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Recurrent chest pain in the well child

A Ives,1 P E F Daubeney,2,3 I M Balfour-Lynn1,3
1Department of Paediatric INTRODUCTION as heart pain. A cardiac cause, although poten-
Respiratory Medicine, Royal Chest pain is a common symptom in paediatric tially serious, is among the least common causes
Brompton Hospital, London,
UK practice and a frequent cause of attendance to of chest pain in an otherwise healthy child.111
2Department of Paediatric general practitioners and hospital outpatients Most children with congenital heart disease do
Cardiology, Royal Brompton departments. Public education campaigns have not have chest pain, and conversely, most chil-
Hospital, London, UK emphasised its potentially serious cause in adults, dren with chest pain do not have congenital or
3NHLI Imperial College London,
namely a heart attack, leading to heightened acquired heart disease. Cardiogenic pain is usu-
London, UK
concern when it occurs in a child.1 2 However, in ally crushing, left-sided, radiating to the left arm,
Correspondence to children it is typically benign and self-limiting, sometimes with paraesthesia, brought on by
Dr Ian M Balfour-Lynn, Royal not usually a manifestation of organic disease, exercise and settling at rest, and associated with
Brompton Hospital, Sydney and rarely cardiac in origin.111 The patients are sweating and nausea. Pericarditic pain differs and
Street, London SW3 6NP, UK;
[email protected] often referred by their general practitioner or local is described below. Cardiac chest pain may be pre-
accident & emergency department to general pae- cipitated by arrhythmia, acquired heart disease
Accepted 8 December 2009 diatricians, respiratory paediatricians or paediat- and underlying congenital defects.9 10
ric cardiologists. Chest pain is in fact the second
most common cause of referral to paediatric cardi- Arrhythmia
ologists in the USA,6 murmurs being the largest.12 Children with arrhythmias will occasionally
The exact cause of chest pain often remains undi- present with chest pain, but normally have tachy-
agnosed13 and is labelled as idiopathic.1 36 9 11 14 cardia or an irregular heart beat by history and/or
There are a number of chronic conditions examination.
known to be associated with recurrent chest pain, Acquired heart disease
for example cystic brosis and sickle cell disease. Acquired lesions can produce chest pain.
This article will not discuss these conditions as the Pericarditis frequently presents with non-specic
underlying diagnosis is usually known. There are chest pain and can follow a viral illness, or be
a number of acute causes of chest pain, for exam- part of rheumatic fever. It is almost always associ-
ple pneumothorax or empyema, that are not usu- ated with fever and other signs of in ammation
ally recurrent and where the child is often unwell, and the children are unwell. The pain is often
so they will also not be discussed. Of course all sharp, typically sternal or substernal and worse
recurrent cases will, at one point, have to present on deep breathing, coughing or leaning forward.10
for the rst time, so acute causes will sometimes Endocarditis, cardiomyopathies, myocarditis and
need to be considered at the initial presentation. rheumatic fever are more likely to present with
This article will highlight the most likely causes other classic symptoms such as fevers or exer-
of recurrent chest pain in a child who is otherwise cise intolerance, but also can be associated with
well, although the full differential diagnosis is chest pain. Acquired coronary artery lesions due
long (table 1). to Kawasaki disease, accelerated atherosclerotic
coronary artery disease and cocaine use,15 can
CAUSES OF RECURRENT CHEST PAIN produce true ischaemic chest pain. Pulmonary
There are a number of causes of recurrent chest hypertension is a rare cause of chest pain; these
pain broadly divided into the following categories: patients normally have fatigue, exercise intoler-
cardiac, respiratory, gastrointestinal, musculoskel- ance, palpitations and/or syncope.
etal, psychological, miscellaneous, and idiopathic
(unknown). The frequency of these causes varies Congenital heart disease
widely between studies.6 Generally, idiopathic The vast majority of structural cardiac abnormali-
pain is the most common cause1 36 9 11 14 followed ties are not associated with chest pain. Patients
by musculoskeletal pain, while cardiac pain is the who have recently undergone cardiac surgery
least common. may have postpericardiotomy syndrome. Lesions
The gender of the child is not usually relevant such as aortic stenosis, that can lead to decreased
to the cause of the pain, 5 although a psychological myocardial perfusion and ischaemia, may cause
cause is more common in girls.4 5 Age does play an exercise-induced chest pain. Other less common
important role chest pain in children <12 years causes include aortic aneurysm with dissection
old is more likely to be due to organic disease, (as seen in aortopathies such as Marfan syndrome,
whereas psychogenic disease is more common in bicuspid aortic valve or coarctation of the aorta),
teenagers.4 5 mitral valve prolapse or congenital coronary artery
abnormalities (such as anomalous origin or course).
Cardiac
Chest pain, particularly originating in the left Respiratory
precordium, is often assumed by the patient to Respiratory pain can originate from the main air-
be cardiac in origin and may even be described ways, parietal pleura, chest wall or diaphragm. It

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Table 1 Causes of chest pain by site of origin Although chest pain can be caused by any site along the gas-
Cardiac Structural lesions
trointestinal tract, 20 oesophagitis (reux or eosinophilic) and
Acquired/inflammatory conditions (eg, vasculitis, gastritis are probably the most common causes.8 14 Pain from
cardiomyopathy) these sites often presents with the classic symptom of heart-
Arrhythmias burn although may be non-specic.14 Oesophageal spasm is
Respiratory Infection particularly associated with chest pain,14 and may be due to
Pleural disease
an oesophageal motor disorder and/or a primary motor dis-
Asthma
Foreign body order. 20 Pain is often associated with posture and eating, and
Pneumothorax, pneumomediastinum is usually felt in the epigastrium, retrosternally, at the chest
Inhalant irritation bases or at the shoulder tip. Pain associated with dysphagia
Dysfunctional breathing (difculty swallowing) is a strong pointer to an oesophageal
Thoracic malignancy
cause. Tenderness in the epigastrium is a particularly good sign
Gastrointestinal Gastro-oesophageal reflux
Oesophagitis (reflux, eosinophilic)
that the pain may be of gastrointestinal origin.8 Abdominal/
Gastritis subphrenic abscesses may cause diaphragmatic irritation with
Oesophageal spasm chest pain that radiates to the shoulder or lower chest,16 but
Achalasia the child is unwell. One of the difculties in diagnosing gas-
Oesophageal foreign body
trointestinal chest pain is that the tests are typically invasive
Pancreatitis
Subdiaphragmatic abscess and disliked by the children.11
Musculoskeletal Slipping rib syndrome
Tietzes syndrome
Costochondritis
Musculoskeletal
Vertebral deformities or collapse Musculoskeletal chest pain tends to be sharp, well localised and
Myofascial trauma or strain brief, lasting seconds to minutes. It is usually exacerbated by
Miscellaneous Precordial catch syndrome movement of the affected part and so is often worse on breath-
Stitch ing or coughing. Localised tenderness is the dominant feature, 21
Breast development
and needs to be distinguished from pleuritis, where there is often
Herpes zoster
signicant muscular irritation and resultant pain. There may be
a history of trauma such as a strain or recent participation in
should be remembered that respiratory pain can occasionally active sports such as swimming, tennis or trampolining.
present as abdominal pain (eg, lower lobe pneumonia), and due Fam and Smythe21 have categorised localised musculoskel-
to phrenic nerve innervation diaphragmatic irritation can pres- etal chest wall pain into four groups (1) arising from ribs
ent as ipsilateral shoulder pain. Major airway pain is usually and articulations, (2) arising from sternum and articulations,
retrosternal, while pleuritic pain is typically sharp and well (3) arising from myofascial structures, and (4) arising from the
localised laterally. Respiratory pain is classically character- thoracic spine, spinal cord and spinal nerves.
ised by pain on coughing and respiratory disease is often sug-
gested by other symptoms such as cough, wheeze and sputum Ribs and articulations
production, and clinical signs such as asymmetrical air entry, Tietzes syndrome. This is due to an isolated painful, swollen
wheeze or crepitations. costochondral junction that the child localises precisely. 22
Asthma and exercise-induced bronchospasm are common The second and third junctions are more commonly affected,
causes of respiratory pain, although it is more often described but lower ribs can be also affected in children.22 The affected
as chest tightness or discomfort.13 16 17 Asthma-associated chest area is tender and swollen but not hot.21 The cause is usu-
pain is usually benign and may be due to dyspnoea, hyperin- ally unknown but may be related to trauma, a history of
ation, cough or muscle strain; rarely it is secondary to more violent coughing or an upper respiratory tract infection. 21 22
serious acute causes such as a pneumothorax or pneumome- Investigations (including blood in ammatory markers) tend
diastinum.16 Children with exercise-induced bronchospasm to be normal, but it is important to exclude septic arthritis,
may have no wheeze, even if they have signicant shortness of osteomyelitis or malignancy (although in these cases the child
breath or chest pain,13 and a prevalence of 1020% in athletes will be unwell). 22 Chest radiographs and bone scans tend to be
has been suggested.18 normal, 22 although a chest CT scan may show osteochondri-
Infectious causes such as tracheitis, pneumonia, pleuritis or tis of the rib. It can usually be diagnosed on clinical grounds
parapneumonic effusion/empyema are also common causes alone however. It tends to run a self-limiting course, typically
of respiratory chest pain. More rarely pleurodynia (pleuritic resolving within a few weeks or months. 21 22 Treatment is
pain without an effusion) due to coxsackie virus (Bornholms anti-in ammatory medication or occasionally an intercostal
disease) is described.16 19 However, in these cases the children block if the pain is severe. 21 22
are clearly unwell. Costochondritis. This differs from Tietzes syndrome in that it
is often at multiple sites and there is no swelling. 21 The pain
Gastrointestinal usually involves the costochondral or costosternal areas of the
Gastrointestinal causes of chest pain are relatively infrequently second to fth costal cartilages. 3 21 Its aetiology is unknown
diagnosed,1 5 9 14 however are often confused with pain origi- but it may be viral or trauma related. 3 21 The condition is self-
nating in other sites. Chest pain and abdominal pain are easily limiting but can last for many months. 3 Treatment is rest and
confused in smaller children, and even in adults cardiac pain non-steroidal anti-in ammatory drugs. 3 21
and oesophageal pain can be difcult to distinguish as they Rib trauma. The history is obvious and it is more common in
have very similar autonomic nerve pathways.14 20 Over half boys.
of adults with angina-like pain and normal coronary arterio- Slipping rib syndrome. The 8th to 10th ribs do not attach to
grams are found to have oesophageal disorders.14 20 the sternum directly but to each other via brous tissue,

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allowing mobility but at the cost of a susceptibility to trauma.23 competition, which can lead to psychogenic pain, perhaps as
Inadequacy or rupture of the brous attachments due to direct an excuse for being able to stop the sport. Our experience is
trauma, or indirectly due to lifting can allow the costal cartilage the child is often accompanied by one or both parents who
tips to curl upwards and press on the intercostal nerves.21 23 24 have strongly encouraged the child in the sport and who insist
The pain can last several months,21 and is often worse in situa- how much the child loves taking part.
tions that cause forceful upward movement of the costal carti- This kind of pain is often eeting or vague, or localised
lages, such as horse riding; it is also worsened by exing of the over the heart and/or left arm. It does not generally get worse
trunk, heavy lifting and even stretching or coughing.23 The pain on exercise and may occur at rest. Often the parents may
can mimic biliary or renal colic.24 The main diagnostic test is the be extremely anxious while the child shows a classic belle
hooking manoeuvre the drawing forward of the lowest costal indiffrence seen in many psychosomatic disorders. The
cartilages, which reproduces the symptoms and gives a clicking physical examination is usually normal.
sensation.21 24 A chest radiograph is of no value except to rule out Dysfunctional breathing. A very common form of psychogenic
other diagnoses.23 Treatment is analgesia and sometimes local pain, again particularly common in adolescents, 28 is dysfunc-
anaesthetic in ltration, or in extreme situations surgical resec- tional breathing or hyperventilation. It has been suggested
tion of the affected cartilage.21 23 24 that hyperventilation can cause up to 20% of all chest pain in
adolescent patients. 28 While the origin of the breathing dys-
Sternum function is psychological, it can cause very real physical pain.
Sternoclavicular joint pain is worse on shoulder shrugging. A resultant hypocapnoeic alkalosis can cause coronary artery
Pain and tenderness can also originate from the manubrioster- vasoconstriction, albeit only after up to 30 min of deep breath-
nal joint or the xiphisternal joint and xiphoid cartilage. ing.1 29 Deep breathing can also cause stomach distension due
Myofascial to aerophagia, spasm of the left hemidiaphragm and transient
Pain from the intercostal and thoracic muscles is usually trau- arrhythmias.1 Experienced physiotherapists can often make
matic in origin. 21 The muscles may be strained during active the diagnosis and after retraining the child to breathe prop-
sports. It presents as localised pain and tenderness over the erly, the symptoms usually disappear. The children often have
affected muscles, and is worsened by muscle movement. poor posture accompanying the problem which also needs to
Myositis due to bacterial and viral agents, such as Bornholms be corrected.
disease,19 causes severe, sharp pains in the upper abdomen
(more common in children)21 or lateral chest wall, with muscu- Miscellaneous
lar tenderness.19 It tends to be short lived, lasting 37 days. 21 Breast development
Spine Breast growth during puberty can lead to breast pain in girls.
Causes include trauma, tumours, infection, thoracic disc dis- Trauma and mastitis may also lead to localised pain.16 Boys
ease and arthritis. are not immune to pain from this gynaecomastia may cause
discomfort, even when something as minor as clothing rub-
bing over the skin occurs.16
Psychogenic
Psychogenic chest pain is a diagnosis of exclusion, and is not Herpes zoster
necessarily the same as idiopathic pain. It is seen in all ages, Shingles can cause an intercostal neuralgia with a severe
but has a higher incidence in teenagers,4 5 particularly girls.4 5 25 sharp, stabbing pain along the area of the nerve, as well as loc-
Chronic pain of more than 6 months duration is more likely alised tenderness. The pain is often worse on movement, deep
to be due to psychological problems. 5 Although there is usu- breathing and exposure to cold.16 The pain tends to resolve
ally no organic cause for the pain, patients often have a sig- when the vesicles begin to heal, but occasionally post-herpetic
nicant degree of functional impairment, 26 and for them the neuralgia may require regional nerve blocks or even surgical
pain can be very real.16 27 In fact some psychogenic causes can intervention.16 The child is unwell and the rash obvious.
cause genuine physical pain, and vice versa the child with
recent organic disease is at increased risk for stress-related Precordial catch
psychological pain.16 Psychological and organic aetiologies This is a self-limiting, well-localised, very brief, sharp pain
may not always be mutually exclusive.16 Some patients may in young healthy individuals sometimes called Texidors
have minor physiological symptoms but these are made worse twinge. 21 30 It is usually felt in the precordial area but can also
by the belief that they are evidence of severe physical illness. occur under the left breast or by the left sternal border, or other
When a group of adolescents was asked what they were afraid sites. 21 30 It is most common in those aged 612 years old. 31
of with regard to the cause of their pain, 56% said that they The origin of the pain is uncertain, but it may come from the
feared heart disease and 12% were worried about cancer; 70% parietal pleura (the visceral pleura has no pain receptors) or
of this same group believed that people of their ages could be due to a muscular spasm. 21 It is certainly not of cardiac or
have heart attacks.1 pericardial origin. 31 The pain can occur at rest or on exercise,
Psychogenic pain is much more likely where there is a family and generally lasts from 30 s to 3 min it rarely lasts for lon-
history of chest pain or heart disease 5 7 a model or example ger than 1 min. 21 30 31 The pain does not radiate and is usually
in the family. 27 Studies have shown that there is often a his- made substantially worse on deep inspiration. 31 The descrip-
tory of other psychological disorders such as panic disorder, tion of this pain is usually so classic that it can be diagnosed
anxiety or depression.9 16 26 Emotional stress is very com- on history alone. There is no local tenderness and the physical
mon; adverse life events such as changes in the family struc- examination is completely normal. No investigations are nec-
ture (births, deaths, divorce, etc), school problems and family essary. There is normally a sudden and complete resolution of
physical or psychological illness can often be identied as the the pain but the pain can sometimes be helped by a change
stressor.7 27 A not infrequent situation is the keen sportsperson of posture or even forced deep inspiration. 21 30 31 Usually,
who has realised they are simply not good enough for top-level however, no treatment is required. 31

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Stitch 2 days to 1 month.4 A typical presentation is of a child who

A stitch is classically a pain in the lower chest or side of the complains of pain that has lasted (intermittently) for weeks or
abdomen occurring during exercise, that is worse after eat- months but who attends the clinic looking completely well.
ing or drinking. Its cause is uncertain but may be due to an Chest pain that wakes children from sleep should always be
engorged gut pulling on visceral ligaments where they attach taken seriously as it suggests organic pain (but not necessarily
to the diaphragm, or ischaemic pain in the diaphragm due to a serious cause). 5 A history of fever, weight loss or fatigue is
blood shunting to the gut and limb muscles during exercise. 32 also a key pointer to potentially serious pathology.
Treatment involves delaying exercise for 23 h after eating, Patients often have a history of asthma or a previously diag-
the wearing of a light, wide belt around the waist and the tak- nosed heart murmur.4 Similarly a family history of premature
ing of small amounts of uid regularly while exercising, bend- ischaemic heart disease, sudden death, arrhythmia and car-
ing forward to tighten the abdominal muscles, and breathing diomyopathy should be sought, particularly as patients and
through pursed lips. 32 families often worry most about cardiac pain, and it is worth
nding out what the child and parents are most concerned
MAKING THE DIAGNOSIS about. The social history may reveal psychological and emo-
In keeping with the classical mantra taught at medical school, tional factors although these may not be revealed during the
the most useful diagnostic tools are a careful history and exami- initial consultation; they can of course still occur in the pres-
nation.7 11 21 In many cases this is all that is required to identify ence of organic disease. A drug history is important as some
the cause of the pain, removing the need for investigations. medications can cause gastric irritation 6 or be associated with
embolic disease (eg, the oral contraceptive pill).
HISTORY
This is the most important part of making the diagnosis as there EXAMINATION
are often few physical ndings and investigations are usually There may be few physical ndings16 and the physical exami-
normal.26 9 11 The pain can occur as an isolated symptom or nation is normal in 3760% of cases in different series.1 5 9 11
in association with other symptoms, depending on the cause Despite this, non-organic pain remains a diagnosis of exclu-
(table 2). Questions should be directed at the location (includ- sion and so other reasons for the pain should be sought rst. A
ing any radiation), severity and character (sharp, dull throbbing, thorough examination of the cardiac, respiratory and gastro-
crushing, etc) of the pain. Important also are how long it lasts intestinal systems should be performed as well as palpation of
and any exacerbating or alleviating factors. Benign pain tends the chest wall; if normal this is usually very reassuring to all
to have a sudden onset at rest, lasting seconds to minutes with concerned.
a sharp character and is often well localised to the chest wall.
The absence of a crushing, poorly localised pain with radiation INVESTIGATIONS
to the left arm, nausea, sweating and association with exercise Investigations are not often helpful in making the diagnosis26
is reassuring and effectively excludes an ischaemic cause. 9 11and should be tailored to any abnormalities or suspicions
The frequency and overall duration of the pain is important identied after the history and examination. Table 3 gives a
because pain of >6 months duration is more likely to be psy- comprehensive list of possible investigations, most, if not all
chogenic. 5 In one study that included questions about duration of which, will not be required following a careful history and
of pain, 36% was for less than 1 day and 33% had lasted from physical examination. Very frequently any abnormal results
from investigations are either mild or have been previously
Table 2 Clues in history to causes of chest pain
noted.4 5
Cardiac Association with exercise Chest radiographs are also usually normal if there are no
Syncope, dizziness, vertigo other suspicious features from the history or examination and
Colour change (pallor, cyanosis)
Palpitations
are rarely diagnostic.2 4 911 They may still be useful though as
Sweating and nausea a normal radiograph often helps to reassure patients and their
Shortness of breath family. ECGs and echocardiograms have almost always been
Family history of premature ischaemic heart normal in most studies of paediatric chest pain.2 3 5 7 911 An ECG
disease, sudden death, arrhythmia and
can, however, show evidence of ischaemic changes, structural
cardiomyopathy
Illicit drug use, particularly cocaine abnormalities or rhythm disturbances (including changes such
Respiratory Worse on inspiration, exercise as the wave in WolffParkinsonWhite syndrome) and should
History of asthma be performed if these are suspected. Simple spirometric lung
Sputum, haemoptysis function or exercise testing with bronchodilator responsiveness
Gastrointestinal Worse with eating or posture may be useful to exclude exercise-induced bronchospasm.
Belching
Tenderness or pain chest base, shoulder tip,
retrosternal, epigastric MANAGEMENT
Heartburn, water-brash Where an organic cause is found for the pain then specic
Dysphagia
treatment is commenced. If there is a strong suspicion of a car-
Haematemesis, melaena
diac, respiratory or gastroenterological cause then referral to
Musculoskeletal Pain elicited by palpation, squeezing of chest wall
Pain worse on movement, breathing, coughing the appropriate specialist is usually necessary (table 4). Certain
History of trauma, sprain, strain symptoms and signs suggest referral to cardiologist is essential
Well-localised pain (box 1). Depending on the circumstances, a therapeutic trial
Psychological Chronic pain may be warranted, for example use of an inhaled bronchodila-
Pain as isolated symptom tor 1520 min before exercise, use of adequate antireux medi-
Family history of heart disease
Stressful life events
cation for 4 weeks, or regular non-steroidal anti-in ammatory
medication for 12 weeks.

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Table 3 Investigations to be considered for chest pain

Cardiac ECG
Box 1 The following are symptoms and signs that
Chest radiograph suggest referral to a cardiologist is mandatory:
Echocardiogram
Exercise tolerance test
24 h ECG monitoring
Serum fasting lipids Crushing left-sided precordial pain
Cardiac enzymes, troponins Pain radiating to left arm or jawparaesthesia
Respiratory Chest radiograph Onset with exercise, subsiding at rest (with asthma
Spirometric lung function testing excluded)
Exercise testing and bronchodilator Dizziness or syncope
responsiveness
Very occasionally CT chest scan
Nausea or sweatiness
Gastrointestinal 24 h pH probe
Palpitations
Isotope milk scan Cyanosis
Impedance studies Abnormal cardiovascular examination
Barium swallow Known structural heart disease or pulmonary hypertension
Endoscopy
Oesophageal manometry
Musculoskeletal None
Psychological None
Competing interests None.
Provenance and peer review Commissioned; externally peer reviewed.
It must be stressed that as most causes of chest pain can be determined
from a careful history and examination, few if any of the investigations in this
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654 Arch Dis Child 2010;95:649654. doi:10.1136/adc.2008.155309

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Recurrent chest pain in the well child

A Ives, P E F Daubeney and I M Balfour-Lynn

Arch Dis Child 2010 95: 649-654 originally published online April 6, 2010
doi: 10.1136/adc.2008.155309

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