Evaluation of Chest Pain in the Pediatric Patient

Evaluation of Chest Pain in the Pediatric Patient Jennifer Thull-Freedman, MD, MSCI, FAAP(PEM) Assistant Professor of Paediatrics University of Toronto Co Co-director, PEM Clinical Fellowship The Hospital for Sick Children

From my residency A 12 -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 aches Arrived several hours later alert but in pain HR=130, BP not done CXR obtained Child waited in room for CXR to be reviewed

From my residency Child suddenly became unresponsive and pulseless Unable to be resuscitated CXR reviewed during resuscitation showed widened mediastinum Autopsy revealed dissection of the aorta

However Most cases of chest pain in children are not related to serious pathology History and physical exam often sufficient evaluation

The challenge Objectives Review relevant literature Review common causes of chest pain in children Discuss uncommon but serious causes Present an approach to the child with chest pain Summarize take-home points

Etiology of chest pain in kids Very few studies Most retrospective Variable inclusion/exclusion criteria Limited detail provided

Selbst et al Objectives: Identify causes of chest pain in children Assess value of echocardiogram Prospective Enrolled all patients with chest pain ECG and echo offered to those with ill-defined or suspected cardiac etiology Pediatrics 1988; 82: 3191988; 319 --323323

Selbst et al. Population 407 patients Philadelphia, Pennsylvania Median age 12. 5 years 55% female, 90% African-American 43% acute pain <48 hours Did not exclude known disease Pediatrics 1988; 82: 3191988; 319 --323323

Selbst et al. ECG’s in 191/235 children 31 abnormal (16%) 27 minor or previously known findings 3 dysrhythmias detected on physical exam 1 with known SLE had findings of pericarditis Pediatrics 1988; 82: 3191988; 319 --323323

Selbst et al. Echocardiograms in 139/235 17 abnormal (12%) 12 mitral valve prolapse (8. 6%) Similar prevalence to general population 2 pericardial effusion 2 mitral valve regurgitation 1 poor LV function Pediatrics 1988; 82: 3191988; 319 --323323

Selbst et al. Chest radiographs in 137/407 37 abnormal (27%) Most frequent: infiltrates, atelectasis, hyperinflation 1 pneumothorax in a child with Marfan’s syndrome 1 clavicle fracture suspected clinically 1 child with SLE had pleural effusion, large heart Pediatrics 1988; 82: 3191988; 319 --323323

Selbst et al. Organic disease related to Age <12 years Pain awakening child from sleep Acute onset Abnormal physical exam Not related to description or location of pain Pediatrics 1988; 82: 3191988; 319 --323323

Selbst et al. #2 6 -month follow-up of 149/407 patients 43% had intermittent or persistent pain No significant disease identified 1 mitral valve prolapse 1 gastrointestinal disease 3 asthma Conclusion: H&P sufficient for identifying majority of significant etiologies Clinical Peds 1990; 29: 3741990; 374 --77

Rowe et al. Chest X X-rays done in 50% 18/161 with positive result 15 infiltrates 2 pneumomediastinum 1 pneumothorax ECG done in 18% 2/60 with significant new findings Tachycardia and ST changes suggested myocarditis WPW CMAJ 1990; 143: 3881990; 388 --9494

Massin et al. 9 cases cardiac etiology in 168 PED patients 3 SVT 2 MVP 4 sick sinus 1 myocarditis 1 pericarditis 1 cardiac hemochromatosis with β-thalassemia 5 cases cardiac etiology in 69 card. clinic patients 5 SVT Clin Pediatr 2004; 43: 231

Massin et al. Results Palpitations or abnormal auscultation predicted all cases of cardiac disease Conclusions Chest pain in children usually benign History and physical usually sufficient Laboratory testing guided by H&P Clin Pediatr 2004; 43: 231 -

Limitations of current literature Small numbers for characterizing rare events Limited detail Children with known disease not excluded Lack of follow-up No 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 Cardiac Angina Coronary abnormalities Hypercoagulable state Cocaine Obstructive heart disease IHSS, aortic stenosis Pericardial effusion/pericarditis Arrhythmias Myocarditis Aortic aneurysm

Cases Case A 12 -year-old girl presents to the emergency department with chest pain for 2 days Started gradually Worse with deep breath Had URTI last week Afebrile Tender on both sides of sternum Remainder of physical exam normal

Costochondritis Inflammation of costochondral cartilage Cause Overuse Preceding URTI with cough Idiopathic Sharp pain, worse with movement All ages Tenderness over costochondral joints

Case A 10 10 -year-old boy presents to the ED with recurrent episodes of left chest pain. Feels like a sudden stab Can’t take a deep breath Lasts 2 2 -3 minutes Occurs at rest Not reproducible Normal physical exam

Precordial Catch Syndrome “Texidor’s twinge” Sudden, brief Occurs at rest Localized Sharp Exacerbated by deep breath No associated symptoms No physical findings

Case A 6 6 -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 pale Now looks and feels better HR=110, normal physical exam

SVT In children >1 year 82% present with palpitations 14% with pain 14% perspiration 14% dizzy 4% pallor 1 -3% of chest pain complaints in ED 6% of chest pain referred to cardiologist Median time from symptoms to diagnosis 138 d

Case A 13 13 -year-old boy presents to the emergency department with sudden severe chest pain Sharp pain in anterior chest Appears anxious BP 80/40 in right arm Diastolic murmur

Marfan syndrome Caused by fibrillin gene mutation Manifestations Musculoskeletal: Tall, long limbs and fingers, pectus Ocular: Lens dislocation Cardiovascular: Aortic root dilation, MVP Pulmonary: Spontaneous pneumothorax 50% have aortic root dilation by age 10 years 90% have aortic root dilation by age 20 years

Aortic dissection Children at risk Marfan syndrome Ehlers-Danlos Coarctation Aortic stenosis Turner syndrome Endocarditis Cocaine use

Case A 17 -year-old female presents to the ED with chest pain that has lasted for 1 hour Pain began during soccer practice Has happened previously with exercise Midsternal, squeezing, radiates to left arm PMH: Admitted to hospital for FUO at age 2 years

Kawasaki Disease Acute febrile vasculitis of childhood Features Fever (>39 degrees for 5 days) Non-exudative conjunctivitis Erythema of oral mucosa and tongue Erythema and swelling of hands and feet Cervical adenitis >1. 5 cm Rash Leading cause of acquired heart disease in kids

Cardiac sequelae of KD Acute and subacute Myocarditis (50% of patients) Pericarditis Mitral, aortic insufficiency Arrhythmias Coronary aneurysms 20 20 -25% if untreated 5% if treated with IVIG Appear 7 days to 4 weeks after onset of fever

Cardiac sequelae of KD Long-term follow-up (> 10 years) of 594 untreated patients IVIG treatment standard since late 1980’s 24. 6% had coronary aneurysms 49% had regression 19% developed stenosis (4% of total) 8% developed myocardial infarction (2% of total) Circulation 1996; 94: 1379 -85

Myocardial ischemia in kids Anomalous coronary arteries Prevalence 2: 1000 Anomalous origin of L coronary from pulm. Artery Presents in first months of life Irritability, heart failure, cardiac enlargement Anomalous origin from incorrect sinus of Valsalva Presents later in childhood Compression between aorta and pulm Artery Hypoplastic coronary arteries

Myocardial ischemia in kids Sickle cell disease Myocardial infarction uncommon but described Perfusion defects in 5% children studied in a Paris sickle cell clinic ( Arch Dis Child 2004; 89: 359 -62) Microvascular occlusion of small vessels Exchange transfusion may be helpful for acute ischemia ( Pediatrics 2003; 111: e 183 -7)

Myocardial ischemia in kids Nephrotic syndrome Thrombotic occlusion of coronary arteries Long-standing diabetes mellitus Familial hypercholesterolemia SLE, Antiphospholipid antibody syndromes Cardiac transplant Cocaine abuse

Case A 16 -year-old boy presents to the emergency department after fainting at a track meet Remembers having chest pain during his race Father died suddenly in his 30 30’s Systolic murmur on exam

Hypertrophic cardiomyopathy Autosomal dominant Symptoms in 2 2 nd nd decade May present with angina-like pain or syncope Impaired diastolic relaxation, increased O O 2 demand Risk of sudden death 6% in children

Hypertrophic cardiomyopathy Case A 6 -year-old girl presents to the ED with cough for 3 weeks and chest pain for 1 week Feels very tired Illness began with URTI 3 weeks ago Afebrile Heart rate = 160 Liver palpable 3 cm below RCM

Myocarditis Usually viral etiology Enterovirus (coxsackie), adenovirus Presentation Heart failure Chest pain More likely in older kids and adults Ischemia or concurrent pericarditis

Myocarditis Physical findings Tachycardia, tachypnea Poor perfusion Muffled heart sounds, S 3, murmur Hepatomegaly CXR Cardiomegaly Pulmonary edema

Myocarditis ECG Sinus tachycardia Decreased voltages (<5 mm) limb leads LVH Prolonged PR interval, prolonged QT interval Echocardiogram Hypokinesis, impaired function

Hypertrophic cardiomyopathy Case A 6 -year-old girl presents to the ED with cough for 3 weeks and chest pain for 1 week Feels very tired Illness began with URTI 3 weeks ago Afebrile Heart rate = 160 Liver palpable 3 cm below RCM

Pericarditis Infectious etiology common in children Pain More common in older children and adolescents Worse when supine, relieved by leaning forward Physical findings Friction rub if effusion small Muffled heart sounds, pulsus paradoxus if large

Pericarditis ECG Low voltages ST elevation Usually leads I, II, V 5, V 6 Electric alternans Produced by swinging motion of heart within effusion

Case A 9 -year-old obese boy is brought to the ED at 11 pm complaining of chest pain since dinner preventing him from sleeping Has been having episodes for few weeks Described as burning Worse after big meals and when lying down Normal physical exam

Gastroesophageal Reflux Berezin et al. 27 children 8 8 -20 years with idiopathic chest pain all received EGD, manometry, p. H monitoring Not blinded, no control group Results: 78% had gastroesophageal cause 16 of 27 (59%) had esophagitis 4 of 27 (15%) had gastritis 1 of 27 (4%) with abnormal manometry

Gastroesophageal Reflux Accounts for 5 5 -10% of PED chest pain visits Classic pain is temporally associated with meals Burning, retrosternal Trial of antacid, H 2 RA, PPI is appropriate Consider p. H probe if diagnostic testing needed

Case A 3 3 -year-old boy is evaluated in the emergency department with chest pain for several hours Points to sternal notch Drooling Refusing juice Afebrile, well-appearing Breath sounds equal

Esophageal foreign body Case An 8 8 -year-old boy is brought to the ED directly from a hockey practice during which he said his chest hurt and he couldn’t breathe Several similar episodes Feeling better since arrival to ED Tight cough Normal breath sounds, no murmur Normal CXR and EKG

Asthma May account for 10 10 -20% chest pain in kids Personal or family history atopic conditions Associated with cough May be worse at night or with exercise Wheezing not always detectable Trial of bronchodilator Consider PFT for pain with exercise

Case A 17 17 -year-old boy presents to the emergency department with right chest pain Just returned hours ago from vacation in Cozumel Pain began one day ago Progressive dyspnea during flight home

Pneumothorax/pneumomediastinum Children at risk Asthma, bronchiolitis Barotrauma Cough, choking, vomiting Crack, cannabis Cystic fibrosis Marfan syndrome Tall male teenagers

Case A 15 -year-old girl presents to the ED with chest pain Present for several days Reports feeling dizzy and short of breath Not associated with exercise Physical exam unremarkable Grandmother died last week of heart attack

Psychogenic 5 -20% of chest pain in children More common in adolescents Recent or current stressful situation Family illness, especially cardiovascular Family history of chest pain Other somatic and sleep complaints Depression

The approach: History Description of pain Not as reliable in children as in adults Precipitating factors Exertion Eating Deep breathing Muscle use Trauma Emotional stress

The approach: History Frequency and chronicity Associated symptoms Fever Cough Shortness of breath Syncope Dizziness Palpitations

The approach: History Past medical history Known heart disease Asthma or atopic conditions Prothrombotic conditions Cancer SLE Nephrotic syndrome Medications and drugs Family history

The approach: Physical exam General appearance Body habitus Vital signs Chest wall palpation Auscultation Abdomen Peripheral 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 evaluation CXR ECG Holter monitor Echocardiogram Cardiology consultation Therapeutic trials

Summary Chest pain in pediatrics usually due to benign, identifiable etiology Cardiac and other life-threatening causes of chest pain rare but do exist Often can be ruled out by history and physical exam Diagnostic tests appropriate in presence of red flags