Common Benign Pediatric Presentations Mimicking Cardiopulmonary Emergencies in the Emergency Department

1. Why Benign Conditions Mimic Cardiopulmonary Emergencies

Many benign pediatric events are abrupt enough to bring great shock to parents who perceive the change as catastrophic. Furthermore, because the sudden change can involve a sudden appearance or disappearance of color, a sudden change in a child’s tone, a sudden onset of chest pain or a sudden collapse, the child’s and family’s immediate concern is for a severe acute event, possibly even a sudden cardiopulmonary arrest, and it is in these circumstances that the family seek a full assessment and exclusion of the worst possible (catastrophic) explanations in the emergency department for events that have been short-lived and have then resolved, and for which the child’s description of events may be misinterpreted by others, such as the description of the baby ‘stopping breathing’ for a short time before regaining normal respiratory activity, children with cyanotic breath-holding spells attracting misinterpretation as epileptic seizures or even as a cardiac arrest, children with vasovagal syncope who collapse due to autonomic dysfunction with decreased venous return and decreased cerebral perfusion, causing a change in respiratory pattern and possible change in color or level of consciousness, children with severe pain from a sharp severe stabbing sensation in their chest wall which they describe as having ‘heart pain’ even though it is not due to cardiac ischemia and has not caused a decrease in oxygen delivery to the tissues, children with cardiac murmurs often of high flow state, usually localized to one area of the heart, which can be of great concern as they are heard in the same general area as the more serious cardiac murmurs [1,2,3,12,16,21,30].

Understanding the benign mimics of critical illness is important for the assessment of a child with acute symptoms of possible cardiopulmonary disease. Transient autonomic dysfunction and physiologic sinus tachycardia are common reasons children feel a racing heart. However, in some instances, this can cause a decrease in venous return with subsequent decrease in cerebral perfusion leading to changes in respiratory pattern. Rarely, a child can even change color and appear to stop breathing for a short period of time. In typical presentations without red flags, these events are considered to be benign and not a result of primary cardiopulmonary failure. Other causes of severe sharp chest wall pain can give great concern to families and clinicians alike. Inflammation of the chest wall as well as Precordial Catch Syndrome can cause severe pain localized to the chest wall. These are common causes of severe ischemic-like pain that are often misdiagnosed. Other causes of a child feeling as though their heart is racing include high cardiac output states, fever, anxiety, dehydration, as well as isolated beats. All of these causes of acute symptoms of disease can be worked up in the emergency department and are generally not life-threatening. The key for the clinician is to use their best clinical judgment to work up the child with acute symptoms of possible cardiopulmonary disease. The clinician must have a working knowledge of the causes of acute symptoms of benign disease in order to work up children with symptoms of possible cardiopulmonary disease in an appropriate fashion [12,16,17,18,19,20,21,22,23,24,30,31,32,33,34,35,36,37,38,39].

There are many normal events that occur in children frequently and may require a diagnosis. If all of the red flags for serious illness have been ruled out by the clinician, a correct diagnosis can be made. Stabilizing the child and screening for the high risk features in the emergency setting would be a good first step rather than immediately trying to make a diagnosis [1,2,3,4,5,6,7,8,9,10,11,16,17,18,19,20,21,22,23,24,30,31,32,33,34,35,36,37,38,39].

2. A Shared Step-by-Step Emergency Department Framework

Step 1: Assess for instability in the child with acute symptoms that may represent a cardiopulmonary emergency. Note that the child must be hemodynamically stable and back to baseline in order to fall into a benign pathway. If during the assessment of a child with symptoms that could place them in the differential for BRUE, syncope, chest pain, murmur, or palpitations, the child is found to have symptoms that would categorize them as having an unstable cardiopulmonary condition (i.e., persistent respiratory distress, development of cyanosis, hypotension, abnormal pulses, signs of severe dehydration, altered mental status, seizure, collapse), then the child should be managed as an unstable cardiopulmonary emergency until proved otherwise [1,2,3,8,11,12,16,21].

Step 2: Formulate a scenario of what may be wrong with the child. The history usually trumps the test results. For instance, for a child with BRUE (brief resolved unexplained event), it is very important to take a complete history and formulate a scenario of what may have happened to that child. For example, a 4-month-old infant with BRUE of crying and becoming blue and floppy while being fed could have a number of differential diagnoses. The key would be to get a history of the child’s feedings, have there been any episodes of choking or cyanosis, has the child had a fever, has anyone in the house been sick, and has there been any trauma to the child. A child with syncope would be questioned about the length of time he or she has been standing before losing consciousness. Does the child get hot or miss meals? Was there any physical exertion before the loss of consciousness? Was there an emotional trigger for the loss of consciousness? Was there a prodrome to the loss of consciousness and is there a family history of heart disease. A child with chest pain could have a variety of causes of pain and the questions would be to list out the relationship of the child’s position and the severity and quality of the pain [1,2,3,4,12,16,17,18,19,20,21,22,23,24].

Step 3: Identify red flags for each of the above diagnoses and how they can affect management and testing. Typically, a child’s presentation will help guide the clinician to possible testing and diagnosis. However, there are often red flags that can be picked out from the history or physical that may suggest a more serious cause for a child’s presentation. Across these presentations, red flags include exertional syncope or chest pain, syncope while supine, absence of prodrome, abnormal or persistent vital sign abnormalities, abnormal cardiac examination beyond an innocent-sounding murmur, abnormal ECG, recurrent clustered episodes, family history of cardiomyopathy or sudden unexplained death, prolonged loss of consciousness, post-event confusion, poor feeding in infants, diaphoresis, focal neurologic findings, or cyanosis not explained by the presenting event. These findings require a high level of suspicion for serious disease and may require immediate cardiac, neurologic, infectious, or pulmonary evaluation with observation or admission when clinically indicated [1,2,3,4,8,11,16,17,18,19,20,21,22,23,24,30,31,32,33,34,35,36,37,38,39].

Step 4: Testing for Specific Questions (Selective Testing Rather than Reflex Testing). There are very few diseases reviewed in this article for which routine testing is indicated for children who are well-appearing, stable, and have returned to baseline. Tests for BRUE in lower-risk children, vasovagal syncope, benign chest pain, and innocent heart murmurs have very low diagnostic yields [1,2,3,4,5,6,7,8,9,16,17,18,19,20,21,22,23,24,30,31,32]. The screening ECG is one of the few tests that is of higher yield in children with suspected cardiac mimics [5,8,11,14,15,16,17,18,19,20,21,22,23,24,30,31,32,33,34,35,36,37,38,39] and testing should be for specific questions or sets of questions that the clinician has for the child’s symptoms of and/or evidence of disease due to anemia, iron deficiency, myocarditis, arrhythmias, infections, pulmonary disease, and/or structural heart disease.

Step 5: Treatment / Management. Supportive treatment for all of the above-mentioned conditions, that can cause severe anxiety in both children and their parents, is usually sufficient when the child remains in a typical low-risk pattern. Children with breath-holding spells typically complete a spell within a few minutes of it having started, and do not require any antiepileptic treatment for their episodes. Children with syncope of reflex origin are treated with supine positioning with oral rehydration and advised on triggers to try to avoid them in the future. Children with musculoskeletal induced chest pain are treated for the pain and given temporary activity modification when needed. Children with an innocent murmur require documentation of findings with planned follow-up for heart evaluation as an outpatient [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39].

Step 6: The disposition of the child in relation to going home with the parents and needing follow-up with primary care or a subspecialist (or observation) in a hospital. The clinician must consider the child’s physiology and degree of risk for future deterioration. A child with a low-risk BRUE who is back to baseline, has a low-risk history and exam, and no abnormal findings on ECG, if obtained, would go home with parental instructions for follow-up with primary care or subspecialist (or observation) as needed. A child with an uncertain diagnosis, recurrent events, very young age, or a family that cannot recognize deterioration would benefit from observation or admission [1,2,3,4,5,6,7,8,9,10,11,16,17,18,19,20,21,22,23,24,30,31,32,33,34,35,36,37,38,39].

3. Lower-Risk Brief Resolved Unexplained Event

The term BRUE (brief resolved unexplained event) is used as a framework for evaluation of acute unexplained events in infants younger than 1 year of age. These events are now resolved and in the absence of another explanation occurred suddenly and lasted briefly. No signs of cardiopulmonary, neurologic or infectious instability have been found after evaluation of the event. The main aspect of the evaluation of a BRUE is risk stratification and not a broad etiologic search [1,2,3,4,8,11].

There are many serious diseases of infants that present with unexplained episodes in seemingly healthy infants. There are many occasions in pediatric practice when infants present with apparently unexplained episodes of behavior. By placing these infants on a BRUE evaluation pathway, there is a risk of delaying the diagnosis of and treatment of serious and life-threatening diseases (e.g. bronchiolitis with cough and/or wheezing; gastroesophageal reflux with emesis with witnessed aspiration; seizures with episodes of tremors etc.). In the above circumstances the infant does not have a BRUE and should be managed as if they had the underlying illness for that episode (e.g. bronchiolitis in an otherwise healthy infant would be managed as a child with bronchiolitis with a focus on the cough and/or wheezing and possible hypoxia and need for oxygen) [1,2,3,4,8].

Step 2: Is the child lower risk? The American Academy of Pediatrics (AAP) outlined the criteria for lower-risk children with BRUE in a 2016 publication. In BRUE assessment, it is most important to remember that the child must be greater than 60 days of age, a gestational age of greater than 32 weeks with a corrected age of greater than 45 weeks, this being the child’s first event with duration of less than 1 minute, and no CPR performed by a trained medical provider. There also should be no concerning historical or physical exam findings. Lower-risk BRUE children have very low rates of serious underlying illness. Studies on BRUE are ongoing and are increasingly showing that indiscriminate testing is of poor yield. Broadening a categorization to “higher risk” is too nonspecific [1,2,3,4,5,6,7,8,9,10,11].

Step 3: A focused physical examination. The history is best gathered in categories to allow a complete and organized physical examination of the infant. The categories for assessment of an infant with a potential BRUE include: 1) inattention or lethargy or altered mental status; 2) change in tone or abnormal movements; 3) increased respiratory effort or decreased perfusion or decreased breath sounds; 4) signs of dehydration or poor perfusion; 5) cutaneous stigmata or signs of trauma; 6) obstruction of the upper airway; and 7) cardiac anomalies. Additionally, one should gather information about the infant’s feeding history (e.g., choking or gagging during feeding), the infant’s sleep position and environment, and the history of recent illness of the infant and his or her contacts (sick contacts). Point-of-care glucose determination may be considered when the event was prolonged, when the infant is very young or small, or when poor feeding or irritability raises concern for hypoglycemia [1,2,3,4,5,8,11].

Step 4: The Child Appears to Be in Good Health – Observation (and a Feeding Trial) Is Appropriate. Once it is clear that a child has a BRUE and is categorized as lower risk, short observation of the child and his or her pulse oximetry with repeat vital signs is appropriate. In addition, the child should be offered a feeding trial to determine if the child can be fed successfully. For the child classified as having a lower risk BRUE, routine complete blood count, blood culture, CSF examination, chest radiograph, neuroimaging, echocardiogram, EEG, and a variety of metabolic studies and acid suppression tests are of no value in the evaluation of a child with a BRUE. A 12-lead ECG may be obtained selectively because it is low burden and may detect rare channelopathy or pre-excitation, but indiscriminate ECG testing in otherwise lower-risk BRUE has low diagnostic yield [1,2,5,8].

Step 5: Parent-Centered Care Interventions—Caregiver-Focused Actions. Even if an infant’s BRUE is determined to be a lower-risk event, parents and caregivers are not going to be happy with a diagnosis of an “unexplained resolved event” in their infant. Such an explanation and counseling to parents and caregivers about sleep positions and sleep environments to reduce risk of SIDS is essential for appropriate care of the infant. Parents and caregivers also must be given specific instruction on when to return their child to the ED for evaluation of new or worsening symptoms. In addition, every parent and caregiver should be offered infant CPR resources should the need arise [1,2,8,11].

Step 6: When does an infant need to be admitted or workup for other causes of an unexplained event? The key is to think of other causes of unexplained events for an infant who does not fall into the lower-risk category for BRUE. This includes: 1) Recurrent events; 2) Age < 60 days; 3) Concerning social situation; 4) Family history of sudden unexplained death; 5) Abnormal physical exam; 6) Poor feeding; 7) Nonreassuring history. Infants in this category would no longer be considered to be in the lower-risk category for an unexplained event and would need to be admitted and worked-up for other causes of an unexplained event [1,2,3,4,8,9,10,11].

4. Breath-Holding Spells

Breath-holding spells are classic benign events that can be mistaken for apnea, seizure, or arrhythmic collapse. They usually occur in healthy infants and toddlers between 6 and 24 months of age and are generally categorized as cyanotic or pallid [12,13]. Cyanotic spells usually follow anger, frustration, or crying. The child cries, exhales, stops breathing transiently in expiration, becomes cyanotic, and may go limp or briefly lose consciousness. Pallid spells usually follow pain or sudden fright and are believed to reflect a vagally mediated cardiac inhibitory reflex with transient bradycardia or asystole, pallor, and brief cerebral hypoperfusion [12,13,14,15]. The event is dramatic because autonomic instability transiently alters oxygenation or cerebral perfusion, but the child is usually normal between events and recovers rapidly.

Step 1. Recognize the trigger-pattern-recovery sequence. The history is the diagnostic anchor. A typical spell has a clear precipitating event, a brief stereotyped sequence, and complete recovery without prolonged postictal confusion. That pattern distinguishes breath-holding from epileptic seizure, myocarditis-associated collapse, malignant arrhythmia, or ongoing respiratory disease. If the history lacks an emotional or pain trigger, if the event occurs during sleep, if recovery is prolonged, or if there are focal neurologic findings, the diagnosis should be reconsidered [12,13,16].

Step 2. Manage the active event safely. Place the child supine or in a side-lying safe position, remove nearby hazards, and do not place anything in the mouth. Oxygen is not usually necessary once the pattern is recognized and the event is already resolving, but it may be given if the child remains cyanotic longer than expected while reassessment is underway. The physiologic goal is simply to protect against injury while cerebral perfusion and normal respiratory effort return spontaneously [12,13].

Step 3. Perform a focused examination after recovery. The clinician should document normal mental status, normal perfusion, absence of focal neurologic deficits, and a normal cardiopulmonary examination. A 12-lead electrocardiogram is reasonable when the history is atypical, spells are frequent, pallid episodes are recurrent, or there is a family history of arrhythmia or sudden death, because prolonged QT syndrome and rare rhythm disorders can occasionally mimic breath-holding spells [12,13,16].

Step 4. Evaluate for iron deficiency when the pattern is typical but spells are recurrent or severe. Multiple studies and reviews support an association between breath-holding spells and iron deficiency, and iron therapy reduces spell frequency even in some children without frank anemia [12,13,14,15]. A practical emergency department approach is to check hemoglobin or arrange outpatient complete blood count and ferritin testing when episodes are frequent, prolonged, or developmentally disruptive. The pathophysiologic rationale is that iron deficiency may alter autonomic regulation and catecholamine metabolism, increasing event susceptibility [12,13,14,15].

Step 5. Treat supportively and counsel clearly. The immediate ED intervention is reassurance based on physiology, not dismissal. Parents should be told that the child is not voluntarily “holding the breath until dangerous” but rather experiencing a short autonomic reflex event. They should be instructed to place the child safely on the side or back, avoid shaking or mouth interventions, allow recovery, and seek urgent care if the episode is prolonged, occurs without a trigger, is associated with persistent abnormal behavior, or looks different from prior spells. If iron deficiency is found, treatment should follow standard pediatric supplementation and outpatient follow-up [12,13,14,15].

5. Reflex and Vasovagal Syncope

Syncope in children is typically a benign, self-limited, event that occurs as a result of reflex activity. Vasovagal syncope is the most common form of syncope in the pediatric population, and it is typically caused by a transient imbalance in autonomic control of heart rate and blood pressure resulting in vasodilation and/or bradycardia and subsequent decreased cerebral perfusion due to decreased cardiac preload as a result of venous pooling. The families of children who experience syncope describe them as events that occur without warning and are often described as the child “fainting for no reason”. The challenge in the evaluation of syncope is to determine if the child’s event(s) were purely vasovagal in origin or if they could be indicative of a serious condition such as cardiogenic syncope, seizure, or even pulmonary embolism [16,17,18,19,20].

Syncope in children can be a life-threatening condition and therefore it is very important to distinguish between a benign cause of syncope (vasovagal) and a potentially life-threatening cause of syncope (cardiac). A child with vasovagal syncope will typically have a history of prolonged standing, exposure to heat, dehydration, missing of meals, strong emotions, pain or sight of blood prior to the loss of consciousness. The typical prodrome for a child with vasovagal syncope is lightheadedness, nausea, feeling of tunnel vision, muffled hearing or feeling of diaphoresis. The loss of consciousness is typically a short event and the child will immediately and completely return to a baseline mental status after the event. Patients with reflex syncope have obvious orthostatic stress and therefore, syncope that occurs with exertion, while the patient is supine or has no prodrome, should increase suspicion for an arrhythmia or structural heart disease [16,17,18,19,20].

Step 2: Assess the child’s hemodynamics (supply of blood and blood pressure) and give the child’s supine position and perform any ED interventions needed to assess and treat decreased perfusion and volume. Children with syncope due to decreased preload (ie, dehydration) will improve rapidly when they are placed in a supine position. In addition, make sure to rule out any injury from a fall and assess for signs and symptoms of poor hydration. Although a child’s heart rate and blood pressure can give you some information regarding orthostatic changes and decreased volume, this information is not diagnostic by itself [16,17,18,19,20].

Step 3: Get an ECG. As previously stated most pediatric syncope is caused by benign causes but as previously stated a prolonged QT, a WPW pattern, signs of hypertrophic cardiomyopathy, a Brugada pattern, conduction disease, pre-excitation of the ventricles (e.g. Wolff-Parkinson-White), ectopic foci, etc. can be diagnosed with an ECG. A normal neurologic exam and the lack of need for neuroimaging and routine broad lab work in a child with syncope who has a normal examination and history of benign cause for syncope is expected. There are, however, a few instances in which the syncope would warrant a more in-depth workup such as anemia, pregnancy in an adolescent, known or suspected intoxication or infection, or marked electrolyte imbalances [16,17,18,19,20].

Children with vasovagal syncope can benefit from simple interventions, including being positioned supine and receiving oral rehydration fluids or a small IV bolus of fluid to rehydrate an already dehydrated child. Older children can even be taught some counter-pressure maneuvers to prevent syncope, as well as some pre-emptive maneuvers to ward off a fainting spell, such as sitting or lying down at the beginning of symptoms, crossing the legs, or tightening up the leg and abdominal muscles [16,17,18,19,20].

Step 5: Final Disposition of Pediatric Patient with Syncope. Most pediatric patients with syncope have vasovagal syncope (reflex), which is a benign condition and they will do very well. In contrast, syncope caused by a cardiac disorder requires urgent evaluation because the underlying disease may carry a risk of sudden cardiac death. In some instances, the very first episode of syncope in a child can be the child’s last. Therefore, the management of the child with syncope in the ED involves trying to find the cause of the syncope, determine if there are any red flags, and then make a disposition for the child based on whether there are red flags present for that child. Most children with typical vasovagal syncope (reflex) with no abnormal cardiac findings have a very good prognosis and no problems as they grow up. In fact, most children with typical faints (vasovagal syncope) have no problems at all and can go on to lead normal lives [16,17,18,19,20].

6. Benign Chest Pain

Chest pain is one of the most feared pediatric complaints, and in the majority of cases, they are noncardiac in nature [21,22,23,24]. Most of the benign patterns of chest pain in the pediatric population present with similar symptoms of serious life-threatening condition such as a cardiopulmonary emergency, and these complaints are misinterpreted by families of the child as serious life-threatening condition such as heart attack or pulmonary problem, especially if the child’s pain is sudden in onset, sharp in nature, or worsens with breathing. The pathophysiologic reasons for these patterns of chest pain are from the chest wall nociception or from pleuritic type of discomfort. These complaints are often misinterpreted by families as cardiopulmonary emergencies, such as heart attacks in children.

Step 1: Determine if pain is of non-cardiac origin (benign). Children with chest pain can present in many different forms. Most cases of chest pain in children are of non-cardiac origin [21,22,23,24]. Sometimes children with non-cardiac chest pain worry themselves and their families because they believe that the pain could be a sign of serious illness of the heart or lungs. Many children with non-cardiac pain report sudden onset of pain. They may describe the pain as sharp or burning. A child with nociception from the chest wall interprets this pain as serious illness of the heart or lungs. A child with non-cardiac origin of chest pain will often report pain that is brief in duration and is sharp in nature. The pain is typically localized to the area of pain and is reproducible. This means that the child can make you reproduce the pain with physical exam. You may also note that the child’s pain is associated with their arm or in certain positions of their child. The pain can also be worse with deep inspirations. All of these characteristics are clues that the child’s pain is of non-cardiac origin and you can lead the emergency physician to choose less expensive tests to rule out other causes of chest pain in children rather than proceeding with expensive cardiac testing as the first step in evaluation of the child with chest pain.

Step 2: Determine if there are any Cardiopulmonary Red Flags that may indicate a more serious cause of pain. These include: pain that occurs with exercise, true syncope, palpitations that occur prior to the onset of pain, persistent shortness of breath or dyspnea, a fever with a very ill-appearing child, abnormal vital signs or hypoxemia, significant trauma of any kind, known heart disease, a family history of sudden unexplained death, an abnormal cardiac exam, or pain that feels or is described as being caused by myocarditis or pericarditis (i.e., a viral illness that started with a sore throat or other upper respiratory infection symptoms several weeks prior and has been accompanied by several days of tachycardia [fast heart rate] or pain that improves when leaning forward) [21,22,23,24].

Step 3: Physical examination of the chest wall and cardiopulmonary system. Look for features that can reproduce the child’s pain. Examination of the chest wall for localized tenderness of costochondral junctions as well as assessment of respiratory effort, auscultation for wheezing, crackles, pericardial rub or new pathologic murmurs. Document child’s ability to move and breathe comfortably between episodes of pain. A child with a normal examination and a classic musculoskeletal story has a high degree of reassurance [21,22,23,24,28,29].

Step 4: Consider selected testing. ECGs are indicated for children with exertional chest pain, children who have had syncope or palpitations prior to the onset of the chest pain, children with findings of cardiopulmonary disease or other unusual physical examination findings. Routine troponin testing for children with noncardiac chest pain is not recommended because this condition is rarely associated with myocardial injury [21,22,23,24]. Chest radiographs are indicated for children with suspicion of a pneumothorax, pneumonia, chest trauma or other structural anomalies of the chest. A chest radiograph is not indicated for children with a diagnosis of precordial catch syndrome or children with episodic, reproducible, localized pain of the chest wall [21,22,23,24].

Step 5: Diagnosis – Provide Treatment based on Cause and Instruction for Return to Activity for future Episodes. Children with costochondritis may benefit from a prescription of ibuprofen or acetaminophen. Additionally, the application of heat, particularly warm pads made in a microwave, and reduction of activity level may help alleviate the discomfort. Precordial catch syndrome is best managed by educating the child and their family that this is a benign condition causing the child to believe that they are having a heart attack. These episodes of pain are of brief duration (seconds to minutes) and typically resolve on their own without resulting in any restriction of activity for the child. However, the child and their family should be instructed as to what to do for future episodes of chest wall discomfort as well as for complaints of persistent pain, shortness of breath, syncope or a general illness with systemic symptoms (e.g. fever) [25,26,27,28,29].

7. Innocent Murmurs

Heart murmurs are found in most children at some time and the vast majority of murmurs heard in children after infancy are innocent. These types of heart murmurs are caused by turbulence of blood flowing across a structurally normal heart. Many factors can cause an innocent heart murmur to be heard. These include the thin chest wall of a child, a child who is ill with a febrile illness, a child who is anxious, a child with a high cardiac output, etc. A great deal of concern is expressed by families and health care workers alike when a murmur is heard coming from the area of the heart of a child. However, the presence of a murmur alone does not mean that the child has a serious cardiopulmonary problem [30,31,32].

Step 1: Are they sick or well? (Global Assessment). This is a simple assessment to establish if the child is critically ill and needs emergency stabilization. The classic example of a critically ill child with heart disease is in a state of heart failure (poor perfusion with abnormal or weak pulses, tachypneic or poor, with signs of fluid overload such as a large hepatomegaly and hypo- or hyper-tension with poor perfusion and possible shock). If the child appears to be sick based on this assessment then leave the benign-murmur pathway and begin urgent stabilization and evaluation [30,31,32].

Step 2. Look for clues that point to an innocent murmur. Typically these are systolic, short and are described as having a ‘vibratory’ or ‘musical’ quality. These types of murmur are localized to a particular area of the chest and can be influenced by the position of the child (e.g. lying down, sitting up). In addition innocent murmurs can vary with a child’s illness (e.g. with a fever) and their emotional state (e.g. anxious child). None of the following features are found in children with innocent heart murmurs: clicks, gallops, cyanosis, abnormal growth and development, symptoms of exertion and abnormal findings of the second heart sound [30,31,32].

Step 3: The policy of referring children with heart murmur for echocardiography based on the quality of the clinical examination alone rather than for a chest radiograph and ECG for all heart murmur is supported in the literature. The clearly well child with a classic innocent murmur does not require urgent echocardiography from the ED. Careful documentation of the findings with an explanation of the innocent nature of the murmur and referral for outpatient follow-up with the child’s primary care clinician or cardiology if the ED examination is not sufficient are the practical actions from the ED for such children [30,31,32].

Step 4: When a murmur is not innocent- When a diastolic murmur is heard, a holosystolic murmur is heard, a very loud or harsh murmur is heard, the child is cyanotic and has poor peripheral circulation, the child has grown and developed abnormally, the child has abnormal exercise tolerance, the child has chest pain with exertion, the child has had syncope, then more evaluation is required and the child will be referred to a pediatric cardiologist. The key is the combination of the murmur and the child’s clinical presentation or context. The sound of the murmur alone does not determine risk for cardiac disease [30,31,32].

8. Benign Palpitations and Isolated Ectopy

Palpitations are a very common symptom in children and adolescents. Typically they are caused by sinus tachycardia, usually secondary to anxiety, but also by situations of stress such as fever or dehydration. Other causes are consumption of stimulants such as caffeine or even legal and illegal drugs, as well as isolated premature atrial or ventricular beats (PACs or PVCs) which are typically felt as a pause following a skipped beat. These typical arrhythmias are not usually associated with unstable arrhythmias and the child’s perception of an irregular rhythm is not typically associated with any compromise of normal cardiac output. Typically children are aware of the normal increase in heart rate as well as the individual ectopic beats and their typical compensatory pause [33,34,35,36,37,38,39].

A helpful approach to managing children with palpitations is to attempt to classify their symptoms into a few different categories such as racing heart, skipped beats or irregular pounding (persistent tachycardia). Often children report being aware of an occasional beat at rest that they perceive as skipping a beat or as a ‘thump’. Typically these are isolated ectopic beats, which are benign in children. Other children report episodes of sustained rapid pounding that occur suddenly and then cease as suddenly. This would be suspicious for supraventricular tachycardia (SVT) [33,34,35,36,37,38,39].

Step 2: Evaluate 12-lead ECG for underlying cause of palpitations. It is not uncommon for children with palpitations to have a normal ECG between episodes of palpitations. Other ECG findings that may be seen include evidence of pre-excitation, a prolonged QT interval, atrial or ventricular ectopy, conduction disturbance, or evidence of structural heart disease [33,34,35,36,37,38,39].

Step 3: Determine if there are factors that are causing the child’s palpitations that are benign in nature and must be addressed in order to alleviate their child’s symptoms. Common factors that can cause palpitations in children include: a child’s fever or their child’s dehydration from losing too many fluids from diarrhea and/or vomiting; consumption of too much caffeine found in soft drinks such as cola and energy drinks; as well as prescribed or over the counter medications that a child may be taking for the treatment of attention (ADHD) as well as for other medical reasons. Other benign factors that may cause palpitations include: a child’s anxiety or their child’s recent lack of sleep and/or their child’s recent illness from a viral syndrome [33,34,35,36,37,38,39].

Step 4: Treatment- Children with isolated premature beats and a normal examination and ECG in the ED do not need any treatment. They and their families should be given an explanation for the child’s palpitations and discussed as to the potential causes and ways to avoid them. The child and their family should have a follow-up appointment if the child’s palpitations persist. Recurrent palpitations in a child where no events were captured by the child and their family in the ED are much better worked up with ambulatory monitoring rather than with antiarrhythmic medications [33,34,35,36,37,38,39].

Step 5: Determine whether palpitations are or are not benign based on the clinical assessment. Children with palpitations and syncope, palpitations that occur with physical activity, palpitations and chest pain, palpitations in children with a family history of sudden death, and children with palpitations and abnormal physical examination findings and/or an abnormal ECG require an immediate assessment and workup by a pediatric cardiologist or other specialist. Palpitations in these children are often associated with a serious problem affecting the heart rhythm that could potentially decrease cardiac output and result in serious harm, as opposed to the benign isolated ectopic beats found in most children with palpitations [33,34,35,36,37,38,39].

9. Cross-Cutting Red Flags and Practical Disposition

In all of the mimics listed above, there are a number of clinical features that will typically prompt the clinician to reject the diagnosis of a benign mimic.

• abnormal vital signs that do not normalize
• hypoxemia
• poor perfusion
• hepatomegaly
• exertional symptoms
• abnormal ECG
• family history of sudden unexplained death
• recurrent clustered events
• prolonged post-event confusion
• focal neurologic findings
• toxic appearance
• persistent chest pain or dyspnea
• failure to return to baseline [1,2,3,4,5,6,7,8,9,10,11,16,17,18,19,20,21,22,23,24,30,31,32,33,34,35,36,37,38,39]

These conditions are typically benign when they present within typical limits for a given age [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39].

A practical discharge process should include four elements:

• Name the working diagnosis and explain why it is believed to be benign.
• Explain the reasons for which a dangerous cardiopulmonary condition was considered, but which are less likely with the given information.
• Give return precautions in a format that families can apply to a child’s symptoms: an event that is longer, different, exertional, associated with color change that does not resolve, chest pain during exercise, fainting without warning, shortness of breath, or inability to return to baseline [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39].
• Plan follow-up care. Some of the most common benign conditions for which children visit the emergency department need to be followed by primary care. For instance, a BRUE or a child with a murmur of ‘low concern’ might need to be followed by a pediatrician. Children with breath-holding episodes and iron deficiency would likely be followed by primary care, with hematology involvement if anemia is severe, recurrent, or refractory. Children with recurrent syncope or palpitations (especially with ‘worrisome features’ of history, physical examination or ECG) would be best followed by a pediatric cardiologist. Children with recurrent chest pain that has not been so easily reassured would likely be followed by a pediatric cardiologist, or by a primary care physician (if the cardiologist is not readily available) [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39].

A clear, specific, and complete explanation of the discharge plan to families of patients with benign conditions that are being managed within the ED will help to improve safety for patients and their families and to improve the efficiency of the ED in managing the volume of patients with benign conditions that are being managed on an emergency basis and that remain within expected limits for the diagnosis. Families must understand that the clinician did not find evidence of a serious life-threatening cardiopulmonary condition and why the clinician believes that condition to be benign. There is potential for a huge mismatch between what the clinician means by ‘everything is okay’ and what families interpret by those very same words [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39].