Jennifer Thull Thull-Freedman, MD, MSCI, FAAP(PEM) Assistant Professor of Paediatrics University of Toronto Co Co-director, PEM Clinical Fellowship The.

Jennifer Thull Thull-Freedman, MD, MSCI, FAAP(PEM)Assistant Professor of Paediatrics

University of TorontoCo Co-director, PEM Clinical Fellowship

The Hospital for Sick Children

From my residency A 12-year year-old previously healthy boy

presented to the ED after first seeking care at the neighborhood fire department for chest pain

Told to take a warm bath for muscle achesArrived several hours later alert but in painHR=130, BP not doneCXR obtainedChild waited in room for CXR to be reviewed

From my residency Child suddenly became unresponsive and

pulselessUnable to be resuscitatedCXR reviewed during resuscitation showedwidened mediastinumAutopsy revealed dissection of the aorta

HoweverMost cases of chest pain in children are not

related to serious pathologyHistory and physical exam often sufficient

evaluation

The challengeObjectives

Review relevant literatureReview common causes of chest pain in

childrenDiscuss uncommon but serious causesPresent an approach to the child with chest

painSummarize take take-home points

Etiology of chest pain in kids

Very few studiesMost retrospectiveVariable inclusion/exclusion criteriaLimited detail provided

Selbst et alObjectives:Identify causes of chest pain in childrenAssess value of echocardiogramProspectiveEnrolled all patients with chest painECG and echo offered to those with ill ill-

defined or suspected cardiac etiologyPediatricsPediatrics1988; 82: 3191988; 319--

323323

Selbst et al.

Population407 patientsPhiladelphia, PennsylvaniaMedian age 12.5 years55% female, 90% African African-American43% acute pain <48 hoursDid not exclude known diseasePediatricsPediatrics1988; 82: 3191988; 319--

323323

Selbst et al.

ECG ECG’s in 191/235 children31 abnormal (16%)27 minor or previously known findings3 dysrhythmias detected on physical exam1 with known SLE had findings of pericarditisPediatricsPediatrics1988; 82: 3191988; 319--

323323

Selbst et al.

Echocardiograms in 139/23517 abnormal (12%)

12 mitral valve prolapse (8.6%)Similar prevalence to general population

2 pericardial effusion2 mitral valve regurgitation1 poor LV functionPediatricsPediatrics1988; 82: 3191988; 319--

323323

Selbst et al.

Chest radiographs in 137/40737 abnormal (27%)

Most frequent: infiltrates, atelectasis, hyperinflation

1 pneumothorax in a child with Marfan Marfan’s syndrome

1 clavicle fracture suspected clinically1 child with SLE had pleural effusion, large heart

PediatricsPediatrics1988; 82: 3191988; 319--323323

Selbst et al.

Organic disease related toAge <12 yearsPain awakening child from sleepAcute onsetAbnormal physical examNot related to description or location of painPediatricsPediatrics1988; 82: 3191988; 319--

323323

Selbst et al. #2

6-month follow follow-up of 149/407 patients43% had intermittent or persistent painNo significant disease identified

1 mitral valve prolapse1 gastrointestinal disease3 asthma

Conclusion:H&P sufficient for identifying majority of

significant etiologiesClinical PedsPeds1990; 29: 3741990; 374--77

Rowe et al.

Chest X X-rays done in 50%18/161 with positive result

15 infiltrates2 pneumomediastinum1 pneumothorax

ECG done in 18%2/60 with significant new findingsTachycardia and ST changes suggested

myocarditisWPW

CMAJCMAJ1990; 143:3881990; 388--9494

Massin et al.9 cases cardiac etiology in 168 PED patients

3 SVT2 MVP4 sick sinus1 myocarditis1 pericarditis1 cardiac hemochromatosis with β-thalassemia

5 cases cardiac etiology in 69 card. clinic patients

5 SVTClin Pediatr 2004;43:231 231

Massin et al.Results

Palpitations or abnormal auscultation predicted all

cases of cardiac diseaseConclusions

Chest pain in children usually benignHistory and physical usually sufficientLaboratory testing guided by H&P

Clin Pediatr 2004;43:231 231-

Limitations of current literature

Small numbers for characterizing rare eventsLimited detailChildren with known disease not excludedLack of follow follow-upNo evidence evidence-based guidelines

Differential Diagnosis

Chest wall Trauma Costochondritis Precordial catch Slipping rib Infection Mastalgia Zoster

Gastroesophageal Reflux Foreign body

Pulmonary Asthma Pneumonia/effusion Pneumothorax Pleurisy Pulmonary embolus Malignancy

Hematologic Sickle cell disease Psychogenic

Differential Diagnosis

CardiacAngina

Coronary abnormalitiesHypercoagulable stateCocaine

Obstructive heart diseaseIHSS, aortic stenosis

Pericardial effusion/pericarditisArrhythmiasMyocarditisAortic aneurysm

Cases

CaseA 12-year year-old girl presents to the

emergency department with chest pain for 2 days

Started graduallyWorse with deep breathHad URTI last weekAfebrileTender on both sides of sternumRemainder of physical exam normal

Costochondritis

Inflammation of costochondral cartilageCause

OverusePreceding URTI with coughIdiopathic

Sharp pain, worse with movementAll agesTenderness over costochondral joints

Case

A 10 10-year year-old boy presents to the ED with recurrent episodes of left chest pain.

Feels like a sudden stabCan’t take a deep breathLasts 2 2-3 minutesOccurs at restNot reproducibleNormal physical exam

Precordial Catch Syndrome

“Texidor’s twinge”Sudden, briefOccurs at restLocalizedSharpExacerbated by deep breathNo associated symptomsNo physical findings

Case

A 6 6-year year-old girl comes to the emergency department after having chest pain at home.

Stopped playing, became clingy, said chest hurt

Mom thought she looked paleNow looks and feels betterHR=110, normal physical exam

SVT

In children >1 year 82% present with palpitations 14% with pain

14% perspiration14% dizzy4% pallor

1-3% of chest pain complaints in ED6% of chest pain referred to cardiologistMedian time from symptoms to diagnosis 138d

CaseA 13 13-year year-old boy presents to the

emergency department with sudden severe chest painSharp pain in anterior chestAppears anxiousBP 80/40 in right armDiastolic murmur

Marfan syndromeCaused by fibrillin gene mutationManifestations

Musculoskeletal: Tall, long limbs and fingers, pectus

Ocular: Lens dislocationCardiovascular: Aortic root dilation, MVPPulmonary: Spontaneous pneumothorax

50% have aortic root dilation by age 10 years90% have aortic root dilation by age 20 years

Aortic dissection

Children at riskMarfan syndromeEhlers-DanlosCoarctationAortic stenosisTurner syndromeEndocarditisCocaine use

CaseA 17-year year-old female presents to the ED

with chest pain that has lasted for 1 hourPain began during soccer practiceHas happened previously with exerciseMidsternal, squeezing, radiates to left armPMH: Admitted to hospital for FUO at age 2

years

Kawasaki Disease

Acute febrile vasculitis of childhoodFeatures

Fever (>39 degrees for 5 days)Non Non-exudative conjunctivitisErythema of oral mucosa and tongueErythema and swelling of hands and feetCervical adenitis >1.5 cmRash

Leading cause of acquired heart disease in kids

Cardiac sequelae of KDAcute and subacute

Myocarditis (50% of patients)PericarditisMitral, aortic insufficiencyArrhythmias

Coronary aneurysms20 20-25% if untreated5% if treated with IVIGAppear 7 days to 4 weeks after onset of fever

Cardiac sequelae of KD

Long-term follow follow-up (> 10 years) of 594 untreated patientsIVIG treatment standard since late 1980 1980’s24.6% had coronary aneurysms

49% had regression 19% developed stenosis (4% of total) 8% developed myocardial infarction (2% of total)

Circulation1996;94:1379-85

Myocardial ischemia in kidsAnomalous coronary arteriesPrevalence 2:1000Anomalous origin of L coronary from pulm.

ArteryPresents in first months of lifeIrritability, heart failure, cardiac enlargement

Anomalous origin from incorrect sinus of ValsalvaPresents later in childhoodCompression between aorta and pulm Artery

Hypoplastic coronary arteries

Myocardial ischemia in kids

Sickle cell diseaseMyocardial infarction uncommon but describedPerfusion defects in 5% children studied in a

Paris sickle cell clinic ( Arch Dis Child 2004;89:359 359-62)

Microvascular occlusion of small vesselsExchange transfusion may be helpful for acute

ischemia ( Pediatrics 2003;111:e183 e183-7)

Myocardial ischemia in kids

Nephrotic syndromeThrombotic occlusion of coronary arteries

Long Long-standing diabetes mellitusFamilial hypercholesterolemiaSLE, Antiphospholipid antibody syndromesCardiac transplantCocaine abuse

CaseA 16-year year-old boy presents to the

emergency department after fainting at a track meet

Remembers having chest pain during his raceFather died suddenly in his 30 30’sSystolic murmur on exam

Hypertrophic cardiomyopathy

Autosomal dominantSymptoms in 2 2nd nd decadeMay present with angina angina-like pain or

syncopeImpaired diastolic relaxation, increased O O2

demandRisk of sudden death 6% in children

Hypertrophic cardiomyopathyCaseA 6-year year-old girl presents to the ED

with cough for 3 weeks and chest pain for 1 week

Feels very tiredIllness began with URTI 3 weeks agoAfebrileHeart rate = 160Liver palpable 3 cm below RCM

Myocarditis

Usually viral etiologyEnterovirus (coxsackie), adenovirus

PresentationHeart failureChest pain

More likely in older kids and adultsIschemia or concurrent pericarditis

Myocarditis

Physical findingsTachycardia, tachypneaPoor perfusionMuffled heart sounds, S3, murmurHepatomegaly

CXRCardiomegalyPulmonary edema

MyocarditisECG

Sinus tachycardiaDecreased voltages (<5 mm) limb leadsLVHProlonged PR interval, prolonged QT interval

EchocardiogramHypokinesis, impaired function

Hypertrophic cardiomyopathy

CaseA 6-year year-old girl presents to the ED with

coughfor 3 weeks and chest pain for 1 weekFeels very tiredIllness began with URTI 3 weeks agoAfebrileHeart rate = 160Liver palpable 3 cm below RCM

Pericarditis

Infectious etiology common in childrenPain

More common in older children and adolescents

Worse when supine, relieved by leaning forward

Physical findingsFriction rub if effusion smallMuffled heart sounds, pulsus paradoxus if

large

Pericarditis

ECGLow voltagesST elevation

Usually leads I, II, V5, V6

Electric alternansProduced by swinging motion of heart within

effusion

CaseA 9-year year-old obese boy is brought to the

ED at11 pm complaining of chest pain since dinner

preventing him from sleepingHas been having episodes for few weeksDescribed as burningWorse after big meals and when lying downNormal physical exam

Gastroesophageal Reflux

Berezin et al.27 children 8 8-20 years with idiopathic chest

pain all received EGD, manometry, pH monitoring

Not blinded, no control groupResults: 78% had gastroesophageal cause

16 of 27 (59%) had esophagitis4 of 27 (15%) had gastritis1 of 27 (4%) with abnormal manometry

Gastroesophageal RefluxAccounts for 5 5-10% of PED chest pain visitsClassic pain is temporally associated with

mealsBurning, retrosternalTrial of antacid, H2RA, PPI is appropriateConsider pH probe if diagnostic testing

needed

Case

A 3 3-year year-old boy is evaluated in the emergency department with chest pain for several hoursPoints to sternal notchDroolingRefusing juiceAfebrile, well well-appearingBreath sounds equal

Esophageal foreign bodyCaseAn 8 8-year year-old boy is brought to the ED

directlyfrom a hockey practice during which he said

his chest hurt and he couldn couldn’t breathe Several similar episodesFeeling better since arrival to EDTight coughNormal breath sounds, no murmurNormal CXR and EKG

AsthmaMay account for 10 10-20% chest pain in kidsPersonal or family history atopic conditionsAssociated with coughMay be worse at night or with exerciseWheezing not always detectableTrial of bronchodilatorConsider PFT for pain with exercise

Case

A 17 17-year year-old boy presents to the emergency department with right chest painJust returned hours ago from vacation in

CozumelPain began one day agoProgressive dyspnea during flight home

Pneumothorax/pneumomediastinum

Children at riskAsthma, bronchiolitisBarotraumaCough, choking, vomitingCrack, cannabisCystic fibrosisMarfan syndromeTall male teenagers

Case

A 15-year year-old girl presents to the ED with chest

pain Present for several daysReports feeling dizzy and short of breathNot associated with exercisePhysical exam unremarkableGrandmother died last week of heart attack

PsychogenicPsychogenic5-20% of chest pain in childrenMore common in adolescentsRecent or current stressful situationFamily illness, especially cardiovascularFamily history of chest painOther somatic and sleep complaintsDepression

The approach: History

Description of painNot as reliable in children as in adultsPrecipitating factors

ExertionEatingDeep breathingMuscle useTraumaEmotional stress

The approach: History

Frequency and chronicityAssociated symptoms

FeverCoughShortness of breathSyncopeDizzinessPalpitations

The approach: History

The approach: HistoryPast medical historyKnown heart diseaseAsthma or atopic conditionsProthrombotic conditions

Cancer SLE Nephrotic syndrome

Medications and drugsFamily history

The approach: Physical examGeneral appearanceBody habitusVital signsChest wall palpationAuscultationAbdomenPeripheral perfusion

Red flags

Pain associated with exercise, palpitations, or syncope

Shortness of breath Pain limits daily activities or disturbs sleep Substance abuse Presence of prothrombotic conditions PMH consistent with Kawasaki disease Family history of sudden death or early cardiac

death Abnormal vital signs or physical findings

The approach

Further evaluationCXRECGHolter monitorEchocardiogramCardiology consultationTherapeutic trials

SummaryChest pain in pediatrics usually due to

benign,identifiable etiologyCardiac and other life life-threatening causes

ofchest pain rare but do existOften can be ruled out by history and

physical examDiagnostic tests appropriate in presence of

red flags