Ensuring the safety of young athletes is a priority among health care providers. Controversy remains as to the best method of preparticipation screening. The American Academy of Pediatrics and the American Heart Association currently recommend screening with history and physical examination, without routine electrocardiogram or echocardiography. Meticulous conduction of a cardiac focused history and exam during the preparticipation evaluation can help identify those who may be at risk for sudden cardiac arrest. Understanding presenting signs of the most common cardiac diseases is useful in recognizing those in need of a directed cardiac evaluation before sports participation.
Key points
- •
American Academy of Pediatrics and American Heart Association recommendations for preparticipation screening include the use of patient history, family history, and physical examination, with additional testing if there is clinical suspicion of heart disease.
- •
Chest pain or syncope with exercise should raise concern for underlying cardiac disorder; further cardiac evaluation is appropriate.
- •
The athlete’s heart is a constellation of adaptive changes in response to intense athletic training. Differentiation from pathologic cardiac enlargement or hypertrophy can be challenging.
Introduction
More than 7.5 million young people are estimated to participate in competitive high school athletics annually in the United States, with additional youth participate in recreational sports. Though infrequent, sudden cardiac arrest (SCA) in young athletes is devastating to families, schools, and communities when it occurs. With this in mind, local, county, and statewide initiatives for preparticipation sports screening have grown in recent years. Although formal nationwide screening programs exist elsewhere, controversy remains within the United States as to what type of screening is most appropriate for young US athletes. As a result, providing sports clearance is often to the responsibility of individual pediatric practitioners. Given this setting, this article provides:
- •
A summary of current screening philosophies and preparticipation sports guidelines put forth by the American Association of Pediatrics (AAP) and the American Heart Association (AHA)
- •
Pertinent screening history and physical examination with red flags of which to be aware
- •
Understanding of the athlete’s heart
- •
A brief review of the most common disorders associated with risk of SCA
Introduction
More than 7.5 million young people are estimated to participate in competitive high school athletics annually in the United States, with additional youth participate in recreational sports. Though infrequent, sudden cardiac arrest (SCA) in young athletes is devastating to families, schools, and communities when it occurs. With this in mind, local, county, and statewide initiatives for preparticipation sports screening have grown in recent years. Although formal nationwide screening programs exist elsewhere, controversy remains within the United States as to what type of screening is most appropriate for young US athletes. As a result, providing sports clearance is often to the responsibility of individual pediatric practitioners. Given this setting, this article provides:
- •
A summary of current screening philosophies and preparticipation sports guidelines put forth by the American Association of Pediatrics (AAP) and the American Heart Association (AHA)
- •
Pertinent screening history and physical examination with red flags of which to be aware
- •
Understanding of the athlete’s heart
- •
A brief review of the most common disorders associated with risk of SCA
Preparticipation screening
Although it is generally agreed that some form of preparticipation screening should take place before allowing involvement in competitive athletics, controversy remains as to what, if any, testing should be conducted on a screening basis. Advocacy for routine electrocardiogram (ECG) screening of athletes has been well reported in the literature. Corrado and colleagues, based in Italy, are perhaps the most prominent of these advocates. Following the initiation of a mandatory national screening program consisting of personal and family history, pointed physical examination, and screening ECG, they showed an 89% decrease in the annual incidence of sudden cardiac deaths. The principle behind this ECG-based program has been that hypertrophic cardiomyopathy (HCM) and arrhythmogenic right ventricular cardiomyopathy, two leading entities responsible for sudden cardiac death, may be detectable via ECG in asymptomatic individuals.
Furthermore, the use of echocardiographic screening has been proposed by some cardiologists. Proponents cite the ability to obtain technically adequate imaging that can augment history, physical examination, and screening ECG, but also acknowledge that a significant learning curve exists in order to do so. This screening usually takes place outside the medical office using portable echocardiogram devices and obtaining images via a specific screening protocol, which differs from the routine adult or pediatric examination in both comprehensiveness and time in which it is completed.
Despite the merits of the screening programs mentioned earlier, the feasibility of applying universal athletic screening to the population of young athletes in the United States has come into question. Halkin and colleagues argued that implementation of a similar program in the United States is not as economically feasible as implementation of secondary prevention strategies such as lay person cardiopulmonary resuscitation (CPR) and external automatic defibrillator (AED) training. Maron and colleagues refuted the idea that the United States’ screening process was inferior to the Italian process in a study that compared the rate of sudden cardiac death in Minnesota versus Veneto, Italy. Despite the absence of routine ECG screening in Minnesota, there was no statistically significant difference in sudden death rates between the two geographic locations over a similar time span.
The use of history and physical alone in screening US high school athletes has been detailed in recent publications. Roberts and Stovitz reported an incidence of 0.24 sudden cardiac deaths per 100,000 athlete years in a population of high school athletes and proposed that ECG screening may not add any significant benefit to screening. At the same time, they cautioned that their results may not be applicable to other populations that differ demographically in terms of age, race, or gender.
Furthermore, concern has been raised that false-positive screening may lead to additional testing, unnecessary restrictions or therapies, and unwarranted patient and family anxiety. A recent meta-analysis from Tuft’s University School of Medicine reviewed the literature between 1950 and 2010 to determine the efficacy of ECG and/or echocardiogram testing to diagnose HCM, long QT syndrome (LQTS), and Wolff-Parkinson-White syndrome in asymptomatic individuals. They concluded that, although ECG and/or echocardiogram screening had an excellent ability to reassure that patients had no disease (nearly 100% negative predictive value), it was at the expense of too many false-positive reports.
Acknowledging reasons for differences in outcome/philosophy
In trying to reconcile these polarized opinions by respected leaders in medicine, it is important to understand the fundamental differences in experience and the populations served by these various groups. The success or failure of a screening program may depend more on the infrastructure of the health care system, diversity of the population (ie, age, ethnicity, gender, level and type of activity), and prevalence of disease in that area than on the screening examination or tests. With this in mind, it is important to interpret the data from the literature in the context of the parameters discussed earlier in order to better gauge its applicability to a population.
Current recommendations for preparticipation screening in the United States
With these concerns in mind, the American Academy of Pediatrics and AHA have chosen to take a more conservative approach to their screening recommendations than their European counterparts. The 2007 AHA scientific statement on preparticipation screening in competitive athletes does not recommend the routine use of ECG or echocardiography in the context of mass, universal screening and concludes that mandated 12-lead ECG screening is, “probably impractical and would require considerable resources that do not currently exist.”
In its 2012 policy statement on pediatric SCA, the AAP echoes this sentiment and endorses the use of standardized preparticipation evaluation forms to obtain a specific patient and family history and physical examination to solicit warning signs of those who may be at risk for SCA, as well as to identify individuals and families who may benefit from referral to a pediatric cardiologist. Several such preparticipation forms have been published. The policy statement goes on to state that, “Not all SCA can be foreseen, even in the best of circumstances. No screening protocol has yet proven to be effective in this role or validated as highly effective.” The article states that primary prevention of SCA should be specific to an individual’s cardiac disorder and should consist of a combination of medical therapy, device implantation, activity restriction, and avoidance of particular medications. Because this form of primary prevention requires a specific diagnosis, and the initial presentation of these occult cardiac diseases is often SCA or aborted SCA, secondary preventative measures are advocated. These measures include early symptom recognition, ability to provide effective CPR, and access to an AED. Through implementation of secondary prevention programs, the ability to successfully resuscitate both individuals previously diagnosed and those undiagnosed with cardiac disorders at the time of an SCA has been shown.
- •
Considerable controversies exist as to the best type of screening in athletic adolescents
- •
Routine use of ECG or echocardiography is currently not recommended by the AAP and AHA because of concerns for false-negative and false-positive results and availability of resources
- •
In the absence of a perfect screening tool, secondary preventative measures including early symptom recognition, ability to provide effective CPR, and access to an AED are advocated
Approach to cardiac history and physical examination in the athlete
Although limitations of the preparticipation evaluation have been described, there is merit in obtaining a thorough cardiac history and physical examination. Positive findings can trigger further evaluation that may lead to a diagnosis of heart disease in the athlete or even an athlete’s family member. The AHA has published a 12-element recommendation for cardiovascular screening in competitive athletes, which is reflected in the 2010 Preparticipation Physical Evaluation Monograph published by the American Academy of Family Physicians and the American Academy of Pediatrics ( Box 1 ).
Patient history
Chest pain with exercise
Syncope or presyncope without clear noncardiac cause
Shortness of breath or fatigue out of proportion to activity
History of heart murmur on examination
History of systolic hypertension
Family history
Relative with unexpected death before 50 years of age
Close relative with cardiac disease or disability before 50 years of age
Relative with cardiomyopathy, LQTS, channelopathy, connective tissue disorder, or arrhythmia for which they receive treatment
Physical examination findings
Heart murmur
Increased blood pressure
Marfanoid body habitus
Weak femoral pulses
History
The goal of the cardiac history should be to elicit an account of symptoms with exercise, including chest pain, shortness of breath, palpitations, near syncope, syncope, or easy fatigue. Taking a careful history can help differentiate cardiac from noncardiac causes of these symptoms.
Chest pain
Chest pain is most likely to be of musculoskeletal origin in young athletes; however, chest pain that occurs with exercise should lead to further evaluation. Location, radiation, intensity, character, duration, provocative and palliative factors, and associated symptoms need to be identified in order to describe the pain. The presence of associated symptoms, such as diaphoresis, pallor, palpitations, dizziness, or radiation of the pain to the jaw or left arm, can suggest cardiac chest pain. Chest pain that causes athletes to stop their activities is also worrisome. A history of recent viral illness in a patient with cardiac symptoms may be caused by myocarditis or pericarditis. In addition, a previous history of Kawasaki disease should be elicited, which could place the athlete at risk for coronary artery disease.
Dyspnea on exertion
Dyspnea on exertion can be a nebulous symptom, but certain characteristics should raise concern for cardiac involvement. When shortness of breath occurs at a notably lower threshold than previously in a given individual, in the absence of a period of deconditioning or known respiratory illness, further cardiac evaluation is warranted. It is often also helpful to gauge the degree of shortness of breath against peers who were performing the same activity at that time. Symptoms that impair the athlete’s performance are generally more concerning than those that they can work through. Referral to a pulmonary specialist may be warranted when cardiac causes are unlikely.
Palpitations
A palpitation is defined as a sensation of rapid or irregular beating of the heart and may represent different events to different young athletes. Sensations of a pounding heart during exercise, in the absence of other associated symptoms, often represent a normal physiologic response to exercise. In this case, the increase and subsequent decrease in heart rate are gradual and correspond with the warm-up and cool-down period of the activity. A careful history of the nature of the onset and cessation of sensed tachycardia can provide clues to its cause. Supraventricular tachycardia characteristically has an abrupt onset and termination, often with a perceived prominent beat or thump ending the cycle. In all instances, knowledge of the heart rate at the time of sensed tachycardia is helpful in establishing its mechanism. Teaching athletes, parents, and coaches to take a pulse can help elicit valuable information should the symptoms recur. In general, heart rates of more than 180 beats per minute in teenagers should raise concern for arrhythmia. Palpitations associated with near syncope, syncope, chest pain, diaphoresis, or pallor, are concerning for a cardiac disorder.
Syncope
Syncope and near syncope are common occurrences in teens. Classic vasovagal syncope consists of a prodrome of feeling unwell, followed by visual changes, culminating in a loss of consciousness. Intense fear, heat, pain, sight of blood, dehydration, prolonged standing, micturition, and even hair combing are all well-known triggers for benign syncope. Syncope with exercise is less common and far more concerning. Sudden loss of consciousness occurring in the middle of exercise, with no recollection of the events immediately surrounding the syncopal event is a harbinger of severe cardiac disease. This type of passing out is different from postexercise syncope, which is often accompanied by the prodrome of vasovagal syncope after the activity is completed.
Easy fatigue
Tiring with exercise can be a normal phenomenon in athletes undergoing intense training. It is most likely to occur following a period of deconditioning, when athletes are unable to perform to the level of exertion to which they were previously accustomed. It is noteworthy that significant physical deconditioning can occur within 3 weeks of cessation of training. Easy fatigue is most concerning when it is of recent onset in an otherwise well-conditioned athlete or when the fatigue is disproportionate to that experienced by the athlete’s peers of similar athletic ability. Associated symptoms of chest pain, diaphoresis, pallor, palpitations, dizziness, or syncope are all concerning. Recent viral prodrome may be innocuous or could raise concern for myocarditis. A history of worsening fatigue is more worrisome than improving fatigue.
Medications, supplements, and other substances
Investigating what teenage athletes are putting into their bodies can identify individuals with risky behavior. Although the use of prescription stimulant medications (under the guidance of a medical professional) has been shown to be safe, abuse of stimulants can be hazardous. The athlete should be questioned and counseled about the use of energy drinks, so-called performance-enhancing drugs, recreational drugs, and dietary supplements. Abuse of cocaine, amphetamines, ecstasy, LSD (lysergic acid diethylamide), psilocybin (mushrooms), narcotics, cannabis, and volatile substances have all been shown to have adverse, and sometimes deadly, effects on the cardiovascular system. The use of synthetic marijuana has been shown to cause myocardial damage. Knowledge of consumption habits can aid in the explanation of the symptom and may lead to prevention of a catastrophic event.
Family History
A history of cardiac problems in the family, particularly in first-degree relatives, portends an increased risk of quiescent cardiac disease in the young athlete. History of congenital heart disease, sudden infant death syndrome, sudden cardiac death, cardiomyopathy, arrhythmias, pacemakers, familial hypercholesterolemia, connective tissue disorders (such as Marfan syndrome and the vascular form of Ehlers-Danlos syndrome), seizure disorder, congenital deafness, drownings, or other unexplained deaths in young people are noteworthy and deserve further investigation. It is particularly appropriate to inquire specifically about relatives with hypertrophic or dilated cardiomyopathies, LQTS, cardiac ion channelopathies, Brugada syndrome, and Marfan syndrome. A detailed review of the implications of positive family history is presented by Miller and colleagues, elsewhere in this issue.
Physical Examination
Great care should be taken in performing a physical examination, because findings may be subtle in certain heart diseases. The use of history and extracardiac physical findings to guide a more in-depth cardiac analysis can be helpful. The physical examination, as it pertains to the discovery of cardiac disease, is discussed later.
Vital signs
Heart rate, respiratory rate, and blood pressure should be routinely obtained, with the addition of pulse oximetry if there is any question of cyanosis. Tachypnea and tachycardia make up two-thirds of the clinical triad consistent with congestive heart failure (the third being organomegaly). Patients may often have essential hypertension, but coarctation of the aorta must be excluded. Palpating for weak or absent femoral pulses and obtaining upper and lower extremity blood pressures to look for a gradient is helpful to evaluate for coarctation. Height and weight are useful to estimate body mass index, and can identify those at risk for developing hypertension.
General appearance
Tall, lanky body habitus with abnormally long arm span should arouse suspicion for Marfan syndrome. Obese individuals may be at risk for development of metabolic syndrome.
Chest wall
The presence of pectus excavatum or carinatum can be a manifestation of Marfan syndrome. The presence of reproducible chest tenderness on palpation favors a diagnosis of costochondritis or musculoskeletal chest pain, making cardiac chest pain unlikely.
Lung examination
Crackles in the lungs can have a variety of causes, but rales can be a manifestation of pulmonary congestion secondary to heart failure. Tachypnea can be a manifestation of pulmonary edema caused by left-sided heart failure or pulmonary overcirculation.
Cardiac examination
The chest wall should be palpated for displacement of the cardiac apex and the presence of any lifts, heaves, or thrills. Auscultation of rate and rhythm can differentiate between sinus arrhythmia (variation in heart rate with respiratory phase) and ectopic beats. Physiologic splitting of the second heart sound (S2), which varies with respiration, should be differentiated from a widely fixed split S2, which may represent a left-to-right shunt at the atrial level (atrial septal defect or partial anomalous pulmonary venous connection) or some form of intraventricular conduction delay (such as right bundle branch block). The presence of a third heart sound (S3) may be normal, whereas a fourth heart sound (S4) represents diastolic dysfunction, which can be present in cardiomyopathies or pathologic ventricular hypertrophy. The presence of systolic clicks may represent a bicuspid semilunar valve when associated with the ejection phase of the cardiac cycle or mitral valve prolapse when occurring during midsystole. Particular attention should be paid to the timing, intensity, and character of cardiac murmurs. In general, low-frequency, low-intensity, systolic murmurs are likely to be benign in the asymptomatic individual, although findings of atrial level shunts and mild pulmonary stenosis may be subtle. Murmurs that are loud, harsh, high frequency, radiate to the back, louder when standing, diastolic, or associated with symptoms warrant evaluation by a pediatric cardiologist. An in depth review of teenage heart murmurs is given elsewhere in this issue.
Abdominal examination
Palpation for hepatosplenomegaly, as an indicator of right-sided heart failure, should be performed. Abdominal distension from ascites is unlikely to be present in an asymptomatic individual.
Extremities
Examination for cyanosis, clubbing, and edema is routine, but unlikely in the asymptomatic athlete.
Limitations of Testing
From a cardiologist’s perspective, it is often most desirable to consult on a patient in person, and put together history, physical examination, and cardiac test results to form a complete picture of the patient before diagnosing or attempting to exclude cardiac disorders. Realizing that many geographic, economic, organizational, and temporal barriers may prevent this ideal situation, it is important to understand the strengths, weaknesses, and limitations of different forms of cardiac testing, particularly when they are performed in isolation. Resourceful use of testing, with a healthy respect for these limitations, can help practitioners diagnose occult disorders and minimize false reassurance or unwarranted alarm.
Some clinical pearls, helpful in understanding the limitations of cardiac testing, are as follows:
- •
Electrical diseases, such as supraventricular tachycardia and LQTS, often occur in a structurally normal heart with normal findings on echocardiogram.
- •
Cardiomyopathies can develop at various stages of life. A normal echocardiogram does not preclude future development of disease.
- •
Abnormal coronary artery origins and intramural coronary arteries can be difficult to appreciate on echocardiogram. High clinical suspicion for coronary artery disease should lead to additional imaging with different modalities.
- •
Risk of sudden cardiac death is never zero. Some disorders responsible for sudden cardiac death are diagnosed only on autopsy with a previously normal cardiac work-up.
- •
The AHA recommends 12 elements for screening young athletes, including 8 items for personal and family history and 4 items for physical examination
- •
Symptoms with exercise including chest pain, shortness of breath, palpitations, near syncope, syncope, or easy fatigability should be taken seriously and referred to a cardiologist
- •
Further testing can be based on the findings of a comprehensive history and physical examination
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
