Chest Pain in Children MUHAMMAD ALI Cardiology Division Department of Child Health University of Sumatera Utara

Chest pain is frequently encountered in children in the office and emergency room. Does not indicate serious disease of the heart or other systems in most pediatric patients, but a society with a high prevalence of atherosclerotic cardiovascular disease, it can be alarming to the child and parents. Be aware of the DD of chest pain in children and should make every effort to find a specific cause before making a referral to a specialist or reassuring the child and the parents of the benign nature of the complaint. Making a routine referral to a cardiologist is not always a good idea; it may increase the family's concern and may result in a prolonged and expensive cardiac evaluation.

Clinical Manifestations IDIOPATHIC No cause can be found in 12% to 45% of patients, even after a moderately extensive investigation. Although an organic cause is less likely in children with chronic chest pain, some of these children are eventually referred for specialty evaluations. NONCARDIAC CAUSES OF CHEST PAIN Most cases of pediatric chest pain originate in organ systems other than the CV system. Identifiable noncardiac causes of chest pain are found in 56% to 86% of reported cases.

NONCARDIAC CAUSES OF CHEST PAIN… Costochondritis Causes chest pain in 9% to 22% of children with such pain. Characterized by mild to moderate anterior chest pain, usually unilateral but occasionally bilateral. The pain may be preceded by exercise, an URI, or physical activity; a specific position may also cause the pain. The pain may radiate to the remainder of the chest, back, and abdomen; it may be exaggerated by breathing, and it may persist for several months. Physical examination is diagnostic; the clinician finds a reproducible tenderness on palpation over the chondrosternal or costochondral junction. Tietze's syndrome is a rare form of costochondritis characterized by a large, tender, fusiform (spindle-shaped), nonsuppurative swelling at the chondrosternal junction. It usually affects the upper ribs, particularly the second and third costochondral junctions.

Musculoskeletal Chest pain is also common in children. The pain is caused by strains of the pectoral, shoulder, or back muscles after exercise or by trauma to the chest wall from sports, fights, or accidents. A history of vigorous exercise, weight lifting, or direct trauma to the chest and the presence of tenderness of the chest wall or muscles clearly indicate muscle strain or trauma. Abnormalities of the rib cage or thoracic spine can cause mild, chronic chest pain in children.

Respiratory Respiratory problems are responsible for about 20% of cases of pediatric chest pain, which may result from overused chest wall muscles or from pleural irritation. A history of severe cough, with tenderness of intercostal or abdominal muscles, is usually present. The presence of crackles, wheezing, tachypnea, retraction, or fever on examination suggests a respiratory cause of chest pain. Pleural effusion may cause pain that is worsened by deep inspiration. Chest x-ray examination may confirm the diagnosis of pleural effusion, pneumonia, or pneumothorax. In some children, asthma may be the cause of chest pain on exertion, but it may not be suspected until stress tests reveal exercise-induced asthma.

Gastrointestinal Some gastrointestinal disorders may present as chest pain in children. The onset and relief of pain in relation to eating and diet may help clarify the diagnosis. Esophagitis should be suspected in a child who complains of burning substernal pain that worsens with a reclining posture or abdominal pressure or that worsens after certain foods are eaten. Young children sometimes ingest foreign bodies, such as coins, that lodge in the upper esophagus, or they may ingest caustic substances that burn the entire esophagus. In such cases, the history makes the diagnosis obvious.

Psychogenic Psychogenic disturbances account for about 9% of cases and are seen equally in male and female adolescents. Often a recent stressful situation parallels the onset of the chest pain: a death or separation in the family, a serious illness, a disability, a recent move, failure in school, or sexual molestation. However, a psychological cause of chest pain should not be lightly assigned without a thorough history taking and a follow-up evaluation. Psychological or psychiatric consultation may reveal conversion symptoms, a somatization disorder, or even depression.

Miscellaneous 1.The precordial catch (Texidor's twinge or stitch in the side), a one-sided chest pain, lasts a few seconds or minutes and is associated with bending or slouching. The cause is unclear, but the pain is relieved by straightening and taking a few shallow breaths or one deep breath. The pain may recur frequently or remain absent for months. 2.Some male and female adolescents complain of chest pain caused by breast masses (mastalgia). These tender masses may be cysts (in postpubertal girls) or may be part of normal breast development in pubertal boys and girls. 3.Pleurodynia (devil's grip), an unusual cause of chest pain caused by coxsackievirus infection, is characterized by sudden episodes of sharp pain in the chest or abdomen. 4. Herpes zoster is another unusual cause of chest pain.

Miscellaneous… 5.Spontaneous pneumothorax and pneumomediastinum are serious but rare respiratory causes of acute chest pain in children; children with asthma, cystic fibrosis, or Marfan syndrome are at risk. Inhalation of cocaine can provoke pneumomediastinum and pneumothorax with subcutaneous emphysema. 6.Pulmonary embolism, although extremely rare in children, has been reported in female adolescents who use oral contraceptives or have had elective abortions. It has also been reported in male adolescents with recent trauma of the lower extremities and in children with shunted hydrocephalus. Affected patients usually have dyspnea, pleuritic pain, fever, cough, and hemoptysis. 7. Hyperventilation can produce chest discomfort and is often associated with paresthesia and lightheadedness

CARDIOVASCULAR CAUSES OF CHEST PAIN Cardiovascular disease is identified as the cause of pediatric chest pain in <4% of cases. Cardiac chest pain may be caused by ischemic ventricular dysfunction, pericardial or myocardial inflammatory processes, or arrhythmias. A typical anginal pain is located in the precordial or substernal area and radiates to the neck, jaw, either or both arms, back, or abdomen. The patient describes the pain as a deep, heavy pressure; the feeling of choking; or a squeezing sensation. Exercise, cold stress, emotional upset, or a large meal typically precipitates anginal pain.

Ischemic Myocardial Dysfunction Congenital Heart Defects Severe obstructive lesions, such as aortic stenosis (AS), subaortic stenosis, severe pulmonary stenosis (PS), and pulmonary vascular obstructive disease (Eisenmenger's complex), may cause chest pain. Mild stenotic lesions do not cause ischemic chest pain. Chest pain from severe obstructive lesions results from increased myocardial oxygen demands from tachycardia and increased blood pressure. Therefore, the pain is usually associated with exercise and is a typical anginal pain, as previously discussed.

Cardiac examination often reveals a loud heart murmur best audible at the upper right or left sternal border, usually with a thrill. The ECG usually shows ventricular hypertrophy with or without “strain” pattern. Chest x-ray films may be abnormal in patients with AS and PS and are definitely abnormal in patients with Eisenmenger's syndrome, with a marked prominence of the main pulmonary artery segment. Echocardiography and Doppler studies permit accurate determination of the type and severity of the obstructive lesion. An exercise ECG may aid in the functional assessment of severity.

Mitral Valve Prolapse Chest pain associated with mitral valve prolapse (MVP) has been reported in about 20% of patients. The pain is usually a vague, nonexertional pain of short duration, located at the apex, without a constant relationship to effort or emotion. The pain is presumed to result from papillary muscle or left ventricular endomyocardial ischemia. Nearly all patients with Marfan syndrome have MVP. Midsystolic click with or without a late systolic murmur. ECG may show T-wave inversion in the inferior leads. Two-dimensional echo findings of MVP in adults are well established, but diagnostic echo findings for MVP have not been established in children.

Cardiomyopathy Hypertrophic and dilated cardiomyopathy can cause chest pain from ischemia, with or without exercise, or from rhythm disturbances. Cardiac examination reveals no diagnostic findings, but the ECG or chest x-ray films are abnormal, leading to further studies. Echo studies are diagnostic of the condition. Coronary Artery Disease Coronary artery anomalies rarely cause chest pain. They include rare cases of anomalous origin of the left coronary artery from the pulmonary artery/ALCAPA (usually symptomatic during early infancy), coronary artery fistula, aneurysm or stenosis of the coronary arteries as a result of Kawasaki's disease, or coronary insufficiency secondary to previous cardiac surgery involving the coronary arteries or the vicinity of these arteries.

Coronary Artery Disease… - Typical of anginal pain. - Cardiac examination may be normal or may reveal a heart murmur (systolic murmur of mitral regurgitation or continuous murmur of fistulas). - The ECG may show myocardial ischemia (ST-segment elevation) or old myocardial infarction. - CXR films may reveal abnormalities suggestive of these conditions. An abnormal exercise ECG further indicates myocardial ischemia. - Although echo can be helpful, coronary angiography is usually indicated for the definitive diagnosis.

Cocaine Abuse Even children with normal hearts are at risk of ischemia and myocardial infarction if cocaine is used. Cocaine blocks the reuptake of catecholamines in the central nervous system and peripheral sympathetic nerves. An increase in the sympathetic output and circulating level of catecholamines causes coronary vasoconstriction. Cocaine also induces the activation of platelets in some but not all patients. The resulting increase in HR and BP, increase in myocardial oxygen consumption, possible increase in platelet activation, and myocardial electrical abnormalities may collectively produce anginal pain, infarction, arrhythmias, or sudden death. Aortic Dissection or Aortic Aneurysm Aortic dissection or aortic aneurysm rarely causes chest pain. Children with Turner's, Marfan, and Noonan's syndromes are at risk.

Pericardial or Myocardial Disease Pericarditis Irritation of the pericardium may result from inflammatory pericardial disease; pericarditis may have a viral, bacterial, or rheumatic origin. In a child who had recent open-heart surgery, the cause of the pain may be postpericardiotomy syndrome. Older children with pericarditis may complain of a sharp, stabbing precordial pain that worsens when lying down and improves after sitting and leaning forward. The ECG may reveal low QRS voltages and ST-T changes, and chest x-ray films may show varying degrees of cardiac enlargement and changes in the cardiac silhouette. Diagnosis of pericardial effusion with or without tamponade can be accurately made by echo examination.

Myocarditis Acute myocarditis often involves the pericardium to a certain extent and can cause chest pain. Examination may reveal fever, respiratory distress, distant heart sounds, neck vein distention, friction rub, and paradoxical pulse. Chest x-ray films and the ECG may suggest the correct diagnosis, which can be confirmed by echo examination. Arrhythmias Chest pain may result from a variety of arrhythmias, especially with sustained tachycardia resulting in myocardial ischemia. Even without ischemia, children may consider palpitation or forceful heartbeats as chest pain. When chest pain is associated with dizziness and palpitation, a resting ECG and a 24-hour ambulatory ECG using a Holter monitor should be obtained. Alternatively, a telephone transmission device may be used to relay the ECG while the patient experiences symptoms.

HISTORY OF PRESENT ILLNESS AND NATURE OF THE PAIN The following are some examples of questions to ask: What seems to bring on the pain (e.g., exercise, eating, trauma, emotional stress)? Do you get the same type of pain while you watch TV or sit in class? What is the pain like (e.g., sharp, pressure sensation, squeezing)?

What is the location (e.g., specific point, localized or diffuse), severity, radiation, and duration (seconds, minutes) of the pain? Does the pain get worse with deep breathing? (If so, the pain may be caused by pleural irritation or chest wall pathology.) Does the pain improve with certain body positions? (This is sometimes seen with pericarditis.) How often and how long have you had similar pain (frequency and chronicity)? Have you been hurt while playing, or have you used your arms excessively for any reason? Are there any associated symptoms, such as cough, fever, syncope, dizziness, or palpitation? What treatments for the pain have already been tried?

PAST AND FAMILY HISTORIES After gaining some idea about the nature of the pain, the clinician should focus on important past and family histories. Examples of questions follow: Are there any known medical conditions (e.g., congenital or acquired heart disease, cardiac surgery, infection, asthma), and is the patient on any medications, including birth control pills? Has there been recent heart disease, chest pain, or a cardiac death in the family? Does any disease run in the family? What is the patient or family member concerned about? Has the child been exposed to drugs (cocaine) or cigarettes?

PHYSICAL EXAMINATION A careful general physical examination should be performed before the focus turns to the chest. The clinician should note whether the child is in severe distress from pain, is in emotional stress, or is hyperventilating. The skin and extremities should be examined for trauma or chronic disease. Bruising elsewhere on the body may indicate chest trauma that cannot be seen. The abdomen should be carefully examined, because it may be the source of pain referred to the chest.

The chest should be carefully inspected for trauma or asymmetry. The chest wall should be palpated for signs of tenderness or subcutaneous air. Special attention should be paid to the possibility of costochondritis by palpating each costochondral and chondrosternal junction. The heart and lungs should be auscultated for arrhythmias, heart murmurs, rubs, muffled heart sounds, gallop rhythm, crackles, wheezes, or decreased breath sounds.

FURTHER LABORATORY INVESTIGATION Drug screening for cocaine may be worthwhile in adolescents who have acute, severe chest pain and distress with an unclear cause. REFERRAL TO CARDIOLOGISTS The following are some of the indications for referral to a cardiologist for cardiac evaluation: When chest pain suggests anginal pain, when there are abnormal findings in the cardiac examination, or when abnormalities occur in the chest x-ray films or ECG, cardiac referral is clearly indicated. The examiner's ability to recognize common innocent heart murmurs minimizes the frequency of such referrals. When there is a positive family history for cardiomyopathy, long QT syndrome, or another hereditary disease commonly associated with cardiac abnormalities, cardiology referral may be indicated. High levels of anxiety in the family and patient and a chronic, recurring nature of the pain are also important reasons for referral to a cardiologist.

Management Treatment is directed at correcting or improving the cause. Costochondritis can be treated by reassurance and occasionally by acetaminophen or nonsteroidal anti- inflammatory agents. Most musculoskeletal and nonorganic causes of chest pain can be treated with rest, acetaminophen, or nonsteroidal anti-inflammatory agents. If respiratory causes of chest pain are found, treatment is directed at those causes.

If serious cardiac anomalies, arrhythmias, or exercise-induced asthma is diagnosed, a referral is made to the cardiology or pulmonary service. Cardiac evaluation requires further specialized studies such as echo, an exercise stress test, Holter monitoring, or even cardiac catheterization. Depending on the cause, treatment may be surgical or medical. The correct therapy of acute cocaine toxicity has not been established. Calcium channel blockers (nifedipine, nitrendipine), β-adrenergic blockers, nitrates, and thrombolytic agents have resulted in varying levels of success. The use of beta blockers is controversial; they may worsen coronary blood flow.

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