Chest Pain in Children and Adolescents. Angela Romano, MD

Chest Pain in Children and Adolescents Angela Romano, MD Disclosures • I have no conflicts of interest. • I have no financial disclosures. Pediatr...
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Chest Pain in Children and Adolescents Angela Romano, MD

Disclosures • I have no conflicts of interest. • I have no financial disclosures.

Pediatric Chest Pain • Second most common reason for referral to a pediatric cardiologist – Murmur is #1

• Average age of complaint = 12 to 14 years • Can occur as young as 4 years • 650,000 physician visits per year – 10 to 21 years of age – 0.3% to 0.6% of visits to a pediatric ED

ED discharge diagnoses: pediatric chest pain

Classification of pediatric chest pain types from all studies

Pediatric Chest Pain - Epidemiology • Idiopathic chest pain (NOS): most common cause (36%). These patients will have a negative history, PE and lab tests. • In those cases in which a specific etiology can be found, musculoskeletal causes are the most common (12 – 20%).

Pediatric Chest Pain - Epidemiology • Approximately 3-10% of cases presenting with chest pain may be linked to a cardiac etiology. • Often, cardiac pathology is identified as an incidental finding, but is not a cause for the chest pain • bicuspid aortic valve, ASD, asymptomatic WPW

• A BROAD differential diagnosis must always be kept in mind when assessing a child with chest pain. • NON-CARDIAC • CARDIAC

• Do NOT immediately assume pediatric chest pain is cardiac in nature. • Do NOT immediately rule out potential serious pathology in children with chest pain.

Chest pain may be the harbinger of a sudden cardiac death event. Potentially lethal cardiac pathology may be clinically silent.

• Dramatic media accounts of sudden death in young athletes have focused attention on chest pain as a potentially serious sign of cardiac disease.

• Source of great deal of concern and anxiety for the child and family. Treat the complaint seriously! Families are seeking reassurance. • Chest pain in children and adolescents is most commonly non-cardiac.

• We will not discuss today chest pain in children with known congenital heart disease: operated and unoperated (TOF, TGA, Aortic stenosis) • These patients are under the care of pediatric cardiologists. Such patients presenting with chest pain should receive special attention and be referred to a cardiologist.

Evaluation of Pediatric Chest Pain



History • Description of the chest pain: – location, quality, severity, radiation, duration

• Onset of chest pain: – acute, chronic

• What precipitates the pain? – exercise, movement

• Associated symptoms? – dizziness, syncope, fever, dyspnea, dysphagia, palpitations

History • Pertinent recent medical history: fever, pharyngitis, URI, trauma/accidents, drug use, psychological stressors • Pertinent past medical history: genetic disorders, asthma, cardiac disease, Kawasaki syndrome, SS disease, rheumatologic disorders (SLE, JIA) • Pertinent past surgical history

History • Family History: • Premature cardiac death • Sudden unexplained death • Cardiomyopathies • Cardiac transplantation • Arrhythmias • Pacemakers or implantable defibrillators • Marfan or Marfan-related syndromes

Approach to general physical exam • Assess vital signs • General appearance • dysmorphic features, chronically ill child

• Thorough inspection/palpation of chest wall (bruising, pectus, scoliosis, rash, subcutaneous air) • Cardiac/pulmonary exam (murmur, rub, wheezing) • Abdominal exam • Extremities: arthritis, hypermobility

RED FLAGS • • • • •

Young age Acute onset Ill-appearing child in extremis Pain precipitated by exercise Pain associated with: – dizziness, syncope – palpitations – respiratory distress – abnormal cardio-respiratory examination – trauma

General Pearls • Child who appears ill and in acute distress with the complaint of chest pain needs to be stabilized and referred to appropriate emergency/consulting services • Younger children are more likely to have cardio-respiratory etiology for chest pain • Adolescents are more likely to have pain associated with stress, anxiety. Their pain may be more chronic, indolent, intermittent.

EKG • If, based on history and physical exam, there is a suspicion of cardiac disease, the next best screening test is an electrocardiogram • Non-invasive, inexpensive tool

Non-cardiac causes of pediatric chest pain • • • • •

Musculoskeletal Pulmonary or Airway-related Gastrointestinal Psychogenic Miscellaneous

CASE • 11 yr old girl complains of left upper sternal chest pain for the past 5 days. The pain is sharp, increases with inspiration and lasts < 1 minute in duration. No history of fever, cough, palpitations, dizziness, syncope. Prior to the onset of the pain, she had gone rock climbing for the first time with her friends. • P.E.: Well appearing in no distress; normal cardiac exam; palpation of the chest yields mild to moderate tenderness of the left second and third costochondral junctions (this pain is similar to the pain she has had for the past 5 days)

Non-cardiac Causes of Chest Pain Musculoskeletal ● Costochondritis/costosternal syndrome ● Tietze syndrome ● Nonspecific or idiopathic chest wall pain ● Slipping rib syndrome ● Trauma and muscle strain–overuse injury ● Xiphoid pain (xiphoidalgia)

Costochondritis • Involves 2 to 4 contiguous costochondral junctions • Unilateral • More in the cephalad joints • Sharp pain • Lasts seconds to minutes • Exacerbated by inspiration • Reproducible • Traumatic strain • Self-limited

Musculoskeletal Chest pain • Rest • Anti-inflammatory drugs: • NSAIDs

• Reassurance

Non-cardiac Causes of Chest Pain Pulmonary or Airway-related ● Bronchial asthma ● Exercise-induced asthma ● Bronchitis ● Pleurisy ● Pneumonia ● Pneumothorax ● Pulmonary embolism ● Acute chest syndrome (sickle cell disease)

Asthma • Consider reactive airway disease: – Dyspnea with exercise – Exercise associated chest pain – Exertional cough – Wheezing – Eczema – Family history of asthma

CASE • 9 year old was quickly finishing his last chicken nugget at the dinner table before rushing off to the den to watch the college BB play-offs. Without any premonitory symptoms, he complained of severe chest pain and had a syncopal episode. He awoke within 30 seconds and appeared well. He continued to complain of mild chest and epigastric pain which resolved within a few minutes.

Non-cardiac Causes of Chest Pain Gastrointestinal • • • • • •

Esophageal spasm Gastroesophageal reflux disease Peptic ulcer disease Drug-induced esophagitis/gastritis Cholecystitis Foreign Body in esophagus

Non-cardiac Causes of Chest Pain • Breast tenderness (puberty/pregnancy) • Herpes zoster: pain may occur prior to the onset of rash

Non-cardiac Causes of Chest Pain Psychogenic: stress related pain • Preceding event: – – – – –

Death of friend or family member Divorce or separation Illness or trauma of a family member Cardiac disease in a loved one Depression

CASE • A 14 yr old boy attends a high school for gifted children. He is racing to catch the train, sprinting up 2 flights of steps. He experiences a syncopal episode on the train platform. He reports that he had severe midsternal chest pain and lightheadedness prior to passing out. • He has no previous cardiac history. • Family history: father has a hypertrophic cardiomyopathy and a defibrillator.

Abnormal EKG in patient with HCM:

LVH with repolarization abnormalities

Abnormal EKG in patient with HCM: deep narrow Q waves

EKG in Patients with HCM • Deep, narrow q waves (II, III, aVF, v5, v6) in young patients may be the most specific EKG finding for HCM • These EKG changes may precede ventricular wall hypertrophy shown on the echocardiogram

Ventricular tachycardia


• Heterogeneous genetic disorder that affects proteins of the cardiac sarcomere • Sarcomere: the contractile unit within the cardiac myocyte that is comprised of thick myosin and thin actin filaments

HYPERTROPHIC CARDIOMYOPATHY • Single most common cause of sudden cardiac death in young athletes • Incidence in general population is 1 in 500 • Histopathologically – myocardial disarray in which the cellular architecture is disorganized • Hypertrophied left ventricle: • asymmetrical (ventricular septum) or concentric • non-obstructive or obstructive

HYPERTROPHIC CARDIOMYOPATHY • Heterogeneous group of genetically transmitted diseases • Autosomal dominant • Non-familial variants also recognized • HCM can be caused by mutations in genes coding for sarcomeric proteins

Hypertrophic Cardiomyopathy • Diagnostic findings may not be evident until adolescence or later • Maximal hypertrophy can occur after 18 years of age • EKG is abnormal in the majority of patients (90%) • EKG changes are NOT specific for HCM

EKG findings in patients with obstructive and non-obstructive HCM Circulation, 1978

• One hundred and thirty-four patients with hypertrophic cardiomyopathy were evaluated by standard 12-lead electrocardiography. • Normal electrocardiograms were extremely uncommon, occurring in less than 7% of each subgroup of patients

EKG findings in patients with obstructive and non-obstructive HCM Circulation, 1978

• Repolarization abnormalities and left ventricular hypertrophy (voltage criteria, deep q waves) were the most common abnormalities, occurring in 81% and 62%, respectively, of the total population.

HCM • Due to the increased muscle mass and disarray of the myocardial muscle cells, the heart is more vulnerable to ischemia with resultant ventricular fibrillation. • The ventricular tachycardia/fibrillation event may self-terminate. • When arrhythmias “self-terminate”, the patient may present with syncope or near-syncope depending on the duration of the arrhythmia.

Ventricular fibrillation may be the final common pathway in syncopal events in patients with underlying cardiac pathology - structural heart disease - primary arrhythmia

Patients with HCM at high risk for sudden death • Family history of sudden or other premature HCM related deaths • Unexplained syncope • Hypotensive response to exercise • Non-sustained ventricular tachycardia on ambulatory monitoring • Extreme L ventricular hypertrophy (> 3cm)

Hypertrophic Cardiomyopathy Therapeutic interventions • Negative inotropic agents: beta-blockers, calcium channel blockers • Surgical myectomy • Exercise restrictions – no competitive sports • Implantable cardioverter-defibrillators

CASE 16 year old presents to the ED with a few hour history of substernal, burning chest pain which radiates to his jaw. He seems on edge and has a history of nosebleeds. He is hypertensive and tachycardic.

CASE • 14 yr old athletic adolescent male presented with crushing chest pain and near collapse while playing basketball at school. • Previous history of a cardiac murmur. • “Normal” echocardiogram in the past • He was brought by EMS to our ED

EKG obtained in the ED

•Labs: elevated cardiac enzymes (troponin)

Congenital Coronary Artery Anomalies • Coronary artery arises from the wrong sinus of Valsalva • Diagnosis: echocardiography, MRI • Therapy: surgical reimplantation of the coronary artery or un-roofing of the coronary artery

CASE • 14 yr old athletic adolescent male presented with crushing chest pain and near collapse while playing basketball at school. • Previous history of a cardiac murmur. • “Normal” echocardiogram in the past • He was brought by EMS to our ED

Coronary Artery Anomalies • Second most common non-acquired cause of sudden death in the young • Ischemia – Ventricular arrhythmias • acute angled kinking at the origin of the coronary artery • a small slit-like orifice to the coronary artery • compression of the coronary artery between the aorta and pulmonary artery during exercise

Anomalous left coronary artery from the right sinus of Valsalva • The crushing chest pain and near collapse in this patient was likely related to a ventricular arrhythmia secondary to myocardial ischemia • He was very fortunate to have recovered completely from what was an aborted “sudden cardiac death” event.

Ventricular fibrillation may be the final common pathway in syncopal events in patients with underlying cardiac pathology - structural heart disease - primary arrhythmia

CASE • 6 year old girl awoke from sleep at night and complained to her parents of abdominal pain. She was distressed, diaphoretic and pale. Two other siblings were recovering from a GI viral infection. She was encouraged to lie down and try to sleep. An hour later, she also shared that her chest was hurting. • History: Kawasaki syndrome at 1 year of age associated with giant coronary artery aneurysms

Myocardial Infarction Secondary to Kawasaki Disease • • • •

73 % of cases occur during 1st year of onset Few cases still occur > 6 years of onset Can occur during sleep & rest Symptoms: – Shock, Chest pain, vomiting, inconsolable crying, abdominal pain – 1/3 asymptomatic – Chest pain less common < 4 years of age

Myocardial Infarction Secondary to Kawasaki Disease • • • •

73 % of cases occur during 1st year of onset Few cases still occur > 6 years of onset Can occur during sleep & rest Symptoms: – Shock, Chest pain, vomiting, inconsolable crying, abdominal pain – 1/3 asymptomatic – Chest pain less common < 4 years of age

Kawasaki Disease • CoronaryAneurysm formation – 20-25% if untreated – 2-4 % if received IVIG within 10 days of fever

• Coronary Aneurysm – Most occur within 1-3 weeks from onset – > 8 mm susceptible to acute myocardial infarction

• Giant aneurysms (≥8mm) may not regress and may develop stenosis, leading to acute myocardial infarction. • Treatment may involve different types of surgical or trans-catheter coronary interventions

An Adolescent with Chest Pain Pediatric Emergency Care. 2004; 20(11): 765-768

• 19 year old female with prior history of Kawasaki Disease as an infant

– Presented to ER with chest pain • Mid-sternal region • 20 minutes • Radiation to left arm


An Adolescent with Chest Pain Pediatric Emergency Care. 2004; 20(11): 765-768

EKG: Anterior repolarization abnormalities consistent with ischemia ECHO: Normal CATH: • 90% eccentric & calcified single discrete stenosis of the proximal segment of the left anterior descending artery • Successful percutaneous transluminal coronary angioplasty with stent insertion

Kawasaki syndrome • Importance of controlling risk factors for atherosclerosis • • • •

Hypertension Diabetes Obesity Hypercholesterolemia

• Lifestyle changes • Sedentary • Smoking • Stress

CASE • 7 year old boy has been complaining of chest pain to his mom over the past two weeks. He described the pain as “gorillas jumping very fast on his chest”. The episodes had been rare and short-lived until last evening, when he approached his mom with the same complaint, but this time he appeared pale and anxious. She brought him to the ED for evaluation.

SVT at 230 bpm reentrant mechanism: sudden onset and termination (“chest pain”) • Chronic, intermittent chest pain

NSR with ventricular pre-excitation (WPW)

Wolff-Parkinson-White syndrome

Wolff-Parkinson-White syndrome • Accessory atrio-ventricular pathway can cause re-entrant tachycardia (SVT) • Typically have a structurally and functionally normal heart • 12% of asymptomatic patients with WPW have experienced sudden cardiac death

EKG following successful radio-frequency ablation of the accessory pathway.

WPW • Children with WPW and intermittent ventricular pre-excitation are considered to have a “low-risk” accessory pathway. This is usually confirmed with a transesophageal electrophysiological study. • No therapy is indicated • No exercise restrictions are imposed

Wolff-Parkinson White syndrome • Rapid electrical conduction from the atrium to the ventricle via the accessory pathway (bypass tract) during atrial fibrillation can result in a rapid and irregular ventricular rhythm that can degenerate into ventricular fibrillation

CASE • 14 yr old female presents with a 3-day history of fever and chest pain. The chest pain was retrosternal, non-radiating and worse with lying flat and with taking deep inspirations. These symptoms improved with sitting forward. • A week prior she had been treated with amoxicillin for a non-strep pharyngitis.

CASE • General appearance: persistent chest discomfort was obvious, anxious • Cardiac exam: tachycardia, no murmur, + rub • Labs: elevated cardiac enzymes (CPK, troponin), elevated ESR and C-reactive protein • Chest X-ray: normal

Pericarditis • Tachycardia, Pericardial friction rub, Fever, • Marked Chest Pain (80% of cases) – intensified in supine position – relieved with leaning forward

• Generalized ST segment elevation on EKG • More commonly viral in etiology • Preceding respiratory or GI illness in 40-75% of patients

Pericarditis Inflammation of the pericardium • With or without associated effusion • Isolated or a manifestation of systemic disease

EKG in Pericarditis

Early Repolarization: Benign finding

EKG in Pericarditis • PR-segment depression, • “notched” J-point • upwardly concave ST-segment elevation (if in fact the ST-segment is really elevated in comparison to the TP segment). • Overall decreased voltages and possibly Electrical/QRS alternans (associated with large pericardial effusion)

Pericarditis: EKG Findings Four stages: • I: diffuse J-point and ST elevation, PR segment depression • II: normalization of ST and PR segments • III: diffuse T wave inversions • IV: normalization of T waves

Electrical alternans • Occurs with excessive motion of the heart within the fluid filled pericardial space

“Myo-pericarditis” • Labs – Troponin I elevated in 32% of adult patients with viral or idiopathic pericarditis • likely due to inflammation of adjacent epicardium and myocardium • returns to normal in 1-2 weeks and not associated with worse prognosis

– Markers of inflammation may be elevated

Pericarditis - Etiology •

Infectious – Viral, bacterial, tuberculosis – Non-infectious • Rheumatologic/autoimmune, neoplastic, metabolic (uremia, hypothyroid), trauma, drug-induced • Idiopathic: –

Caveat: many idiopathic cases are believed to be viral in origin

Treatment: pericarditis • Goal: control inflammation and pain • NSAIDs are the drug of choice (ibuprofen) • Course is usually benign and self-limited - Small percentage of patients will have recurrent pericarditis

Pericardial Tamponade • Increased intrapericardial pressure impedes venous return to the heart • Ventricular compliance is compromised resulting in decreased diastolic filling of the ventricles • End result: Low cardiac output state

Pericardial Tamponade • Maintain fluid status with volume resuscitation • Do NOT administer diuretics • Treatment of choice: pericardiocentesis

CASE • An 18 year old young lady was out doing errands on a Saturday afternoon. • She was 35 weeks pregnant. • She acutely developed severe, intense chest pain which radiated to her posterior thorax. She had difficulty breathing and felt nauseated. • She was a tall, lanky young lady with no previous cardiac or concerning medical history. • The pain peaked and then subsided, though never completely went away.

CASE • Because of persistent chest pain, she was brought to an ED where an astute physician ordered an echocardiogram because of her body habitus. She was diagnosed with a dilated aortic root with dissection. • A C-section was performed and she had an aortic root replacement with a mechanical valve. • She has a genetic mutation in the fibrillin-1 gene. Her son has the same mutation.

Marfan Syndrome • Disorder of connective tissue caused by mutations in gene (FBN1) that encodes fibrillin-1 – Extracelluar matrix protein – Structural role in connective tissue – Principal component of microfibrils associated with elastin fibers (media of aortic wall) – Regulator of the cytokine TGF-β (binds to fibrillin)

Marfan Syndrome • Variable expression in families • Phenotypic expression importantly influenced by factors other than basic genetic defect – Modifier genes – Environmental influences

Chest pain with Marfan and Marfan-related syndromes • Mitral valve prolapse • Redundant, myxomatous valves • Ventricular arrhythmias

• Muscular-skeletal • chest wall deformities, scoliosis

• Aortic Dissection • rare occurrence in pediatrics

Aortic Root Diameter • Aortic root diameter at the sinuses of Valsalva is considered the best predictor of adverse cardiovascular outcome. • It is the most commonly used measure to determine the timing of cardiac surgery.

Summary • Pediatric chest pain is a common presenting complaint. • Most etiologies are benign but an evaluation must exclude a broad differential of uncommon, but potentially life-threatening conditions.

Summary • Consider laboratory studies if the history is concerning or the physical examination is abnormal. • Avoid expensive, laboratory studies in patients with chronic, intermittent chest pain, a benign history and a normal physical examination.

Basic Tools to identify the high-risk patient

• Detailed history (details surrounding the symptom and family history) • Thorough physical exam • EKG • Chest X-ray

Child presenting with chest pain


Is there history of trauma?


Chest X-ray


Fever and respiratory findings

Chest X-Ray Consider pulmonary causes: Pneumonia, Asthma


Abnormal cardiac exam

If risk factors persist, consider D-dimer and CT chest for pulmonary embolism

If abnormal cardiac exam, consider: EKG, ECHO, troponins: Myocarditis and pericarditis


Myocardial ischemia? get troponins


Dysphagia, Pain associated with food, or epigastric pain

EKG, ECHO Consider structural abnormality, Arrhythmia, Myocardial ischemia


Explore GI causes, Consider chest X-Ray for foreign body

Dysphagia, Pain associated with food, or Epigastric pain


Explore GI causes, Consider chest X-Ray for foreign body


Reproducible Pain? Overuse?


Consider musculoskeletal causes


Recent stressors?


Consider psychogenic cause


Idiopathic Surveillance, reassurance

Evaluation with child psychologist

SUMMARY: When to refer to a cardiologist • Atypical episodes • Recurrent episodes not resolved with a conventional approach/therapy • Exertional/exercise related chest pain • Chest pain with syncope, palpitations • Chest pain with abnormal cardiac hx, exam or EKG • Family history of sudden death • Reassurance in particularly anxious patient//family

Go with your gut !!

Cardiac Work-Up • EKG • Echocardiogram • 24 hr Holter monitor, event/loop recorder • correlate clinical symptoms with underlying cardiac rhythm

• Exercise stress test

The primary yield of the above testing is to demonstrate the absence of serious cardiac disease !!