delayed growth
and CHD
Abnormal vital signs
(compared with age-
adjusted norms)
Arrhythmia, tachycardia, hypoxia,
and tachypnea may indicate
underlying structural heart disease
Blood pressure discrepancy between
upper and lower limbs may
indicate coarctation of the aorta
(pressure gradient of  20 mm Hg
with low blood pressure in the
lower extremities)
Adventitial breath
sounds (e.g.,
wheezing, rales,
ronchi, pleural rub)
Wheezing may be associated
with cardiac asthma; rales may
be associated with pulmonary
congestion secondary to
congestive heart failure
Chest contour signaling
maldevelopment of
the sternum15
Defective segmentation of the
sternum may occur in children
with CHD
Dysmorphic features Certain genetic or congenital
conditions increase risk of CHD
Cardiovascular findings
Abnormal S2
Classic finding of wide split fixed
S2
with atrial septal defects;
abnormal S2
may be present in
other types of CHD
Capillary refill Normal peripheral perfusion is less
than 2 to 3 seconds; delay may
indicate poor perfusion secondary
to diminished cardiac output
Displaced point of
maximal impulse;
precordial impulses
(heaves, lifts, thrills)
Possible structural abnormality or
ventricular enlargement  
Edema Congestive heart failure
Left-sided precordial
bulge
Cardiac enlargement
S3
or S4
Can indicate structural heart disease;
S3
can be a normal finding but
usually disappears when patient
is upright
Substernal heave Right ventricular hypertension
Systolic ejection click Semilunar valvular stenosis
Weak or absent
femoral pulses
Coarctation of the aorta
Gastrointestinal findings
Ascites Congestive heart failure
Hepatomegaly Congestive heart failure
Location of liver signals
abdominal situs
High rate of CHD with abdominal
situs
CHD = congenital heart disease.
Information from references 2, 5 through 7, 10, 15, and 16.
Heart Murmurs in Children
796  American Family Physician www.aafp.org/afp	 Volume 84, Number 7 ◆
October 1, 2011
The heart murmur is characterized by its timing dur-
ing the cardiac cycle; its location, quality, intensity, and
pitch (how it sounds); and the presence or absence of
clicks1
(Table 4 5,7,17
and Table 5 20-23
). The intensity of heart
murmurs is graded from 1 to 6. Grade 1 murmurs are
barely audible; grade 2 murmurs are faint but can be
heard immediately; grade 3 murmurs can be heard easily
and are moderately loud; grade 4 murmurs can be heard
easily over a wide area but do not have a palpable thrill;
grade 5 murmurs are loud and have a precordial thrill;
and grade 6 murmurs are loud enough to hear with the
stethoscope raised off the chest.17,24
Certain characteristics
of the murmur may be considered red flags, prompting
stronger consideration for structural heart disease. These
include a holosystolic murmur (odds ratio [OR] of patho-
logic murmur = 54), grade 3 or higher (OR = 4.8), harsh
quality (OR = 2.4), an abnormal S2
(OR = 4.1), maximal
intensity at the upper left sternal border (OR = 4.2), a
systolic click (OR = 8.3), diastolic murmur, or increased
murmur intensity with standing.6,10,25
A decrease or lack
Table 4. Characteristics of Innocent Heart Murmurs
Type Description Age at detection Can sound like
Aortic systolic
murmur
Systolic ejection murmur best heard over the aortic valve Older childhood
into adulthood
—
Mammary artery
soufflé*
High-pitched systolic murmur that can extend into diastole;
best heard along the anterior chest wall over the breast
Rare in adolescence Arteriovenous anastomoses
or patent ductus arteriosus
Peripheral
pulmonary
stenosis
Grade 1 or 2, low-pitched, early- to mid-systolic ejection
murmur heard over axilla or back
 1 year Pulmonary artery stenosis or
normal breath sounds
Pulmonary flow
murmur
Grade 2 or 3, crescendo-decrescendo, early- to mid-systolic
murmur peaking in mid-systole; best heard at the left
sternal border between the second and third intercostal
spaces; characterized by a rough, dissonant quality;
loudest when patient is supine and decreases when
patient is upright and holding breath
All Atrial septal defect or
pulmonary valve stenosis
Still murmur Grade 1 to 3, early systolic murmur; low to medium pitch
with a vibratory or musical quality; best heard at lower
left sternal border; loudest when patient is supine and
decreases when patient stands
Infancy to
adolescence,
often 2 to 6 years
Ventricular septal defect
or hypertrophic
cardiomyopathy
Supraclavicular/
brachiocephalic
systolic murmur
Brief, low-pitched, crescendo-decrescendo murmur heard in
the first two-thirds of systole; best heard above clavicles;
radiates to neck; diminishes when patient hyperextends
shoulders
Childhood to
young adulthood
Bicuspid/stenotic aortic
valve, pulmonary valve
stenosis, or coarctation of
the aorta
Venous hum Grade 1 to 6 continuous murmur; accentuated in diastole;
has a whining, roaring, or whirring quality; best heard
over low anterior neck, lateral to the sternocleinomastoid;
louder on right; resolves or changes when patient is supine
3 to 8 years Cervical arteriovenous
fistulas or patent ductus
arteriosus
*—Mammary artery soufflé murmur is caused by blood flow in the arteries and veins leading to and from the breasts.
Information from references 5, 7, and 17.
URSB ULSB
LLSB
Apex
Figure 1. Listening areas for clicks: upper right sternal bor-
der (URSB) for aortic valve clicks; upper left sternal bor-
der (ULSB) for pulmonary valve clicks; lower left sternal
border (LLSB), or the tricuspid area, for ventricular septal
defects; and the apex for aortic or mitral valve clicks.
Reprinted with permission from McConnell ME, Adkins SB III, Hannon DW.
Heart murmurs in pediatric patients: when do you refer? Am Fam Physician.
1999;60(2):560.
ILLUSTRATIONBYC.BOYTER
October 1, 2011 ◆
Volume 84, Number 7 www.aafp.org/afp American Family Physician  797
Table 5. Prevalence and Characteristics of Pathologic Heart Murmurs
Type of
structural
heart lesion
Prevalence among
children with
congenital heart
disease (%) Symptoms and clinical course Characteristics
Ventricular
septal defect
20 to 25 Small defects: usually asymptomatic
Medium or large defects: CHF,
symptoms of bronchial obstruction,
frequent respiratory infections
Small defects: loud holosystolic murmur at LLSB
(may not last throughout systole if defect is very
small)
Medium and large defects: increased right-to-left
ventricular impulses; thrill at LLSB; split or loud
single S2
; holosystolic murmur at LLSB without
radiation; grade 2 to 5; may also hear a grade 1
or 2 mid-diastolic rumble
Atrial septal
defect
8 to 13 Usually asymptomatic and incidentally
found on physical examination or
echocardiography; large defects can
be present in infants with CHF
Grade 2 or 3 systolic ejection murmur best heard at
ULSB; wide split fixed S2
; absent thrill; may have a
grade 1 or 2 diastolic flow rumble at LLSB
Patent ductus
arteriosus
6 to 11 May be asymptomatic; can cause
easy fatigue, CHF, and respiratory
symptoms
Continuous murmur (grade 1 to 5) in ULSB
(crescendo in systole and decrescendo into
diastole); normal S1
; S2
may be “buried” in the
murmur; thrill or hyperdynamic left ventricular
impulse may be present
Tetralogy of
Fallot
10 Onset depends on severity of
pulmonary stenosis; cyanosis may
appear in infancy (2 to 6 months of
age) or in childhood; other symptoms
include hypercyanotic spells or
decreased exercise tolerance
Central cyanosis; clubbing of nail beds; grade 3
or 4 long systolic ejection murmur heard at ULSB;
may have holosystolic murmur at LLSB; systolic
thrill at ULSB; normal to slightly increased S1
;
single S2
Pulmonary
stenosis
7.5 to 9 Usually asymptomatic but may have
symptoms secondary to pulmonary
congestion
Systolic ejection murmur (grade 2 to 5); heard
best at ULSB radiating to infraclavicular regions,
axillae, and back; normal or loud S1
; variable S2
;
systolic ejection click may be heard at left sternal
border and may vary with respiration
Coarctation of
the aorta
5.1 to 8.1 Newborns and infants may present
with CHF; older children are usually
asymptomatic or may have leg pain
or weakness
Systolic ejection murmur best heard over
interscapular region; normal S1
and S2
; decreased
or delayed femoral pulse; may have increased left
ventricular impulse
Aortic stenosis 5 to 6 Usually asymptomatic; symptoms may
include dyspnea, easy fatigue, chest
pain, or syncope; newborns and
infants may present with CHF
Systolic ejection murmur (grade 2 to 5) best heard
at upper right sternal border with radiation to
carotid arteries; left ventricular heave; thrill at
ULSB or suprasternal notch
Transposition
of the great
arteries
5 Variable presentation depending on
type; may include cyanosis or CHF in
first week of life
Cyanosis; clubbing of nail beds; single S2
; murmur
may be absent or grade 1 or 2 nonspecific systolic
ejection murmur; may have a grade 3 or 4
holosystolic murmur at LLSB and mid-diastolic
murmur at apex
Total anomalous
pulmonary
venous
connection
2 to 3 Onset of CHF at 4 to 6 weeks of age Grade 2 or 3 systolic ejection murmur at ULSB;
grade 1 or 2 mid-diastolic flow rumble at LLSB;
wide split fixed S2
Tricuspid atresia 1.4 Early-onset cyanosis or CHF within the
first month of life
Cyanosis; clubbing of nail beds; normal pulses;
single S2
; holosystolic murmur at LLSB or
midsternal border; murmur may be absent; mid-
diastolic flow murmur at apex may be present
Hypoplastic
left heart
syndrome
Rare May be asymptomatic at birth, with
cyanosis and CHF developing with
duct closure
Hyperdynamic precordium; single S2
; nonspecific
grade 1 or 2 systolic ejection murmur along left
sternal border
Truncus
arteriosus
Rare Onset of CHF in first few weeks of life;
minimal cyanosis
Increased cardiac impulses; holosystolic murmur
(ventricular septal defect); mid-diastolic rumble
CHF = congestive heart failure; LLSB = lower left sternal border; ULSB = upper left sternal border.
Information from references 20 through 23.
Heart Murmurs in Children
798  American Family Physician www.aafp.org/afp	 Volume 84, Number 7 ◆
October 1, 2011
of change in the murmur intensity with passive leg eleva-
tion (likelihood ratio [LR] = 8.0) or when the child moves
from standing to squatting (LR = 4.5) increases the likeli-
hood of hypertrophic cardiomyopathy.26
Characteristics that are more likely to be associated
with an innocent murmur include a systolic (rather than
diastolic) murmur; soft sound; short duration; musical
or low pitch; varying intensity with phases of respiration
and posture (louder in supine position); and murmurs
that become louder with exercise, anxiety, or fear 17,24
(Table 6 27
). The most common innocent murmur is a
Still murmur, which is characteristically loudest at the
lower left sternal border and has a musical or vibratory
quality that is thought to represent vibrations of the left
outflow tract.1,5
Auscultation may be less accurate in younger patients,
when other signs or symptoms of cardiovascular disease
are present, and when findings on radiography or elec-
trocardiography (ECG) are abnormal.28
Online libraries
of digital heart sounds are available to familiarize physi-
cians with the characteristics of abnormal heart sounds
(Table 7).
Role of Diagnostic Testing
ChestradiographyandECGrarelyassistinthediagnosisof
a heart murmur.5,6,29
Studies in newborns30
and children31
with asymptomatic murmurs have shown that chest radi-
ography does not influence clinical management or assist
with diagnosis. A prospective study of 201 newborns who
were referred to pediatric cardiologists for evaluation of
a heart murmur found that the addition of ECG to clini-
cal assessment did not improve the sensitivity or speci-
ficity of detecting structural heart lesions.32
In a study of
128 infants and children who were evaluated for heart
murmurs, the addition of ECG and chest radiography to
cardiac auscultation was more likely to mislead than assist
the physician in making the correct diagnosis.33
In a study of more than 900 children in a pediatric
cardiology clinic who had innocent-sounding mur-
murs, an abnormal finding from the history, physical
examination, or diagnostic tests (ECG, chest radiog-
raphy, or pulse oximetry) was 67 percent sensitive but
only 38 percent specific for the presence of a structural
heart lesion in infants younger than six weeks, yielding
positive and negative LRs very near 1.0 (i.e., no use-
ful diagnostic information).28
In infants older than six
weeks, sensitivity increased to 100 percent, but specific-
ity decreased to 28 percent (positive LR = 1.6; negative
LR = 0.026). Thus, this information is helpful for rul-
ing out structural causes of an innocent-
sounding murmur in infants and children
older than six weeks, but it is not helpful in
younger infants.
In two separate populations geographi-
cally remote from a pediatric cardiolo-
gist, phonocardiography (i.e., digital heart
sound recordings reviewed by a pediatric
cardiologist) had high sensitivity and speci-
ficity, and good intraobserver agreement
in distinguishing between innocent mur-
murs and murmurs that were potentially
or probably pathologic and that required
echocardiography.34,35
Indications for Referral
In children and adolescents, the diagnosis of
an innocent heart murmur can be made if
four criteria are met: absence of abnormal
Table 6. The Seven S’s: Key Features  
of Innocent Murmurs
Sensitive (changes with child’s position or with respiration)
Short duration (not holosystolic)
Single (no associated clicks or gallops)
Small (murmur limited to a small area and nonradiating)
Soft (low amplitude)
Sweet (not harsh sounding)
Systolic (occurs during and is limited to systole)
Information from reference 27.
Table 7. Online Resources for Pediatric Cardiac Auscultation
The Auscultation Assistant
Web site: http://www.wilkes.med.ucla.edu/inex.htm
Blaufuss Medical Multimedia Laboratories
Web site: http://www.blaufuss.org
Heart Sounds and Murmurs
Web site: http://www.dundee.ac.uk/medther/Cardiology/hsmur.html
Johns Hopkins University Cardiac Auscultatory Recording Database
Web site: http://www.murmurlab.com/card6/ (registration required)
Texas Heart Institute
Web site: http://www.texasheart.org/education/cme/explore/events/
eventdetail_5469.cfm
University of Michigan Heart Sound and Murmur Library
Web site: http://www.med.umich.edu/lrc/psb/heartsounds/index.htm
University of Washington Department of Medicine
Demonstrations: Heart Sounds and Murmurs
Web site: http://depts.washington.edu/physdx/heart/demo.html
Heart Murmurs in Children
October 1, 2011 ◆
Volume 84, Number 7 www.aafp.org/afp American Family Physician  799
physical examination findings (except for
the murmur); a negative review of systems
(i.e., child is asymptomatic); a history that
is negative for features that increase the risk
of structural heart disease; and characteris-
tic auscultatory features of a specific inno-
cent heart murmur.2,5
These criteria are not
appropriate for newborns or infants younger
than one year because these patients have
a higher rate of asymptomatic structural
heart disease.36
When an innocent murmur
cannot be definitively diagnosed, the child
should be referred for echocardiography, to
a pediatric cardiologist, or both.
A study in Oman found that the preva-
lence of abnormal findings on echocar-
diography was not significantly different
between patients referred by pediatric cardi-
ologists and those referred by primary care
physicians.37
However, pediatric cardiolo-
gists more accurately detect structural heart
lesions in newborns and children with heart
murmurs,32,38
and can assist family physicians in the
assessment of a suspicious murmur. For both innocent
and pathologic murmurs, referral to a pediatric cardi-
ologist for confirmation or clarification of the diagnosis
is associated with decreased parental anxiety.39
Neonatal Heart Murmurs
Newborns are at higher risk of having serious struc-
tural heart disease that presents as an asymptomatic
murmur.6,10
Approximately 1 percent of newborns have
a heart murmur, and 31 to 86 percent of these infants
have structural heart disease,40-42
including asymp-
tomatic newborns. Because of the higher likelihood of
structural heart disease in asymptomatic newborns and
young infants with heart murmurs, referral to a pediat-
ric cardiologist and/or for echocardiography is recom-
mended.28,42,43
Even potentially life-threatening heart
defects may not be associated with any initial signs or
symptoms other than a heart murmur.41,42
The reported sensitivity for detection of a patho-
logic heart murmur in newborns ranges from 80.5 to
94.9 percent among pediatric cardiologists, with speci-
ficity ranging from 25 to 92 percent.32,43
These varia-
tions are significant because the lowest specificity
corresponds to positive and negative LRs of 1.1 and
0.7, which are uninformative, and the highest specific-
ity corresponds to positive and negative LRs of 10 and
0.21, which are quite accurate. The ability of a pediatric
cardiologist to accurately identify pathologic murmurs
depends on multiple factors, including his or her con-
fidence in the diagnosis. Echocardiography may not be
required in newborns with a heart murmur if a pedi-
atric cardiologist has diagnosed an innocent murmur
with a high degree of confidence32
; however, it is impor-
tant to consider the relatively high prevalence of struc-
tural heart disease among asymptomatic newborns
with a heart murmur.
The evaluation of newborns for CHD may include
pulse oximetry after 24 hours of life. Clinical exami-
nation of asymptomatic newborns has a sensitivity of
46 percent for detection of CHD; this sensitivity
increases to 77 percent when clinical examination
is combined with pulse oximetry (with a cutoff of
94 percent).44
The Authors
JENNIFER E. FRANK, MD, FAAFP, is in private practice at Theda Care
Physicians in Neenah, Wis. At the time this article was written, she was
an assistant professor of family medicine at the University of Wisconsin
School of Medicine and Public Health, Madison, and a faculty member
at the University of Wisconsin Fox Valley Family Medicine Residency Pro-
gram, Appleton.
KATHRYN M. JACOBE, MD, is a third-year resident at the University of
Wisconsin Fox Valley Family Medicine Residency Program.
Address correspondence to Jennifer E. Frank, MD, FAAFP, 1380 Tul-
lar Rd., Neenah, WI 54956 (e-mail: jennifer.frank@thedacare.org).
Reprints are not available from the authors.
Author disclosure: No relevant financial affiliations to disclose.
SORT: KEY RECOMMENDATIONS FOR PRACTICE
Clinical recommendation
Evidence
rating References
Structural heart disease is more likely when
the murmur is holosystolic, diastolic, grade 3
or higher, or associated with a systolic click;
when it increases in intensity with standing; or
when it has a harsh quality.
C 6, 10, 25
Chest radiography and electrocardiography
rarely assist in the diagnosis of heart murmurs
in children.
B 5, 6, 29-33
Family physicians should order echocardiography
or consider referral to a pediatric cardiologist
for newborns with a heart murmur, even if the
child is asymptomatic, because of the higher
prevalence of structural heart lesions in this
population.
B 28, 43
A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-
quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual
practice, expert opinion, or case series. For information about the SORT evidence
rating system, go to http://www.aafp.org/afpsort.xml.
Heart Murmurs in Children
800  American Family Physician www.aafp.org/afp	 Volume 84, Number 7 ◆
October 1, 2011
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Avaliação e tto de sc em criança

  • 1. October 1, 2011 ◆ Volume 84, Number 7 www.aafp.org/afp American Family Physician  793 Evaluation and Management of Heart Murmurs in Children JENNIFER E. FRANK, MD, and KATHRYN M. JACOBE, MD, University of Wisconsin Fox Valley Family Medicine Residency Program, Appleton, Wisconsin H eart murmurs are common in asymptomatic, otherwise healthy children. These mur- murs are often innocent and result from the normal patterns of blood flow through the heart and vessels.1 How- ever, a heart murmur may be the sole find- ing in children with structural heart disease; therefore, a thorough evaluation is necessary. Incidence and Prevalence Congenital heart disease (CHD) may occur in the presence or absence of a heart mur- mur. The incidence of CHD varies between four and 50 per 1,000 live births.2 One review found an incidence of 75 cases per 1,000 live births; of these, six cases per 1,000 were moderate or severe.3 History Certain historical features suggest possible structural heart disease (Table 1).1,2,4-11 Car- diovascular signs and symptoms can be non- specific (e.g., poor feeding, failure to thrive) or specific (e.g., chest pain, palpitations), and can help identify children who are likely to have structural heart disease (Table 2).4,7,10 In infants, feeding difficulties may be the first sign of congestive heart failure, which is present in approximately one-third of infants and children with CHD.4 The most common symptoms in a series of children presenting to the emergency department with acute heart failure included dyspnea (74 percent), nausea and vomiting (60 percent), fatigue (56 percent), and cough (40 percent).12 Exercise tolerance should be assessed in an age-appropriate fashion. Parents of infants should be asked about their child’s ability to play and the duration and vigor of feeding; parents of older children should compare their child’s ability to participate in team sports with that of peers.4 Chest pain is rarely a presenting symptom of cardiac disease in children.13,14 In a pediatric cardiology clinic, chest pain or syncope prompted consulta- tion in approximately 10 percent of children; only 11 percent of those with chest pain and 5 percent of those with syncope had cardiac disease.14 A high degree of suspicion is Heart murmurs are common in healthy infants, children, and adolescents. Although most are not pathologic, a mur- mur may be the sole manifestation of serious heart disease. Historical elements that suggest pathology include fam- ily history of sudden cardiac death or congenital heart disease, in utero exposure to certain medications or alcohol, maternal diabetes mellitus, history of rheumatic fever or Kawasaki disease, and certain genetic disorders. Physical examination should focus on vital signs; age-appropriate exercise capacity; respiratory or gastrointestinal manifes- tations of congestive heart failure; and a thorough cardiovascular examination, including features of the murmur, assessment of peripheral perfusion, and auscultation over the heart valves. Red flags that increase the likelihood of a pathologic murmur include a holosystolic or diastolic murmur, grade 3 or higher murmur, harsh quality, an abnormal S2 , maximal murmur intensity at the upper left sternal border, a systolic click, or increased intensity when the patient stands. Electrocardiography and chest radiography rarely assist in the diagnosis. Referral to a pediatric cardiologist is recommended for patients with any other abnormal physical examination findings, a history of conditions that increase the likelihood of structural heart disease, symptoms suggesting underlying cardiac disease, or when a specific innocent murmur cannot be identified by the family physician. Echocardiography provides a definitive diagnosis and is recommended for evaluation of any potentially pathologic murmur, and for evaluation of neonatal heart murmurs because these are more likely to be manifestations of structural heart disease. (Am Fam Physician. 2011;84(7):793- 800. Copyright © 2011 American Academy of Family Physicians.) Downloaded from the American Family Physician Web site at www.aafp.org/afp. Copyright © 2011 American Academy of Family Physicians. For the private, noncommer- cial use of one individual user of the Web site. All other rights reserved. Contact copyrights@aafp.org for copyright questions and/or permission requests.
  • 2. Heart Murmurs in Children 794  American Family Physician www.aafp.org/afp Volume 84, Number 7 ◆ October 1, 2011 necessary to detect underlying cardiac disease in children who report exertional syncope or chest pain, or who have a family history of hypertrophic cardiomyopathy.1,13,14 Physical Examination The patient’s vital signs should be compared with age- established norms (available at http://www.cc.nih.gov/ ccc/pedweb/pedsstaff/age.html), and a focused exami- nation of the respiratory, cardiovascular, and gastroin- testinal systems should be performed5 (Table 32,5-7,10,15,16 ). Congenital anomalies of other organ systems may be associated with CHD in up to 25 percent of patients.6 The child’s appearance, activity level, color, and respiratory effort should be assessed, and the neck should be exam- ined for prominent vessels, abnormal pulsations, and bruits.1 Jugular venous distension is rare in children.4 The chest wall should be inspected for abnormalities of the sternum, which can be associated with CHD,15 and for abnormal cardiac impulses or thrills.1 The lungs should be auscultated for abnormal breath sounds such as crackles, which may indicate pulmonary congestion, or wheezing, which may indicate cardiac asthma. Abdom- inal examination should focus on the liver location (seeking abdominal situs) and evaluation for liver Table 1. Historical Findings Suggesting Structural Heart Disease in Children with Heart Murmurs Historical finding Significance Family history CHD More common in children with a first-degree relative who has CHD (three- to 10-fold increased risk7 ); high penetrance with ventricular septal defect and mitral valve prolapse Sudden cardiac death or hypertrophic cardiomyopathy Increased risk of hypertrophic cardiomyopathy (autosomal dominant pattern) Sudden infant death syndrome Can be secondary to undiagnosed CHD lesions8 Personal history Conditions that may coexist with CHD: Aneuploidy (e.g., trisomy 21, Turner syndrome) Connective tissue disorder (e.g., Marfan syndrome) Inborn error of metabolism Major congenital defects of other organ systems Syndrome with dysmorphic features Certain genetic disorders (e.g., DiGeorge syndrome, velo-cardio-facial syndrome) are associated with cardiac malformations Trisomy 21 is associated with an increased risk of atrioventricular septal defects, atrial septal defects, ventricular septal defects, patent ductus arteriosus, and tetralogy of Fallot Turner syndrome is associated with increased risk of coarctation of the aorta, aortic valve stenosis, and left ventricular hypertrophy Marfan syndrome is associated with mitral valve prolapse, aortic root dilation, and aortic insufficiency Frequent respiratory infections Respiratory symptoms may be attributable to heart disease (i.e., congestive heart failure); enlarged vessels may lead to atelectasis or difficulty clearing respiratory secretions, thereby promoting infection Kawasaki disease Leading cause of acquired cardiac disease in children; can cause coronary artery aneurysm and stenosis9 Rheumatic fever Associated with development of rheumatic heart disease Prenatal or perinatal history In utero exposure to alcohol or other toxins Fetal alcohol syndrome is associated with an increased risk of atrial and ventricular septal defects, and tetralogy of Fallot10 In utero exposure to selective serotonin reuptake inhibitors or other potentially teratogenic medications Selective serotonin reuptake inhibitor exposure is associated with a small but statistically significant increased risk of mild heart lesions, including ventricular septal defects and bicuspid aortic valve (although not all studies found an increased risk11 ) Lithium exposure is associated with Ebstein anomaly of the tricuspid valve Valproate (Depacon) exposure is associated with coarctation of the aorta and hypoplastic left heart syndrome Intrauterine infection Maternal infections may increase risk of structural heart lesions (e.g., maternal rubella infection is associated with patent ductus arteriosus and peripheral pulmonary stenosis) Maternal diabetes mellitus Increased risk of CHD, including transient hypertrophic cardiomyopathy, tetralogy of Fallot, truncus arteriosus, and double-outlet right ventricle Preterm delivery CHD is associated with other conditions (e.g., genetic disorders, in utero exposure to toxins) that can result in preterm birth; 50 percent of newborns weighing less than 3 lb, 5 oz (1,500 g) at birth have CHD (most commonly patent ductus arteriosus)7 CHD = congenital heart disease. Information from references 1, 2, and 4 through 11.
  • 3. Heart Murmurs in Children October 1, 2011 ◆ Volume 84, Number 7 www.aafp.org/afp American Family Physician  795 enlargement or ascites, which may signal congestive heart failure.5 The peripheral pulses should be examined for rate, rhythm, volume, and character, and capillary refill time should be less than three seconds.4 The heart should be auscultated over the tricuspid, pulmonary, mitral, and aortic areas with the bell and diaphragm of the stetho- scope while the patient is supine, sitting, and standing17 (Figure 118 ).Innocent murmurs are produced by the nor- mal flow of blood through the heart. Changing the flow by changing the patient’s position (for example, decreasing flow to the heart with the Valsalva maneuver) will change the intensity of the murmur. Young children should be prompted to push out their abdomen against the exam- iner’s hand.1 The physician should listen for normal S1 and S2 ; a wide fixed split S2 is characteristic of an atrial septal defect.19 Gallops can be a normal finding in adolescents.1 Table 2. Symptoms Suggesting Cardiac Disease Symptom or sign Significance Cardiovascular Chest pain May be related to aortic stenosis or hypertrophic cardiomyopathy Cyanosis Structural heart lesion with restricted pulmonary blood flow Dizziness Multiple potential causes, including hypoxia and CHF Near-syncope or syncope May be related to aortic stenosis or hypertrophic cardiomyopathy Palpitations May be related to arrhythmias secondary to structural heart lesions Constitutional Developmental delay Congenital heart lesions are more common in children with certain genetic disorders and syndromes Diaphoresis May indicate CHF or poor cardiac fitness Easily fatigued May indicate CHF, hypoxia, or poor cardiac fitness Poor exercise tolerance or capacity for play May indicate CHF, hypoxia, or poor cardiac fitness Poor growth or failure to thrive May indicate CHF, poor cardiac fitness, or a genetic disorder or syndrome; poor weight gain most commonly reflects decreased cardiac output or left-to-right shunts with pulmonary hypertension Respiratory Asthma-like symptoms Cardiac asthma resulting from pulmonary congestion Chronic cough Atelectasis or difficulty clearing secretions because of pulmonary vascular congestion Dyspnea on exertion May indicate CHF, hypoxia, or poor cardiac fitness CHF = congestive heart failure. Information from references 4, 7, and 10. Table 3. Physical Examination of Children   with Heart Murmurs Finding Significance Abnormal growth (height and weight plotted on growth chart) Feeding difficulties may be a sign of cardiac disease in newborns and infants (decreased exercise capacity) Certain genetic disorders may increase risk of delayed growth and CHD Abnormal vital signs (compared with age- adjusted norms) Arrhythmia, tachycardia, hypoxia, and tachypnea may indicate underlying structural heart disease Blood pressure discrepancy between upper and lower limbs may indicate coarctation of the aorta (pressure gradient of 20 mm Hg with low blood pressure in the lower extremities) Adventitial breath sounds (e.g., wheezing, rales, ronchi, pleural rub) Wheezing may be associated with cardiac asthma; rales may be associated with pulmonary congestion secondary to congestive heart failure Chest contour signaling maldevelopment of the sternum15 Defective segmentation of the sternum may occur in children with CHD Dysmorphic features Certain genetic or congenital conditions increase risk of CHD Cardiovascular findings Abnormal S2 Classic finding of wide split fixed S2 with atrial septal defects; abnormal S2 may be present in other types of CHD Capillary refill Normal peripheral perfusion is less than 2 to 3 seconds; delay may indicate poor perfusion secondary to diminished cardiac output Displaced point of maximal impulse; precordial impulses (heaves, lifts, thrills) Possible structural abnormality or ventricular enlargement   Edema Congestive heart failure Left-sided precordial bulge Cardiac enlargement S3 or S4 Can indicate structural heart disease; S3 can be a normal finding but usually disappears when patient is upright Substernal heave Right ventricular hypertension Systolic ejection click Semilunar valvular stenosis Weak or absent femoral pulses Coarctation of the aorta Gastrointestinal findings Ascites Congestive heart failure Hepatomegaly Congestive heart failure Location of liver signals abdominal situs High rate of CHD with abdominal situs CHD = congenital heart disease. Information from references 2, 5 through 7, 10, 15, and 16.
  • 4. Heart Murmurs in Children 796  American Family Physician www.aafp.org/afp Volume 84, Number 7 ◆ October 1, 2011 The heart murmur is characterized by its timing dur- ing the cardiac cycle; its location, quality, intensity, and pitch (how it sounds); and the presence or absence of clicks1 (Table 4 5,7,17 and Table 5 20-23 ). The intensity of heart murmurs is graded from 1 to 6. Grade 1 murmurs are barely audible; grade 2 murmurs are faint but can be heard immediately; grade 3 murmurs can be heard easily and are moderately loud; grade 4 murmurs can be heard easily over a wide area but do not have a palpable thrill; grade 5 murmurs are loud and have a precordial thrill; and grade 6 murmurs are loud enough to hear with the stethoscope raised off the chest.17,24 Certain characteristics of the murmur may be considered red flags, prompting stronger consideration for structural heart disease. These include a holosystolic murmur (odds ratio [OR] of patho- logic murmur = 54), grade 3 or higher (OR = 4.8), harsh quality (OR = 2.4), an abnormal S2 (OR = 4.1), maximal intensity at the upper left sternal border (OR = 4.2), a systolic click (OR = 8.3), diastolic murmur, or increased murmur intensity with standing.6,10,25 A decrease or lack Table 4. Characteristics of Innocent Heart Murmurs Type Description Age at detection Can sound like Aortic systolic murmur Systolic ejection murmur best heard over the aortic valve Older childhood into adulthood — Mammary artery soufflé* High-pitched systolic murmur that can extend into diastole; best heard along the anterior chest wall over the breast Rare in adolescence Arteriovenous anastomoses or patent ductus arteriosus Peripheral pulmonary stenosis Grade 1 or 2, low-pitched, early- to mid-systolic ejection murmur heard over axilla or back 1 year Pulmonary artery stenosis or normal breath sounds Pulmonary flow murmur Grade 2 or 3, crescendo-decrescendo, early- to mid-systolic murmur peaking in mid-systole; best heard at the left sternal border between the second and third intercostal spaces; characterized by a rough, dissonant quality; loudest when patient is supine and decreases when patient is upright and holding breath All Atrial septal defect or pulmonary valve stenosis Still murmur Grade 1 to 3, early systolic murmur; low to medium pitch with a vibratory or musical quality; best heard at lower left sternal border; loudest when patient is supine and decreases when patient stands Infancy to adolescence, often 2 to 6 years Ventricular septal defect or hypertrophic cardiomyopathy Supraclavicular/ brachiocephalic systolic murmur Brief, low-pitched, crescendo-decrescendo murmur heard in the first two-thirds of systole; best heard above clavicles; radiates to neck; diminishes when patient hyperextends shoulders Childhood to young adulthood Bicuspid/stenotic aortic valve, pulmonary valve stenosis, or coarctation of the aorta Venous hum Grade 1 to 6 continuous murmur; accentuated in diastole; has a whining, roaring, or whirring quality; best heard over low anterior neck, lateral to the sternocleinomastoid; louder on right; resolves or changes when patient is supine 3 to 8 years Cervical arteriovenous fistulas or patent ductus arteriosus *—Mammary artery soufflé murmur is caused by blood flow in the arteries and veins leading to and from the breasts. Information from references 5, 7, and 17. URSB ULSB LLSB Apex Figure 1. Listening areas for clicks: upper right sternal bor- der (URSB) for aortic valve clicks; upper left sternal bor- der (ULSB) for pulmonary valve clicks; lower left sternal border (LLSB), or the tricuspid area, for ventricular septal defects; and the apex for aortic or mitral valve clicks. Reprinted with permission from McConnell ME, Adkins SB III, Hannon DW. Heart murmurs in pediatric patients: when do you refer? Am Fam Physician. 1999;60(2):560. ILLUSTRATIONBYC.BOYTER
  • 5. October 1, 2011 ◆ Volume 84, Number 7 www.aafp.org/afp American Family Physician  797 Table 5. Prevalence and Characteristics of Pathologic Heart Murmurs Type of structural heart lesion Prevalence among children with congenital heart disease (%) Symptoms and clinical course Characteristics Ventricular septal defect 20 to 25 Small defects: usually asymptomatic Medium or large defects: CHF, symptoms of bronchial obstruction, frequent respiratory infections Small defects: loud holosystolic murmur at LLSB (may not last throughout systole if defect is very small) Medium and large defects: increased right-to-left ventricular impulses; thrill at LLSB; split or loud single S2 ; holosystolic murmur at LLSB without radiation; grade 2 to 5; may also hear a grade 1 or 2 mid-diastolic rumble Atrial septal defect 8 to 13 Usually asymptomatic and incidentally found on physical examination or echocardiography; large defects can be present in infants with CHF Grade 2 or 3 systolic ejection murmur best heard at ULSB; wide split fixed S2 ; absent thrill; may have a grade 1 or 2 diastolic flow rumble at LLSB Patent ductus arteriosus 6 to 11 May be asymptomatic; can cause easy fatigue, CHF, and respiratory symptoms Continuous murmur (grade 1 to 5) in ULSB (crescendo in systole and decrescendo into diastole); normal S1 ; S2 may be “buried” in the murmur; thrill or hyperdynamic left ventricular impulse may be present Tetralogy of Fallot 10 Onset depends on severity of pulmonary stenosis; cyanosis may appear in infancy (2 to 6 months of age) or in childhood; other symptoms include hypercyanotic spells or decreased exercise tolerance Central cyanosis; clubbing of nail beds; grade 3 or 4 long systolic ejection murmur heard at ULSB; may have holosystolic murmur at LLSB; systolic thrill at ULSB; normal to slightly increased S1 ; single S2 Pulmonary stenosis 7.5 to 9 Usually asymptomatic but may have symptoms secondary to pulmonary congestion Systolic ejection murmur (grade 2 to 5); heard best at ULSB radiating to infraclavicular regions, axillae, and back; normal or loud S1 ; variable S2 ; systolic ejection click may be heard at left sternal border and may vary with respiration Coarctation of the aorta 5.1 to 8.1 Newborns and infants may present with CHF; older children are usually asymptomatic or may have leg pain or weakness Systolic ejection murmur best heard over interscapular region; normal S1 and S2 ; decreased or delayed femoral pulse; may have increased left ventricular impulse Aortic stenosis 5 to 6 Usually asymptomatic; symptoms may include dyspnea, easy fatigue, chest pain, or syncope; newborns and infants may present with CHF Systolic ejection murmur (grade 2 to 5) best heard at upper right sternal border with radiation to carotid arteries; left ventricular heave; thrill at ULSB or suprasternal notch Transposition of the great arteries 5 Variable presentation depending on type; may include cyanosis or CHF in first week of life Cyanosis; clubbing of nail beds; single S2 ; murmur may be absent or grade 1 or 2 nonspecific systolic ejection murmur; may have a grade 3 or 4 holosystolic murmur at LLSB and mid-diastolic murmur at apex Total anomalous pulmonary venous connection 2 to 3 Onset of CHF at 4 to 6 weeks of age Grade 2 or 3 systolic ejection murmur at ULSB; grade 1 or 2 mid-diastolic flow rumble at LLSB; wide split fixed S2 Tricuspid atresia 1.4 Early-onset cyanosis or CHF within the first month of life Cyanosis; clubbing of nail beds; normal pulses; single S2 ; holosystolic murmur at LLSB or midsternal border; murmur may be absent; mid- diastolic flow murmur at apex may be present Hypoplastic left heart syndrome Rare May be asymptomatic at birth, with cyanosis and CHF developing with duct closure Hyperdynamic precordium; single S2 ; nonspecific grade 1 or 2 systolic ejection murmur along left sternal border Truncus arteriosus Rare Onset of CHF in first few weeks of life; minimal cyanosis Increased cardiac impulses; holosystolic murmur (ventricular septal defect); mid-diastolic rumble CHF = congestive heart failure; LLSB = lower left sternal border; ULSB = upper left sternal border. Information from references 20 through 23.
  • 6. Heart Murmurs in Children 798  American Family Physician www.aafp.org/afp Volume 84, Number 7 ◆ October 1, 2011 of change in the murmur intensity with passive leg eleva- tion (likelihood ratio [LR] = 8.0) or when the child moves from standing to squatting (LR = 4.5) increases the likeli- hood of hypertrophic cardiomyopathy.26 Characteristics that are more likely to be associated with an innocent murmur include a systolic (rather than diastolic) murmur; soft sound; short duration; musical or low pitch; varying intensity with phases of respiration and posture (louder in supine position); and murmurs that become louder with exercise, anxiety, or fear 17,24 (Table 6 27 ). The most common innocent murmur is a Still murmur, which is characteristically loudest at the lower left sternal border and has a musical or vibratory quality that is thought to represent vibrations of the left outflow tract.1,5 Auscultation may be less accurate in younger patients, when other signs or symptoms of cardiovascular disease are present, and when findings on radiography or elec- trocardiography (ECG) are abnormal.28 Online libraries of digital heart sounds are available to familiarize physi- cians with the characteristics of abnormal heart sounds (Table 7). Role of Diagnostic Testing ChestradiographyandECGrarelyassistinthediagnosisof a heart murmur.5,6,29 Studies in newborns30 and children31 with asymptomatic murmurs have shown that chest radi- ography does not influence clinical management or assist with diagnosis. A prospective study of 201 newborns who were referred to pediatric cardiologists for evaluation of a heart murmur found that the addition of ECG to clini- cal assessment did not improve the sensitivity or speci- ficity of detecting structural heart lesions.32 In a study of 128 infants and children who were evaluated for heart murmurs, the addition of ECG and chest radiography to cardiac auscultation was more likely to mislead than assist the physician in making the correct diagnosis.33 In a study of more than 900 children in a pediatric cardiology clinic who had innocent-sounding mur- murs, an abnormal finding from the history, physical examination, or diagnostic tests (ECG, chest radiog- raphy, or pulse oximetry) was 67 percent sensitive but only 38 percent specific for the presence of a structural heart lesion in infants younger than six weeks, yielding positive and negative LRs very near 1.0 (i.e., no use- ful diagnostic information).28 In infants older than six weeks, sensitivity increased to 100 percent, but specific- ity decreased to 28 percent (positive LR = 1.6; negative LR = 0.026). Thus, this information is helpful for rul- ing out structural causes of an innocent- sounding murmur in infants and children older than six weeks, but it is not helpful in younger infants. In two separate populations geographi- cally remote from a pediatric cardiolo- gist, phonocardiography (i.e., digital heart sound recordings reviewed by a pediatric cardiologist) had high sensitivity and speci- ficity, and good intraobserver agreement in distinguishing between innocent mur- murs and murmurs that were potentially or probably pathologic and that required echocardiography.34,35 Indications for Referral In children and adolescents, the diagnosis of an innocent heart murmur can be made if four criteria are met: absence of abnormal Table 6. The Seven S’s: Key Features   of Innocent Murmurs Sensitive (changes with child’s position or with respiration) Short duration (not holosystolic) Single (no associated clicks or gallops) Small (murmur limited to a small area and nonradiating) Soft (low amplitude) Sweet (not harsh sounding) Systolic (occurs during and is limited to systole) Information from reference 27. Table 7. Online Resources for Pediatric Cardiac Auscultation The Auscultation Assistant Web site: http://www.wilkes.med.ucla.edu/inex.htm Blaufuss Medical Multimedia Laboratories Web site: http://www.blaufuss.org Heart Sounds and Murmurs Web site: http://www.dundee.ac.uk/medther/Cardiology/hsmur.html Johns Hopkins University Cardiac Auscultatory Recording Database Web site: http://www.murmurlab.com/card6/ (registration required) Texas Heart Institute Web site: http://www.texasheart.org/education/cme/explore/events/ eventdetail_5469.cfm University of Michigan Heart Sound and Murmur Library Web site: http://www.med.umich.edu/lrc/psb/heartsounds/index.htm University of Washington Department of Medicine Demonstrations: Heart Sounds and Murmurs Web site: http://depts.washington.edu/physdx/heart/demo.html
  • 7. Heart Murmurs in Children October 1, 2011 ◆ Volume 84, Number 7 www.aafp.org/afp American Family Physician  799 physical examination findings (except for the murmur); a negative review of systems (i.e., child is asymptomatic); a history that is negative for features that increase the risk of structural heart disease; and characteris- tic auscultatory features of a specific inno- cent heart murmur.2,5 These criteria are not appropriate for newborns or infants younger than one year because these patients have a higher rate of asymptomatic structural heart disease.36 When an innocent murmur cannot be definitively diagnosed, the child should be referred for echocardiography, to a pediatric cardiologist, or both. A study in Oman found that the preva- lence of abnormal findings on echocar- diography was not significantly different between patients referred by pediatric cardi- ologists and those referred by primary care physicians.37 However, pediatric cardiolo- gists more accurately detect structural heart lesions in newborns and children with heart murmurs,32,38 and can assist family physicians in the assessment of a suspicious murmur. For both innocent and pathologic murmurs, referral to a pediatric cardi- ologist for confirmation or clarification of the diagnosis is associated with decreased parental anxiety.39 Neonatal Heart Murmurs Newborns are at higher risk of having serious struc- tural heart disease that presents as an asymptomatic murmur.6,10 Approximately 1 percent of newborns have a heart murmur, and 31 to 86 percent of these infants have structural heart disease,40-42 including asymp- tomatic newborns. Because of the higher likelihood of structural heart disease in asymptomatic newborns and young infants with heart murmurs, referral to a pediat- ric cardiologist and/or for echocardiography is recom- mended.28,42,43 Even potentially life-threatening heart defects may not be associated with any initial signs or symptoms other than a heart murmur.41,42 The reported sensitivity for detection of a patho- logic heart murmur in newborns ranges from 80.5 to 94.9 percent among pediatric cardiologists, with speci- ficity ranging from 25 to 92 percent.32,43 These varia- tions are significant because the lowest specificity corresponds to positive and negative LRs of 1.1 and 0.7, which are uninformative, and the highest specific- ity corresponds to positive and negative LRs of 10 and 0.21, which are quite accurate. The ability of a pediatric cardiologist to accurately identify pathologic murmurs depends on multiple factors, including his or her con- fidence in the diagnosis. Echocardiography may not be required in newborns with a heart murmur if a pedi- atric cardiologist has diagnosed an innocent murmur with a high degree of confidence32 ; however, it is impor- tant to consider the relatively high prevalence of struc- tural heart disease among asymptomatic newborns with a heart murmur. The evaluation of newborns for CHD may include pulse oximetry after 24 hours of life. Clinical exami- nation of asymptomatic newborns has a sensitivity of 46 percent for detection of CHD; this sensitivity increases to 77 percent when clinical examination is combined with pulse oximetry (with a cutoff of 94 percent).44 The Authors JENNIFER E. FRANK, MD, FAAFP, is in private practice at Theda Care Physicians in Neenah, Wis. At the time this article was written, she was an assistant professor of family medicine at the University of Wisconsin School of Medicine and Public Health, Madison, and a faculty member at the University of Wisconsin Fox Valley Family Medicine Residency Pro- gram, Appleton. KATHRYN M. JACOBE, MD, is a third-year resident at the University of Wisconsin Fox Valley Family Medicine Residency Program. Address correspondence to Jennifer E. Frank, MD, FAAFP, 1380 Tul- lar Rd., Neenah, WI 54956 (e-mail: jennifer.frank@thedacare.org). Reprints are not available from the authors. Author disclosure: No relevant financial affiliations to disclose. SORT: KEY RECOMMENDATIONS FOR PRACTICE Clinical recommendation Evidence rating References Structural heart disease is more likely when the murmur is holosystolic, diastolic, grade 3 or higher, or associated with a systolic click; when it increases in intensity with standing; or when it has a harsh quality. C 6, 10, 25 Chest radiography and electrocardiography rarely assist in the diagnosis of heart murmurs in children. B 5, 6, 29-33 Family physicians should order echocardiography or consider referral to a pediatric cardiologist for newborns with a heart murmur, even if the child is asymptomatic, because of the higher prevalence of structural heart lesions in this population. B 28, 43 A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited- quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to http://www.aafp.org/afpsort.xml.
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