Pediatric Cardiology Update: Ofﬁce-Based Practice of Pediatric … · 2015-12-11 · Pediatric...

Pediatric Cardiology Update: Office-Based Practice ofPediatric Cardiology for the Primary Care Provider

Lubabatu Abdurrahman, MD, John R. Bockoven, MD, Arthur S. Pickoff, MD,Michael A. Ralston, MD, and Joseph E. Ross, MD

I n the following article, selected topics arepresented to provide an update on the practiceof pediatric cardiology from the perspective of

the primary care provider and outpatient-based sub-specialist. The approach to the evaluation of verycommon presenting signs and symptoms are reviewed,including heart murmurs, chest pain, and palpitations.The role of the primary care provider in screening andclearing children for participation in sports activities isdiscussed. Finally, issues of insurability, employment,and pregnancy in the adolescent and young adult withcongenital heart disease are presented.

Heart Murmurs in ChildrenA murmur in the child is quite common and, conse-

quently, the evaluation of a murmur is the mostfrequent reason for outpatient referral to most pediatriccardiology practices. It is estimated that up to 72% ofchildren will have a murmur noted at some time duringtheir childhood.1 Given the potential cardiac diseasethat could cause a murmur, this “diagnosis” is a sourceof concern for the primary care physician. The primarycare physician’s comfort level of diagnosing innocent(or functional) murmurs depends on his or her clinicalexperience regarding innocent murmurs and anypathologic murmurs that they have detected in theirtraining and practice. It is common to have a newcommunity physician refer more patients for evalua-tion of a murmur in their first year after residency thanit is during their fifth year. Which patient to refer for

subspecialty evaluation can be influenced by otherfactors besides the murmur, including the anxiety levelof the family. The family of a child referred to apediatric cardiologist for murmur evaluation will comewith many preconceived ideas and fears regardingtheir child’s murmur.2 Family history of cardiac dis-ease and patient symptoms will influence how thefamily approaches this evaluation. In addition, a mur-mur detected during a routine presports physical canresult in significant anxiety for the family as well asthe child because of concerns about sudden cardiacdeath in athletes, a rare occurrence, but one that isprominently reported by the media. However, thecurrent medical community pressures of proper use ofresources have to be balanced with the potentialmedical and legal issues surrounding the evaluation ofmurmurs and a possible missed diagnosis of congen-ital or acquired heart disease. Thus, the primary carephysician must balance clinical skills, various testingmodalities at his or her disposal, and the availability ofsubspecialty referral options. What follows is a frame-work for the evaluation of the new murmur. Topicssuch as diagnostic skills, levels of competency, varietyof testing modalities, and a cost analysis will bediscussed.

Evaluation

History

The evaluation of the murmur begins at the samepoint as any other sign or symptom—a thoroughhistory and physical exam. This process is dependenton the age at presentation, which greatly affects theevaluation of the murmur. Murmurs in the first days toweeks of life are more likely to represent cardiacdisease as compared to new murmurs in the adoles-cent.3 The history of the newborn with a heart murmur

From Wright State University, Department of Pediatrics, Dayton Chil-dren’s Cardiology, The Children’s Medical Center, Dayton, OH.Curr Probl Pediatr Adolesc Health Care 2003;33:318-347.© 2003 Mosby, Inc. All rights reserved.1538-5442/2003 $30.00 � 0doi:10.1016/S1538-5442(03)00137-8

318 Curr Probl Pediatr Adolesc Health Care, November/December 2003

centers on gestational factors—maternal diabetes, in-fections, drug and alcohol use, and findings on fetalultrasound. Room air saturations and response to100% oxygen can be very helpful in this evaluation, ascan noting any dysmorphic features or other systemdisorders present that might suggest a possible syn-drome. Murmurs heard in the first few days of life areinfluenced by changes in pulmonary vascular resis-tance and closure of the ductus arteriosus. Conse-quently, a ventricular septal defect might not bedetected during the first 24 hours after birth until thepulmonary resistance has decreased to a sufficientlevel to allow turbulent flow to be heard. In addition,a baby with hypoplastic left heart syndrome mightlook healthy until the ductus arteriosus closes andprofound shock ensues. Finally, many forms of com-plex and severe congenital heart disease (transpositionof the great arteries, total anomalous pulmonary ve-nous return) might not produce a murmur in thenewborn nursery, and other signs and symptoms ofpotential cardiac disease need to be observed. Thus,the timing of the murmur and presence or absence ofsymptoms in the perinatal period are important in theinitial evaluation.

Murmurs in the first few weeks to months of lifesecondary to lesions associated with left-to-rightshunting might have associated feeding difficulties,growth problems, and signs of “congestive heartfailure” (pulmonary overcirculation). These symp-toms can become more apparent or worsen as thepulmonary vascular resistance decreases over thistime and there is more shunting of blood into thelungs. On the other hand, murmurs in the preschooland elementary school years are more likely to beinnocent. It is common for a previously healthychild to have a “new” murmur when he or she isacutely ill in an urgent care setting. This murmur ismost likely created by increased cardiac output andflow velocity in the outflow tracts secondary tofever, anemia, or the underlying illness. Of course,a child with a history of group A streptococcalpharyngitis and a new-onset heart murmur mighthave acute valvulitis associated with acute rheu-matic fever. Other historical factors and symptomsassociated with Kawasaki disease or bacterial endo-carditis must also be considered in this age group.Finally, a family history of congenital heart diseasemight focus the physician on possible causes of amurmur at any age. Left ventricular outflow tractobstructive lesions may have a familial predomi-

nance; hypertrophic cardiomyopathy is transmittedin an autosomal dominant pattern with high pen-etrance, and velocardiofacial or DiGeorge sequencecaused by 22q� microdeletion has been noted inmany families.4

Physical Exam

General. After a thorough history has been obtained,a comprehensive physical exam should be performed.It is important to not immediately auscultate the childwith a murmur, but rather to first observe the child.The general state of the child should be assessed,specifically detailing color, respiratory effort, and anydysmorphic features. Appropriate vital signs should berecorded including length/height and weight, heart andrespiratory rate, and properly obtained blood pres-sures. Next, pulses can be assessed for character,volume, and correlation with the apical impulse. Anydiscrepancies between brachial and femoral pulsesshould be noted and suspicion of possible coarctationof the aorta should be entertained if there is a lowerintensity in the femoral pulse or a delay between thebrachial and femoral pulsation. The precordiumshould be palpated, noting any thrills or ventricularlifts; both are clues to an underlying cardiacabnormality.

Auscultation. After appropriate observation and pal-pation, the auscultory examination is performed. Thisexamination should be undertaken with ritualistic andsystematic precision. Each heart sound should bedissected out of the general auscultation and listenedto in isolation.5 All medical care providers whoauscultate children should generate a personal system-atic approach. This might include beginning withevaluating the first heart sound in the appropriatelocations, and then isolating the second heart sound.These heart sounds should be evaluated for intensityand appropriate splitting. Any associated valvar clicksshould be noted. Additional heart sounds (eg, S3 orS4) might then be assessed.

Next, the period of systole should be evaluated inisolation and any murmurs should be noted. Theintensity, frequency, duration, quality, location, andradiation of the murmur should be described. Theperiod of diastole then can be assessed and any distinctmurmurs should be recorded using the same parame-ters as in the evaluation of the systolic murmur. Anycontinuous murmur should also be noted. The inten-sity of murmurs has historically been categorized into6 levels or grades of increasing loudness, with a grade

Curr Probl Pediatr Adolesc Health Care, November/December 2003 319

IV murmur being associated with a thrill. The fre-quency of the murmur can provide some indirectphysiologic information. Higher frequency murmursare associated with more turbulent flow created bylarger pressure changes, as in a small patent ductusarteriosus or ventricular septal defect. Lower-pitchmurmurs suggest less turbulence associated with lesspressure change, as might be heard in a very largeventricular septal defect.

Probably the most helpful parameter for distinguish-ing innocent from pathologic murmurs is the quality ofthe sound. The most common innocent murmur (Still’smurmur) has a typical vibratory and musical quality,whereas most pathologic murmurs have a muchharsher, grating sound. In addition, a holosystolic orregurgitant murmur suggests either a ventricular septaldefect or atrioventricular valve regurgitation, whereasa systolic ejection murmur is usually created by aorticor pulmonary valve stenosis. Finally, the location ofmaximal intensity of the murmur offers significantinsight into the cause of this sound, as does the area ofradiation of the murmur. Only after all of the aboveparameters are noted can a complete description of themurmur be made to suggest a reasonable preliminarydiagnosis.

Some murmurs, however, may not be clearly inno-cent or specific for a cardiac lesion, and a definitivediagnosis remains unclear. To further help qualifyabnormal murmurs, McCrindle et al6 assessed 222patients with murmurs to determine the qualities ofmurmurs that suggested cardiac disease. These clinicalfeatures included a grade III or louder intensity,pansystolic timing, a harsh quality, a location ofmaximal intensity at the left upper sternal boarder, andthe presence of abnormal second heart sound or anearly- to mid-systolic click. These features provided92% sensitivity and 94% specificity in the diagnosis ofpathologic murmurs by this group of experiencedpediatric cardiologists.

Auscultation skills. As outlined above, there aremany details to the auscultory exam that need to belearned and perfected over a physician’s training andcareer. There is a great body of literature dealing withvarious levels of competence in auscultory skills atdifferent degrees of experience, but unfortunatelythere appears to be difficulty in the diagnosis ofinnocent murmurs at many levels of expertise. Gaskinet al7 evaluated the diagnostic accuracy of pediatricresidents in the assessment of a wide variety ofmurmurs generated by a patient simulator. These

residents demonstrated only a 33% rate of diagnosticaccuracy with only a slight trend for improvement athigher levels of training. Of note, the sound mostmisinterpreted was the innocent murmur. Similar stud-ies have been performed evaluating the auscultoryskills of office-based and academic pediatricians.Haney et al8 evaluated the ability of 30 office-basedpediatricians to correctly identify pathologic murmursin a group of patients with known cardiac status: 43%of the children had pathologic murmurs, confirmed byechocardiography. These investigators reported amean sensitivity of 82% and a specificity of 72% in theability to differentiate pathologic from innocent mur-murs. Thus, 18% of patients with pathologic murmurswould be misclassified as having innocent murmurs,and 28% of patients with innocent murmurs would bereferred for further testing or evaluation by a subspe-cialist, a result considered “suboptimal” by the au-thors. In another study, Rajakumar et al9 found thatacademic general pediatricians were able to diagnosemurmurs as pathologic with a similar sensitivity ascompared with pediatric cardiologists (79% vs 85%).However, there was a marked difference in the accu-racy of diagnosing innocent murmurs between the 2groups, with the pediatric cardiologist more oftencorrectly identifying innocent murmurs and thus hav-ing a higher overall diagnostic accuracy (76% vs92%). Finally, Smythe et al10 confirmed the high levelof accuracy of the pediatric cardiologist’s clinicalexam in diagnosing pathologic versus innocent mur-murs, with sensitivity values reaching 96% and spec-ificities as high a 95%.

Testing. To increase the diagnostic accuracy ofevaluating murmurs, multiple testing modalities, in-cluding electrocardiograms, chest radiography, andechocardiograms, have been evaluated. There has beenmuch debate regarding the utility of noninvasive testsin the evaluation of murmurs. Newburger et al11

performed a prospective study evaluating the electro-cardiogram, chest radiography and M-mode echocar-diogram in the evaluation of murmurs in children overthe age of 1 month. They found these tests to be ofvery little added value if the pediatric cardiologistbelieved that the murmur represented “no heart dis-ease” or “definite heart disease.” If “possible heartdisease” was suspected, the tests helped to categorizethe disease state. These investigators did, however,support obtaining tests with respect to issues such asfamily reassurance, obtaining baseline values for fu-ture comparison, and quantification of severity of heart


disease. Additional studies have found little value inelectrocardiograms in the clinical diagnosis of inno-cent murmurs by experienced pediatric cardiologists,and suggest that electrocardiogram and chest x-rayfindings at times lead to revisions of diagnoses thatmislead both the primary care provider and thespecialist.10,11

However, Swenson et al12 report utility for nonin-vasive testing, with diagnoses such as small atrialseptal defects, hypertrophic cardiomyopathy, andWolff-Parkinson-White syndrome made with the helpof chest x-rays and electrocardiograms in patientssuspected clinically not to have heart disease. Inaddition, Danford et al13 found that almost 9% (16 of187) of children believed to have innocent murmurs bya pediatric cardiologist demonstrated cardiac diseaseby echocardiogram. However, the majority of thesechildren (88%, 14 of 16) demonstrated abnormal signsor symptoms, electrocardiograms, or chest x-rays. Theauthors concluded that there should be a low thresholdfor the pediatric cardiologist ordering an echocardio-gram in very young infants and for select olderchildren with signs or symptoms that suggest cardiacdisease.

While echocardiography increases the diagnosticaccuracy in the evaluation of murmurs, many studiesreport unsuspected cardiac disease in a minority ofchildren with innocent murmurs, but at what cost? Yiet al14 created multiple models to evaluate the cost-effectiveness for the evaluation of murmurs that in-volved combinations of obtaining non-invasive studies(chest x-ray, electrocardiogram, echocardiogram) andreferral to a pediatric cardiologist. This study con-cluded that, in general, chest x-ray and electrocardio-gram are not cost-effective tools, that referral of onlysuspected pathologic murmurs to a sub-specialist is theleast effective strategy, and that the most effectivestrategies involve either referral of all murmurs to apediatric cardiologist or, at a higher cost ($158,000 peradditional case detected), performing echocardio-grams on all children with murmurs. Unlike Yi andcolleagues, who used statistics from the literature asinput data in their models, Danford et al13 used patientrecords to model cost-effectiveness and found thatreferral to a pediatric cardiologist with selective echo-cardiography to be the most cost-effective model inthe evaluation of murmurs.

Finally, there are many forms of congenital heartdisease that do not manifest themselves in the neonatalperiod until the pulmonary vascular resistance begins

to decrease (small ventricular septal defects), andatrial septal defects might produce quite subtle mur-murs (but characteristic fixed split second heart sound)in very early childhood. These murmurs might evolveinto the more classic sounds as the child matures, andthe primary care provider has the opportunity to repeatevaluations over time to help determine if a possiblecardiac defect is present. An encouraging finding withmost of the studies previously mentioned involves theseverity of the missed diagnoses when murmurs wereinitially believed to be innocent. The forms of congen-ital heart disease were usually mild and of no hemo-dynamic significance (mitral valve prolapse, smallatrial septal defects, and ventricular septal defects) andthus time spent in repeat evaluations would probablyresult in no adverse outcome.

Summary

Murmurs are common in the pediatric populationand the role of the primary care provider is todifferentiate the common innocent murmur from morepathologic sounds that could be created by structuralheart disease. This differentiation begins with apointed history and a systematic physical examinationthat dissects different heart sounds from the myriad of“noises” heard during auscultation. Expertise is en-hanced with thoughtful analysis and experience, butmany murmurs remain ambiguous. Many differenttesting modalities are available to aid in the assess-ment of the unclear murmur, but “ routine” electrocar-diograms and chest x-rays are probably not helpful.Referral to a pediatric cardiologist appears to be thenext step for the ambiguous murmur. If the murmurstill remains unclear, then an echocardiogram, per-formed by a technician trained in pediatric echocardi-ography, using appropriate equipment for the pediatricpatient, and interpreted by a pediatric cardiologist, isthe next most appropriate step in the evaluation.15

Routine echocardiograms ordered by primary carephysicians without pediatric cardiology consultationdo not appear to be cost effective, and can be difficultto interpret if performed by laboratories that do notspecialize in pediatric echocardiography.14,16

Chest Pain in Children: What to Doand When to Refer

The anxious family sat as the pediatric cardiologistwas about to address them. Their 10-year-old son had


just been referred for an echocardiogram because of arecent history of chest discomfort. He seemed to bewell in all other regards, was not overweight, and wasin no obvious distress as he was putting his shirt backon. The cardiologist had just reviewed the study andwas matter-of-factly peering at the family whilechoosing his words carefully. “His heart is structurallyand functionally normal based on the informationobtained from this test. A full report will be forwardedto his physician.” As he turned to leave the room, theboy’s bewildered mother rustled in her chair andquickly inquired, “But doctor, then what is causing hischest to hurt?”

The above occurrence is frequently encountered inthe circles of pediatric cardiology. Primary care phy-sicians are routinely asked to assess children withcomplaints of pain, most commonly headaches andabdominal discomfort. However, the third most com-mon “pain” confronted in this age group emanatesfrom the chest.17 Recent statistics suggest 6 in 1000pediatric emergency room visits are because of chestpain. This rate has more than doubled since themid-1980s. Slightly more boys than girls are re-ferred,18–20 and all races are proportionally represent-ed.21 The most common age range for presentation is8-16 years. Adolescents are more likely to haveassociated obesity and systemic hypertension.19,21

Most pediatric patients with chest pain have chronic orrecurrent discomfort.21

Why have referral rates for chest pain increased,given the fact that pediatric chest pain is rarelylife-threatening?19 Since the late 1970s and early1980s the prevalence of coronary artery disease, obe-sity, and significant systemic hypertension in youngadults has increased fairly dramatically.22 In addition,media reports of sudden unexpected death in youngathletes have become commonplace in a society wherethe benefits of physical fitness are widely publi-cized.18,22 More than 50% of young patients withchest discomfort believe their heart is the cause, yet itis known that “children are not born with the idea thatchest pain comes from the heart.”23,24 Coupling thesefacts with a possible inclination to transfer our ownadult fears onto our children has resulted in substantialincreases in emergency room visits, calls to 911, andreferrals to pediatric cardiologists.18,19 It is commonpractice for youth to be released from school becauseof chest pain. Practitioners fear the repercussions,medical and legal, of misrepresenting myocardial in-farction or the forerunners of sudden death for benign

chest discomfort.17,19 However, heart disease is morelikely to be recognized during routine evaluation ofchildren and adolescents than during an evaluation forchest pain, especially if the latter assessment occurs inan office setting.23,25

As previously stated, the overwhelming majority ofyoung patients with chest pain have etiologies that areconsidered benign.17 In an attempt to uncover thecause in a given individual, it is often useful todistinguish cutaneous or musculoskeletal pain histori-cally from visceral pain. The former tends to be brief,sharp or stabbing, and usually is localized. It immedi-ately follows a stimulus, is maximal at the onset andslowly dissipates, is often positional, and normally isreproducible on exam. Visceral pain is more diffuse;tends to be dull, burning, aching, or pressure-like; lastslonger; waxes and wanes in intensity; and is often notreproducible on examination.26 Benign chest discom-fort is apt to be recurrent, and may often be associatedor replaced with symptoms such as fatigue, abdominalpain, and headache.27

Most important, it should be emphasized that one ofthe least likely causes of chest pain in children is theheart.19,26 Assuredly, musculoskeletal, gastrointesti-nal, pulmonary, and psychiatric etiologies for chestpain are far more commonplace, despite a ferventmisconception to the contrary.19,26

Noncardiac Etiology

Many of the noncardiac etiologies of chest discom-fort are categorized in Table 1. Discrete musculoskel-etal pathology explains approximately 20% of thesemaladies.19 Traumatic injuries, even weeks after theevent, are easily understood sources.26 The keenobserver likewise recognizes that overuse afflictionssuch as sprains and spasms can also occur in the chestwall.26 Costochondritis is common in adolescents,especially in young women. It is usually unilateral,often in the left thorax, and significant discomfort isproduced by movement or palpation of the costochon-dral or chondrosternal articulations.17,26 When thispain is associated with a solitary, spindle-shapedswelling in the chest wall, typically at the rightsternoclavicular junction, it is referred to as Tietze’ssyndrome. A rare entity in the pediatric world, slippingrib syndrome, involves abnormal mobility of the eigth,ninth, and tenth ribs. The ribs do not directly attach tothe sternum but attach to each other by means ofdense, fibrous tissue. Activity or unrecognized traumamay result in thoracic pain, abdominal pain, and can


be elicited by bending over or by pulling anteriorlywith the fingers hooked under the lower rib cage. Thispain is believed to be generated by weakened fibroustissue irritating intercostal nerves.21,28,29 While mostmusculoskeletal ailments benefit from rest and anti-inflammatory medications, the slipping rib syndromemay occasionally require surgical intervention.26,28

Pulmonary sources of chest discomfort comprise anadditional 15% to 20% of the total cases. Chest painassociated with reactive airway disease, especiallywhen exacerbated by exercise and activity, can beparticularly difficult to differentiate from cardiaccauses and often requires extensive investigation.Pneumonia, specifically when coupled with pleuralinflammation, can be very painful because the pleuralmembrane contains many pain receptors.26 Activationleads to searing, sharp discomfort, which may affectbreathing. The development of a spontaneous pneu-mothorax, common in cystic fibrosis and Marfansyndrome, is a well-known cause of chest pain. An-other etiology that is difficult to distinguish fromcardiac disease is a pulmonary embolism. This appearsto be more prevalent in the female, those who areoverweight, those taking oral contraceptives, or any-one with a history of leg trauma.18 Thrombophilicconditions should be investigated in the child with apulmonary embolism.

While not common as a cause of chest pain, gastro-intestinal etiologies are responsible for approximately5% of the cases. In the pediatric population, gastro-esophageal reflux disease is the primary culprit, oftenleading to severe, erosive esophagitis. This pain oftenawakens the patient while they are supine, and can beprecipitated by the introduction of cold or hot sub-stances.26 Gastritis and peptic ulcer disease are lesscommon as causes of chest pain, and are typicallyassociated with chronic disease, immune deficiency, ormedications. Rarely, unrecognized foreign body inges-tion can become a source of chest discomfort.17

Often overlooked but extraordinarily importantcauses of chest pain are psychiatric issues. In at least10% of all individuals presenting with complaints ofchest pain, the primary underlying pathology is psy-chological. The stress associated with family membersdying suddenly or unexpectedly, a myocardial infarc-tion in a parent or grandparent, and the concern thatthey may experience a similar event can be devastatingto a child. Anxiety attacks with subsequent hyperven-tilation may add to the symptoms. It is not unusual todevelop an overt conversion reaction from the over-whelming stress of difficult family events.17 Chestpain is a response that is most common in adolescentwomen, while younger children will typically com-plain of headache or abdominal discomfort.25

A number of other etiologies for chest pain in youngpeople have been described as well. The most com-mon of the “miscellaneous” causes is the precordialcatch syndrome, a frequently seen but poorly under-stood entity. The pain associated with the precordialcatch syndrome is sudden in onset; very sharp innature, usually described as knife-like, needle-like, orshooting; and the pain does not radiate beyond theprecordium.26,30 The pain is exacerbated by inspira-tion in almost all cases, is sporadic with little relation-ship to exercise, and is generally brief in duration (30seconds to 5 minutes). While its etiology is unknown,the chest pain associated with precordial catch syn-drome is benign.30 Precordial catch syndrome may bethe most common cause of chest pain in childrenreferred for elective outpatient evaluation. The acutechest syndrome, linked specifically to sickle cell dis-ease, is frequently confused with myocardial ischemiaand can be extensive in its duration.21,26 The presenceof a chest tumor may also present with chest discom-fort, but this is rare and usually not isolated in itsymptoms.21 Breast tenderness in both adolescentmales and females is encountered with relative regu-

TABLE 1. Noncardiac causes of chest painI. Musculoskeletal (20%)

A. TraumaB. Overuse injuryC. CostochondritisD. Tietze syndromeE. Slipping rib syndrome

II. Pulmonary (15%-20%)A. Reactive airway diseaseB. PneumoniaC. PleuritisD. PneumothoraxE. Pulmonary embolism

III. Gastrointestinal (5%)A. EsophagitisB. Gastroesophageal reflux diseaseC. GastritisD. Peptic ulcer diseaseE. Foreign body ingestion

IV. Psychiatric (10%)V. Miscellaneous (15%-20%)

A. Precordial catch syndromeB. Sickle cell diseaseC. Chest tumorD. Breast pain

VI. Idiopathic (25%)


larity. Finally, despite considerable efforts over theyears to uncover the etiology of chest pains in chil-dren, at least 25% of the cases are truly idiopathic.

Cardiac Etiology

Despite exhaustive efforts to assess the heart inpediatric patients with chest pain, a cardiac origin israrely implicated.19 No more than 3% to 4% ofpediatric chest pain patients have a cardiac ba-sis.20,26,27,31 Cardiac causes of chest pain can becategorized as congenital lesions, acquired processes,and miscellaneous conditions (Table 2).17–19,22,26,32

As a rule, cardiac chest pain is either ischemic,inflammatory, or arrhythmogenic in origin. Ischemicpain is characterized as squeezing, crushing, or pres-sure-like, often with radiation to the arms, neck, back,or jaw. It tends to be reproducible, is provoked byexercise, and is relieved at rest. Ischemic pain can alsobe precipitated by cold exposure, eating, and emo-tions.26 Inflammatory conditions result in almost con-stant discomfort, at times improved with positioning,but clearly exhibiting a visceral quality. Arrhythmo-

genic discomfort usually results from the incessantpounding of the heart, and on occasion is associatedwith sharp chest pain.18

Certain congenital cardiac anomalies may be asso-ciated with ischemic chest pain, but most are not.Patients with “mild” congenital heart disease (eg,atrial septal defects, ventricular septal defect) are nomore likely to experience ischemic chest pain thannormal children.33 On the other hand, those withmoderate to severe left ventricular outflow tract ob-struction (valvar, subvalvar, or supravalvar aortic ste-nosis) may develop subendocardial ischemia underspecific circumstances, such as exercise.22 Rarely willsevere pulmonary stenosis act similarly. The mostlikely right ventricular lesions to result in exertionalchest discomfort are pulmonary hypertension andEisenmenger’s syndrome.18,26

Coronary artery anomalies comprise a very impor-tant set of lesions that must be considered in thecontext of cardiac chest pain. The most prevalentlesion, origin of the left coronary artery from thepulmonary artery, results in myocardial ischemia dueto poor perfusion pressure. This may present as earlyas 6 weeks of age, when pulmonary vascular resis-tance falls with resultant perfusion deficits to the leftcoronary system.19,22,26 When the left coronary arteryarises from the right coronary artery, it may coursebetween the aorta and the main pulmonary artery, andhas potential for compressive ischemia when the greatvessels dilate, as during exercise. The same holds truewhen the right coronary artery arises from the leftcoronary artery. Coronary artery fistula can establish acoronary steal phenomenon, especially if the vessel islarge.32

Hypertrophic cardiomyopathy is a relatively rare butimportant lesion to recognize.18 During periods ofincreased myocardial oxygen demands, such as exer-cise, which increases both heart rate and blood pres-sure, diastolic filling time shortens and systolic wallstress increases. This results in substantial subendo-cardial ischemia and even malignant arrhythmias, all afunction of diminished coronary perfusion.22

Although mitral valve prolapse is seen with somedegree of frequency in the pediatric population,whether there is simply “an association” with chestpain or it is “a cause” of chest pain is not clear. It iscorrelated with tachyarrhythmias, but it is unknown ifmitral valve prolapse actually predisposes the individ-ual to papillary muscle ischemia.22,26

TABLE 2. Cardiac causes of chest pain (3%-4%)I. Congenital anomalies

A. Left ventricular outflow tract obstruction1. Aortic stenosis2. Subaortic stenosis3. Supravalvar aortic stenosis

B. Severe pulmonary or subpulmonary stenosisC. Pulmonary hypertension, primaryD. Eisenmenger syndromeE. Coronary artery anomalies

1. Left coronary origin from pulmonary artery2. Left coronary origin from right coronary artery3. Right coronary origin from left coronary artery4. Coronary atrioventricular fistula

F. Hypertrophic cardiomyopathyG. Mitral valve prolapseH. Partial absence of pericardium

II. Acquired abnormalitiesA. EndocarditisB. MyocarditisC. PericarditisD. Kawasaki diseaseE. Acute rheumatic feverF. Autoimmune diseaseG. Dilated cardiomyopathy

III. MiscellaneousA. Myocardial infarctionB. Cocaine abuseC. Aortic root dissection

1. Marfan syndrome2. Turner syndrome

D. Arrhythmia


A most unusual congenital cardiac lesion known tocause chest pain is absence or partial absence of thepericardium.19,22,34 Usually the pain associated with itis sharp, non-exertional, but often is positional, be-coming more intense when the patient is supine or leftin a lateral recumbent position. The discomfort itselfrelates to herniation of the left atrial appendage orother portions of the heart muscle through the de-formed pericardium. This surgically amenable lesionis occasionally recognized initially during postmortemexamination.18

While inflammatory or acquired cardiac conditionsare uncommon in children, the chest pain associatedwith them is most often encountered when the peri-cardium is involved, such as in pericarditis, post-pericardiotomy syndrome, and autoimmune dis-ease.17,26 Patients with a history of Kawasaki diseaseshould always be taken seriously when complaining ofchest pain, because of possibly unrecognized coronaryartery stenosis. Patients with significant left ventricu-lar dysfunction (myocarditis and dilated cardiomyop-athy) develop chest discomfort with exertion becauseof coronary insufficiency.18,26

In children, true myocardial infarctions unassociatedwith congenital or acquired heart disease are quiterare, yet they do occur. More and more young patientswith familial hyperlipidemias are being recognizedbecause of identification of these disorders in parentsor family members. Unfortunately, illicit drugs remainprevalent in our society, specifically cocaine. Cocaineis a well-known to increase sympathetic activity,increase myocardial oxygen consumption, and causecoronary artery vasospasm—with subsequent myocar-dial ischemia.26 Patients with Marfan syndrome andTurner syndrome are prone to aortic root dissection, awell-described cause of acute chest pain in theadult.19,26,32

History

Of course, the initial approach to these patients is athorough, complete history.18,26 Whether a primarypractitioner or a pediatric cardiologist, the process istime-consuming and should not be rushed. Even ifone’s goal is simply to exclude a cardiac etiology, theeffort can be extensive. It is vital that the physicianallow the patient to describe the pain, as much aspossible, in their own words. This also prolongs theencounter and may confuse the portrayal, but it mayrepresent the most important aspect of the interac-tion.26

It is important to obtain as much information aspossible about the pain before its occurrence, duringthe episode, and after the event.19 Ischemic chest painis typically severe, mid-sternal, radiating to the shoul-der, jaw, or left arm, and is described as diffuse,crushing, pressure-like, and is associated with signif-icant difficulty breathing.18 There can be nausea,diaphoresis, and pallor, though the latter is quitenonspecific. Palpitations or a feeling of inappropriatetachycardia before the episode should be a tip toaggressively investigate for an underlying rhythmdisturbance. Of utmost importance is association withexercise. Cardiac chest pain is frequently precipitatedby activity and dissipated by rest, and it is character-istically reproducible. Most benign chest pain is spo-radic, unpredictable, and is not reproducible unlessthere is palpable tenderness over the sternum.18,19,26 Ittends to be nonvisceral in quality, usually sharp, andwith little radiation.18 Because the pain can worsenwith deep inspiration, associated shortness of breathand hyperventilation symptomatology is common,even in benign chest pain. Its relationship to exerciseis inconsistent at best. Pay particular attention to veryyoung children (�6 to 8 years of age) when theycomplain of chest pain. They often perceive palpita-tions as pain and have difficulty explaining and under-standing what they experience.19

Physical Examination

Most children with chest pain have normal exami-nations, unless they have underlying congenital heartdisease.26 Particular attention should be paid to theoverall level of distress, the vital signs, and tendernessover the chest or abdomen.19 Intensity of the pulsesand the cardiac impulse are noted. Lung auscultationshould detect the status of air exchange and anyunusual adventitious sounds.18 Cardiac auscultation isfocused on the detection of murmurs, rubs, clicks, andextra heart sounds.19

An abnormal cardiac exam further heightens suspi-cions of a cardiac cause for the pain. Left or rightventricular outflow tract obstruction murmurs shouldbe readily apparent. A coronary artery fistula is sug-gested by a soft, continuous murmur over the precor-dium.22 Hypertrophic cardiomyopathy with obstruc-tion usually produces a fairly harsh systolic murmur atthe right upper sternal border, with radiation to theapex and carotids. The click and subsequent murmurresulting from mitral valve prolapse may be accentu-ated with the patient in multiple positions, including


supine, sitting, standing, and squatting. Friction rubsmay be a sign of a pericardial process or exacerbationof an autoimmune disease.18

Laboratory

Following completion of the history and physicalexamination of the patient with chest pain, the focusshifts to laboratory testing. While each patient must beaddressed individually, there are many for whomlaboratory studies are simply unnecessary.19 There-fore, the need for testing and further evaluation shouldbe tailored to the patient in all cases.18 Generallyspeaking, the need for extensive studies is rare.31 Forthose patients where there is a strong suspicion of apulmonary or a cardiac etiology to the pain, some orall of the following may be indicated: electrocardiog-raphy, chest x-ray, echocardiography, exercise testing(with or without radionuclide imaging), ambulatoryelectrocardiographic monitoring, and pulmonary func-tion tests. Depending on the history and physicalexamination, laboratory studies such as a completeblood cell count, antinuclear antibody titer, sedimen-tation rate, creatine phosphokinase concentration, tro-ponin I, thyroid function tests, and drug screening maybe warranted.18,22 Clearly, the more impressive thehistory and physical findings, the more important andthe more detailed laboratory testing will be.18

As a primary practitioner initially evaluating a childwith chest pain, it can be difficult to know how far toproceed with the evaluation. A complete, thoroughhistory and physical examination is mandatory for allindividuals. Both the patient and the parents take thesymptomatology complex seriously and expect thephysician to do the same. A cursory time commitmentand a lack of interest on the part of the physician willresult in considerable frustration within the family andefforts to seek appraisal elsewhere.23 As a generalrule, the primary care provider should be neither toohasty nor overly exhaustive in the assessment, but seeka comfortable balance.19 Those individuals with obvi-ous musculoskeletal symptoms should be treated withrest, anti-inflammatory agents, and strong reassurance.Those with pulmonary or gastrointestinal symptoms orfindings should be managed with the appropriatetherapy and followed accordingly. An electrocardio-gram with (or without) a chest x-ray may serve asuseful “screening” tools here and are cost-effectiveentities. It is indeed very difficult to place a price onreassurance.35

Referral

As noted above, most patients and families seekingmedical attention because of chest pain in their childdo so because of concerns about the heart. However,only a very small percentage (3% to 4%) is cardiac-based and, thus, it would be inappropriate for all ofthem to be referred to a pediatric cardiologist. Patientswith visceral-type chest discomfort associated withnausea, palpitations, syncope, presyncope, or exercise,as well as those with abnormal cardiac examinations,chest x-rays, or electrocardiograms, deserve a muchmore thorough cardiovascular assessment. Particularattention should also be given to those who haveundergone previous cardiac surgery and those with afamily history of sudden unexpected death or earlycoronary artery disease.26 These patients are bestevaluated by a pediatric cardiologist. While mostpatients do not require immediate referral to an emer-gency room, those with a history of significant heartdisease, chest trauma, severe respiratory distress, pal-pitations, or syncope are best evaluated in this settingif the discomfort is persistent.18

The most difficult patients to assess with chest painare those where the etiology is not readily apparent tothe practitioner. While most of these individuals haveidiopathic or benign chest discomfort, there remainsan uneasiness regarding the extensiveness of theirtesting. The primary caregiver in this situation isconcerned about missing a life-threatening cardiaccondition, tries to do the right thing, and calm ananxious child and his or her family.22 While many ofthese children are ultimately referred to a pediatriccardiologist, it is important not to routinely orderstudies unless they can be justified. It is the role of thepediatric cardiologist to carefully evaluate the heartand confidently relate those findings to the patient andthe family.26 When no cardiac condition is found, theability of the cardiologist to communicate the benignnature of the discomfort to the patient and their familyis vital. If the family is left with the impression that thereason for the pain is unknown, they will continue toseek out others to help them and repeated trips to theemergency room will occur.17,23

Summary

In summary, chest pain in children and adolescentsis quite common, but rarely is it cardiac in origin orlife-threatening. A thorough history and examination,followed by appropriate testing is germane for their


initial assessment. In patients with visceral-type dis-comfort associated with exercise, palpitations, syn-cope, significant congenital or acquired heart disease,abnormal physical exams, and a positive family his-tory, a more extensive cardiac evaluation is justified.Above all, the practitioner is obligated to effectivelycommunicate the findings to the patient and family,and when appropriate, reassure them in order to allayany unnecessary anxiety or emotional turmoil.

PalpitationsPalpitations are defined as “ forcible pulsations of the

heart perceptible to the patient usually associated withan increase in frequency, with or without an irregular-ity in rhythm.”36 Palpitations in children have beenshown to be neither a sensitive nor a specific symptomof heart disease.37 Children and adolescents may use avariety of terms to describe their feelings of palpita-tions. Young children may have a difficult time de-scribing the sensations they feel. Further complicatingthis is the fact that the sensation of palpitations canoften occur in the presence of normal sinus rhythm.37

In contrast, patients with incessant tachycardias maynot report feelings of palpitations and some life-threatening arrhythmias may not present with palpita-tions.38,39 How, then, can one best evaluate a patientpresenting with a complaint of palpitations?

History

The key to an accurate diagnosis and effectivetreatment begins with a thorough history of the pa-tient’s symptoms. The report of palpitations should betaken seriously and the child should be asked todescribe the symptoms in their own words. It may bedifficult for young children to describe the sensationsthey experience and the report from parents of whatthe child has sensed or words that the child has usedwith them to describe the symptoms should be ob-tained. Historical points recommended39 for inclusionin a thorough evaluation include:

● Can you describe the symptoms?Children who are old enough to describe their

symptoms should be encouraged to do so. It is oftenhelpful to also have parental reports of the symptoms.Young children may have difficulty verbalizing theirsymptoms and parental reports of rapid pulse, pres-ence of pallor, dizziness, or seeing the chest wallvibrate rapidly can be useful.

● How long has the patient had these symptoms?When did the symptoms first begin? Did the symptomsbegin after some specific event? How many episodeshave actually occurred?

These are questions that help a physician understandwhat the patient is experiencing. For example, if thepatient had been riding a bicycle and the parents notedthe heart beating rapidly, and this was the only event,it was likely related to the patient’s recent exerciserather than an arrhythmia. Of course, if the parentsreport that the child participated in only quiet activitiesand presented with a rapid heart rate associated withchest pain, dizziness, or other such symptoms, thenthis was more likely an arrhythmic event.

● How often do the episodes occur?Patients with an arrhythmogenic etiology to their

symptoms rarely have symptoms on a daily basis.Their symptoms are typically separated by weeks oreven months. Sometimes their symptoms can occur inclusters consisting of several episodes occurring overa several day period, and then interspersed withperiods of quiescence. However, patients without anarrhythmogenic etiology to their symptoms may com-plain of symptoms occurring several times each day.This question also allows selection of testing that ismost likely to lead to a diagnosis. For example,patients who have symptoms on a daily basis shouldundergo 24-hour to 48-hour ambulatory electrocardio-graphic monitoring (Holter monitoring) in order tocapture a recording of the event. On the other hand, apatient with an episode once a month will need othertypes of monitoring.

● How long do the episodes last?Duration of symptoms is important to determine the

likelihood of an arrhythmia. Patients who report pal-pitations of a few seconds duration are less likely tohave a clinically significant arrhythmia than those thatreport palpitations lasting several minutes or more.Patients that notice irregular heartbeats or “skippedheart beats” lasting only seconds in duration requiredifferent types of monitoring than those with sustainedepisodes.

● Are there any particular events that make theepisodes occur?

Some patients have specific triggers for their epi-sodes. Exercise may be an important precursor, or theevents may occur at random times. This informationallows development of an appropriate strategy tomonitor such events.


● What do you typically do when you have anepisode?

This is useful to determine any specific actions thatproduce exacerbations or remissions. Children mayhave learned to self-perform a Valsalva maneuver,including gagging, yawning, drinking cold water, orother vagal stimulant activities that terminate su-praventricular tachycardia. Other patients may reportthat the episode gradually resolves. Episodes that havea gradual onset and gradual resolution are less likely tobe arrhythmogenic than those with sudden onset andsudden termination.

● Are there any associated symptoms with yourpalpitations?

It is more likely to be an arrhythmogenic eventshould the patient report dizziness, loss of conscious-ness, or anginal type chest pain during their symptoms.Patients with arrhythmias will often complain offeeling the palpitations “ in the back of their throat.”

● How does the patient appear during an episode?Again, it is more worrisome and more likely to be an

arrhythmogenic event if the patient appears pale, hasdizziness, or appears quite frightened during the epi-sode. It is important to inquire about diaphoresis andnausea, as these are more typically associated with anarrhythmia.

Finally, inquiring about the patient’s general life-style is useful.

● Does the patient ingest an excessive amount ofcaffeine? Are they athletic? Is there a family history ofarrhythmia or sudden death?

Investigate anxiety as a cause of (or created by) thepatient’s symptoms, especially if a relative has re-cently been diagnosed with an arrhythmia or even diedbecause of an arrhythmia. Such anxiety may exacer-bate a benign problem or create the illusion of anarrhythmia when none is present.

Physical Examination

Coupled with a thorough history is a completephysical examination, which should emphasize thecardiovascular system. Structural heart disease shouldbe actively investigated. Abnormalities in the cardio-vascular examination should be evaluated with addi-tional laboratory testing because the prognosis of anarrhythmia depends on the presence of underlyingcardiac disease.40,41 For example, premature ventric-ular contractions in a structurally normal heart arelikely benign.42–44 Even asymptomatic nonsustainedventricular tachycardia in a structurally normal heart is

likely to be benign.45,46 However, the same rhythms ina patient with structural heart disease, for example,dilated cardiomyopathy or postoperative tetralogy ofFallot, may be a risk factor for sudden death.47–49 Oneshould also be aware that children with rhythm prob-lems might have a completely normal cardiac exami-nation. Abnormalities of the electrical system may notproduce abnormal physical findings, such as murmurs,cardiomegaly, or abnormal pulses, unless associatedwith structural heart disease.

In addition, searching for other disease processes isimportant. Disease processes such as fever and anemiamay secondarily cause murmurs related to a high-output cardiac state and may also lead to sinustachycardia.50 Hyperthyroidism can lead to sinustachycardia and the perception of palpitations.51,52

Differential Diagnoses

Following the history and physical examination, oneshould have an idea of the patient’s diagnosis. Thedifferential diagnoses commonly include patient anx-iety, systemic illness, premature beats resulting fromatrial or ventricular ectopy, or a more complex ar-rhythmia of some type.

Anxiety. Anxiety in children can often trigger thesensation of palpitations. The anxiety is usually relatedto either school or family stressors. From the answersto the historical questions outlined previously, thechild with anxiety will typically relate that the epi-sodes occur multiple times each day and are short-lived in nature. The child will not have associatedsymptoms although occasionally they may have short-ness of breath associated with panic attacks. Theepisodes resolve gradually. Specific inquiries aboutthe family situation or the child’s situation at schoolare extremely helpful. Developing a rhythm monitor-ing strategy that allows these events to be recorded isimportant.

Systemic illness. Systemic illnesses that result in ahigh-output cardiac state can also induce the symp-toms of palpitations. Usually the patient will haveother symptoms associated with the palpitations. Anyillness that results in a fever will increase the heart rateand cause sinus tachycardia. Systemic illnesses thatcause anemia may also result in palpitations. If anemiais suspected clinically from the physical examinationdue to patient pallor, a complete blood count willconfirm it. Hyperthyroidism can also result in symp-toms of palpitations due to intermittent sinus tachy-cardia. Patients may have symptoms associated with


their hyperthyroidism, such as emotional lability,tremors, weight loss, or exophthalmos.52 Thyroidfunction testing provides confirmation. These patientsmay require a monitoring strategy to evaluate theirrhythm, which will reveal sinus tachycardia or, rarely,atrial fibrillation.

Ectopic beats. Some patients are able to sense andreport premature beats. These can be due to prematureatrial contractions or premature ventricular contrac-tions (Figures 1 and 2). Although isolated prematureatrial or premature ventricular contractions are benignwith a structurally normal heart, a monitoring strategyis necessary to capture these events and make adefinitive diagnosis. The patients can then be coun-seled regarding the benign nature of these events.Occasionally, should the events persist with continuedpatient symptomatology, medications may rarely benecessary to decrease the frequency of the prematurebeats and therefore the frequency of symptoms. In astructurally abnormal heart, further evaluation of boththe underlying cardiac disease and the arrhythmia isnecessary to determine a prognosis and select effectivetreatment.

Arrhythmia. The most common arrhythmogenic eti-ology for palpitations in children is supraventriculartachycardia (Figure 3). In younger children this istypically orthodromic atrioventricular reentry tachy-cardia utilizing a concealed accessory pathway. Thismeans that the tachycardia has antegrade conductionthrough the atrioventricular node and retrograde con-duction through an accessory pathway with reactiva-tion of the atrial tissue. This mechanism is calledreentry because the impulse “ reenters” the atrium toinitiate another beat of tachycardia. This is the mostcommon type of supraventricular tachycardia in youngchildren. In older children, adolescents, and youngadults supraventricular tachycardia is more likely theresult of atrioventricular nodal reentry. Atrioventricu-lar nodal reentry tachycardia is the result of reentrywithin the atrioventricular node itself.

Ventricular tachycardia is an uncommon presenta-tion for the complaint of palpitations, but may benoted occasionally in children (Figure 4). If the epi-sodes are nonsustained, with a structurally normalheart, then the episodes may be benign. If the episodesare sustained, however, or should the child have a

FIG 1. Rhythm strip demonstrates sinus rhythm with single conducted premature atrial contraction. Note different P wavemorphology before early QRS complex.

FIG 2. Rhythm strip demonstrates occasional premature ventricular contractions.


structurally abnormal heart, then further evaluationand therapy are necessary. Ventricular tachycardia canbe the result of electrolyte abnormalities, myocarditis,arrhythmogenic right ventricular dysplasia, hamarto-mas, Purkinje cell tumors, other ventricular tumors,central nervous system disorders, postoperative car-diac disease, mitral valve prolapse, drug-induced ven-tricular tachycardia, long QT syndrome, coronaryartery disease, or any systemic illness that results inanoxia or hypoxia.46

Finally, atrial fibrillation or atrial flutter may alsopresent in childhood (Figure 5). Investigation for struc-tural heart disease is required, focusing on those condi-tions associated with dilation or stretch of the atria, suchas atrial septal defects, or following extensive atrialsurgery, such as the older Mustard repair of transposition.

Adjunctive Testing

Electrocardiogram. All patients with palpitationsshould have an electrocardiogram performed to lookfor substrates for arrhythmia such as long QT syn-

drome or the presence of pre-excitation (Figures 6 and7). The electrocardiogram can also identify hypertro-phy of chambers or abnormal conduction intervals. Itis important that pediatric electrocardiograms be per-formed properly and interpreted by those experiencedwith pediatric standards. The electrocardiograms of a5-year-old, a 10-year-old, and an adult are dramati-cally different.

Chest X-ray. A chest x-ray is rarely of benefit in theevaluation of palpitations unless one is using it toidentify those with known or suspected structural heartdisease. Patients with cardiomyopathy may demon-strate an enlarged cardiac silhouette on the chestradiograph. However, chest x-rays have been shown tobe quite inaccurate for the diagnosis of specific typesof heart disease. Patients with palpitations, and partic-ularly those without evidence of structural heart dis-ease by the physical examination, will typically dem-onstrate a normal chest x-ray.

Echocardiogram. An electrocardiogram is usefulwhen structural heart disease is suspected. Echocardiog-

FIG 3. Rhythm strip demonstrates supraventricular tachycardia, a narrow QRS complex tachycardia at 230 beats per minute.

FIG 4. Rhythm strip demonstrates run of ventricular tachycardia at 115 beats per minute, with spontaneous termination.


raphy is a highly accurate tool for the diagnosis ofcardiomyopathy in children, both dilated cardiomyopa-thy and hypertrophic cardiomyopathy. Patients with in-cessant tachyarrhythmias deserve an echocardiogram toevaluate myocardial performance, because incessanttachyarrhythmias can sometimes lead to dilatedcardiomyopathy.

Holter monitor. Ambulatory electrocardiographicmonitoring (Holter monitor) is useful for patientsexperiencing symptoms on a daily basis. In the ab-sence of daily symptoms, a Holter monitor can docu-ment normal heart rate variation and identify clues toarrhythmia risk, such as abnormal conduction. Again,the test needs to be performed and interpreted by thoseexperienced with pediatric norms. Children have a

wide variety of normal heart rates. Heart rates of 50beats per minute when sleeping and 180 or 190 beatsper minute when exercising may be normal. Thisnormal variation should not be misinterpreted asabnormal bradycardia or tachycardia.

Event monitor. Event monitoring is also beneficial,and there are of 3 basic types. The first is self-applied anda recording performed at the time of patient symptoms. Itis useful for patients who are old enough to perform thistest themselves and have symptoms that last at leastseveral minutes in duration. After recording the event,the patient typically transtelephonically transmits the datato a receiving station for interpretation.

The second type of event monitor has a loop mem-ory. The patient wears the monitor continuously, and

FIG 5. Rhythm strip demonstrates atrial flutter. Initially, typical “saw-tooth” flutter waves are difficult to identify. During transienthigher grade atrioventricular nodal block, flutter waves are clearly identified.

FIG 6. Rhythm strip shows sinus bradycardia with prolonged QT interval.


when symptoms occur presses a recorder button. Themonitor retains the data from a predetermined timeperiod prior to the occurrence, during the occurrenceof symptoms, and a predetermined period after thebutton has been pressed. These monitors are typicallythe size of a pager and are useful for patients withfleeting symptoms or those who have neurologicimpairment of some type during the symptoms, whichwould prevent self-application of a monitor at the timeof symptoms.

The third type of monitor is implanted. These mon-itors are surgically placed subcutaneously, typically inthe pectoral area. Once an event occurs, the patientreports to his or her cardiologist to have the informa-tion transmitted. This is used for patients with signif-icant neurologic symptomatology, rarely occurringevents, or when the patient has been noncompliantwith previous attempts at documentation.

Stress test. Graded exercise testing is another ad-junctive test that is useful for patients whose symp-toms are provoked by exercise.46,53 Using this ap-proach, the patient’s cardiac rhythm is monitored in acontrolled environment during strenuous exercise todocument the type of rhythm that occurs.54

Therapy

Once the correct diagnosis is determined, therapycan be initiated. It is important to withhold therapyuntil a definitive diagnosis is reached. Many patients

reporting palpitations have normal chronotropic orinotropic variation in their heart rate. These patientssense this variation and feel anxious. Reassurance canbe given to these families after the normal variation isdemonstrated. Other patients may have arrhythmias ofa benign nature. Those with structurally normal heartsand isolated premature atrial or premature ventricularcontractions can also be given reassurance. Patientswith arrhythmias secondary to other conditions shouldhave those problems treated. For example, hyperthy-roidism, anemia, and fever can result in sinus tachy-cardia but the tachycardia is not harmful to the patientand the underlying disease process must be treated.Finally, some patients will have an arrhythmogenicetiology to their palpitations. These patients requiremedical, surgical, or catheter-based radiofrequencytherapy. Some patients benefit from a combination oftherapies. A discussion of specific therapies for eachrhythm problem is beyond the scope of this article butconsultation with a pediatric cardiologist is essentialfor effective management.

Summary

The evaluation of palpitations begins with a com-plete history and physical examination. Importantgeneral principles have been discussed and are sum-marized43:

● The description of the symptoms of palpitationsvaries depending on the patient’s age.

FIG 7. Electrocardiogram in a patient with Wolff-Parkinson-White syndrome. Note shortened PR intervals and slurring of initialphase of QRS complexes (ie, delta waves).


● Documenting the event is essential before advanc-ing a diagnosis or initiating treatment.

● The urgency of an evaluation for palpitations de-pends on the symptoms experienced and the likeli-hood of structural heart disease.

● Physical examination may be entirely normal, evenin those with serious rhythm problems.

● Adjunctive testing should be performed and inter-preted by those familiar with pediatric norms.

● Therapy is guided by the specific type of arrhyth-mia, associated symptomatology, presence of struc-tural disease, and each patient’s unique clinicalcircumstances.

Participation in Athletics: TheCardiovascular PreparticipationEvaluation

Each year millions of children and adolescentsacross the United States visit their primary carephysician with requests for “clearance” to participatein organized sports, usually activities organized withinthe school setting. Preparticipation examinations arenow required in 49 of the 50 states before a child oradolescent may participate in organized athletic activ-ities. The goals of the preparticipation examination areto identify medical and musculoskeletal conditionsthat could make participation in a given sport unsafe;to screen for underlying illness through a medical andfamily history, review of systems and physical exam-ination; and to recognize preexisting injury patterns inorder to recommend rehabilitation programs and toprevent recurrence.55

While the ultimate objective of the preparticipationevaluation is to protect the health and well-being of thechild athlete, these efforts may be compromised bymultiple factors, including the very strong desire ofmany adolescents to participate in organized athleticsin spite of known or discovered medical concerns anda lack of standardization of the content and method forconducting the preparticipation examination. Finally,there is a lack of established criteria at the high school,college, and professional levels for exclusion fromparticipation. In this section, we will discuss currentrecommendations and guidelines for athletic participa-tion that are specifically geared toward the cardiovas-cular system. The approach to the child with knowncardiovascular disease and the child without a specifichistory of a cardiac problem will be discussed.

Athletic activities invariably place an increased de-mand on the cardiovascular system. To be able toparticipate safely in an athletic activity, one must beable to accommodate the hemodynamic demands im-posed by the activity. The most useful classification ofathletic activities with respect to the demands placedon the cardiovascular system divides exercise into 2main categories, dynamic exercise and static exercise(Table 3).56 Dynamic exercise is characterized bylarge changes in muscle size and repetitive jointmovements with little developed intramuscular force,whereas static exercise is characterized by increasedintramuscular force with little change in musclelength. Many athletic activities share features of bothdynamic and static exercise. This classification isbelieved to be more useful in the formation of partic-ipation guidelines than is the metabolic-based classi-fication of sports as aerobic or anaerobic.

The cardiovascular adaptations to dynamic and staticexercise are well characterized. In response to dy-namic exercise one observes significant increases inoxygen consumption and heart rate, and a substantialincrease in cardiac output. During dynamic exercisesystolic blood pressure rises while diastolic bloodpressure typically falls, reflecting a decrease in periph-eral resistance. The heart of the competitive athletewho engages in a high dynamic exercise sport ischaracterized by an increase in chamber size and leftventricular mass, reflecting the volume load imposedby dynamic exercise. In contrast, static exercise resultsin little change in cardiac output and heart rate, andelevations of both systolic and diastolic blood pres-sures occur. The heart of the athlete who engages inprimarily static exercise is characterized by an in-crease in left ventricular mass without increases inchamber size, reflecting the predominant pressure loadof static exercise. Finally, it should be recognized thateven the most “sedentary” of athletic activities, suchas riflery or billiards, might result in significant,transient elevations in heart rate or blood pressure, asa reflection of competition-related stress with resultantactivation of the sympathetic nervous system.

Children Without Known CardiovascularDisease

For the primary care physician, providing the childwith known heart disease appropriate counseling forparticipation in athletic activities requires matching aknown diagnosis with a set of reference guidelines,


and at times referral to a pediatric cardiologist. Theapparently normal child without known heart diseasepresents a somewhat different challenge. While pre-cise figures are difficult to come by, it is estimated thatsudden unexpected cardiac death occurs in children(excluding SIDS) at a rate of 1.5 to 8 per100,000 peryear, and may be somewhat higher(1 in 50,000 to 1 in100,000) in competitive athletes.57 A lower rate ofsudden death, 1 per 200,000, was reported over a 12years among high school athletes in Minnesota.58 It isestimated that 20 to 50 sudden cardiac deaths occurannually among high school and college athletes.Though still rare, when such a tragedy occurs it oftenresults in widespread media coverage, probing ques-tions of school officials and health care providers, andlong-lasting anxiety and emotional trauma within thecommunity. By examining the known causes of sud-den unexpected cardiac death in children, and consid-ering the accuracy and costs associated with variousscreening approaches for occult heart disease, a ratio-nal approach for the identification of “at risk” childrenand adolescents can be proposed.

In one of the most comprehensive series addressingsudden unexpected cardiac death in young competitiveathletes, Maron et al59 identified the following causesin 158 cases (Table 4): hypertrophic cardiomyopathyand possible hypertrophic cardiomyopathy (46%), cor-onary artery anomalies (19%), rupture of the aorta aspart of Marfan’s syndrome (5%), aortic stenosis (4%)and other causes, including myocarditis, mitral pro-lapse, arrhythmogenic right ventricular dysplasia,asthma, heat stroke, and arrhythmias. With the excep-

TABLE 3. Classification of sports according to peak dynamic and static components during competitionA. Low dynamic B. Moderate dynamic C. High dynamic

I. Low static Billiards Baseball BadmintonBowling Softball Cross-country skiing (classic)Cricket Table tennis Field hockey*

Curling Tennis (doubles) OrienteeringGolf Volleyball Race walkingRiflery Racquetball

Running (long distance)Soccer*

SquashTennis (singles)

II. Moderate static Archery Fencing Basketball*

Auto racing*† Field events (jumping) Ice hockey*

Diving*† Figure skating* Cross-country skiing (skating)Equestrian*† Football (American)* Football (Australian rules)*

Motorcycling*† Rodeoing*† Lacrosse*

Rugby* Running (middle distance)Running (sprint) SwimmingSurfing*† Team handballSynchronized swimming†

III. High static Bobsledding*† Body building*† Boxing*

Field events (throwing) Downhill skiing*† Canoeing or kayakingGymnastics*† Wrestling* Cycling*†

Karate or judo* DecathlonLuge*† RowingSailing Speed skatingRock climbing*†

Water skiing*†

Weight lifting*†

Windsurfing*†

*Danger of bodily collision.†Increased risk if syncope occurs.Reprinted with permission from the American College of Cardiology Foundation. Mitchell BJ, Haskell WL, Raven PB. Classification of sports. J. Am Coll Cardiol1994;24:845-99.

TABLE 4. Causes of sudden cardiac death in young athleteDisorder %

Hypertrophic cardiomyopathy 46(and possible hypertrophic cardiomyopathy)

Coronary artery anomalies 19Aortic rupture (Marfan syndrome) 5Aortic stenosis 4Other causes (myocarditis, mitral valve prolapse,

arrhythmogenic)�3

Adapted with permission from Maron BJ, Shirani J, Poliac LC, Mathenge R,Roberts WC, Mueller FO. Sudden death in young competitive athletes. JAMA1996; 276:199-204. Copyrighted 1996, American Medical Association.


tion of aortic rupture, heat stroke and possibly asthma,the final common pathway to sudden cardiac death ineach condition is a lethal arrhythmia. The category of“arrhythmia” may include conditions such as Wolff-Parkinson-White syndrome and long QT syndrome,which represent primary electrical abnormalities inwhich electrophysiologic substrates for a lethal ar-rhythmia may exist in the absence of an underlyingstructural heart defect. Others such as coronary arteryanomalies, and arrhythmogenic right ventricular dys-plasia are conditions associated with structural defectsthat can be quite subtle and difficult to detect. Finally,geographic differences in the etiology of sudden car-diac death in young athletes have been reported. Forexample, in Italy arrhythmogenic right ventriculardysplasia, not hypertrophic cardiomyopathy, is theleading cause of sudden cardiac death in young ath-letes.60 Genetic differences, as well as the inclusion ofan electrocardiogram as part of the routine screeningof all student athletes in Italy, which may detect andthus exclude from athletics children with hypertrophiccardiomyopathy, may account for this geographicdifference.61

The Child With Known CardiovascularDisease

The most recent, comprehensive and widely usedguidelines to address the eligibility for participation insports for children and adolescents with known car-diovascular disease are derived from the 26th Be-thesda Conference.62 While death or disability directlyresulting from participation in sports is rare, even inthe child with a significant congenital or acquired heartdefect, certain heart defects are recognized as beingmore highly associated with a risk for sudden death.These include various forms of left ventricular outflowobstruction, such as aortic valve stenosis, cardiomy-opathies, congenital coronary artery anomalies, andMarfan’s syndrome with aortic root dilatation. Anelevated risk for sudden death is also present postop-eratively in children following repair of aortic steno-sis, coarctation of the aorta, and complex congenitalheart defects such as tetralogy of Fallot and transpo-sition of the great arteries.63 With early repair ofcongenital heart defects becoming the standard, mostchildren of school age will have had repair or pallia-tion of their heart defect already performed. The riskof sudden death after repair of tetralogy of Fallot hasbeen estimated to be approximately 1.5 children per

1000 patient-years, or 1 in 700 operations peryear.63,64 While elevated right ventricular pressure,significant pulmonary insufficiency, older age of re-pair and QRS complex greater than 180 ms onelectrocardiogram have been identified as potentialrisk factors for death, predicting level of risk inindividual patients is exceptionally difficult. For chil-dren who have undergone the older atrial repairs oftransposition of the great arteries (Mustard or Senningoperations), the risk of sudden death may be as high as4.9 children per 1000 patent-years, or 1 in 180operations per year.63,64 With the arterial switch nowthe standard method of repair of transposition of thegreat arteries, it is expected that the incidence ofpostoperative sudden death in these patients will besignificantly reduced. For both postoperative tetralogyof Fallot and (the atrial repair of) transposition of thegreat arteries, arrhythmia is the presumed mechanismfor sudden cardiac death. The risk of sudden death forother complex heart defects, such as double-outletventricle and single ventricle, may be of similar orgreater magnitude, even after repair. Children withcongenital heart disease associated with elevated pul-monary vascular resistance (pulmonary hypertension)are also at significantly elevated risk.65

For the child with a known congenital heart defect,the 26th Bethesda Conference offers excellent guid-ance for basing decisions about eligibility for partici-pation in athletic activities.62 In general, in the mildforms of the most common congenital heart defects(ventricular septal defect, atrial septal defects, patentductus arteriosus, valvar pulmonic stenosis, aorticstenosis) most sports listed in Table 3 are permitted. Inpatients with intermediate forms of the common de-fects, specific evaluations are recommended to guidefinal decisions concerning allowable sports. In themost severe forms of these congenital heart defectsstrenuous athletic activity is usually prohibited. Thus,children with small atrial, or small to moderate ven-tricular septal defects or small patent ductus arteriosusmay participate in all sports. However, in childrenwith atrial septal defects or PDAs with significantpulmonary hypertension, or large ventricular septaldefects, participation in sports may be limited to thosein class IA in Table 3. For asymptomatic children withvalvar pulmonic stenosis, those with peak systolicgradients of less than 50 mm Hg may participate in allcompetitive sports, while those with peak systolicgradients greater than 50 mm Hg are restricted to lowintensity sports (Class IA). Because aortic valve ste-


nosis, particularly when severe, is strongly associatedwith a risk for sudden death, the recommendationswith regards to sports participation for this lesion aremore conservative. Any patient with aortic stenosisand exertional symptoms such as chest pain or syn-cope should be immediately excluded from athleticparticipation until a complete evaluation can be per-formed. The asymptomatic child with mild aorticstenosis (defined by the 26th Bethesda Conference asaortic stenosis with less than a 20 mm Hg peakgradient) may participate in all sports as long as theresting electrocardiogram is normal. Those with mod-erate aortic stenosis, defined as a peak gradient of 21to 49 mm Hg, who are asymptomatic, have normalexercise stress test, and no greater than mild LVH onelectrocardiogram, may participate in low static/low tomoderate dynamic, and moderate static/low dynamicsports (classes IA and IB and IIA). Children withcoarctation of the aorta with less than a 20 mm Hggradient between upper and lower extremities, andwhose systolic blood pressure with exercise testingremains less than 230 mm Hg at peak exercise areallowed to participate in all athletic activities, althoughthe avoidance of high-intensity chest impact has beenrecommended by some to reduce the risk of aorticdissection.

Recommendations for athletic participation in post-operative patients are also addressed in the 26thBethesda Conference report. In general, 6 months afterrepair of atrial septal defects or ventricular septaldefect and 3 months after repair of PDA, if the child isasymptomatic and if there is no evidence of pulmonaryhypertension or arrhythmia, and no more than a smallresidual shunt, participation in all sports is permitted.In the case of children who are post-repair of tetralogyof Fallot, decisions concerning athletic participationgenerally require a careful evaluation that includes aphysical examination, chest x-ray, electrocardiogram,24-hour electrocardiogram monitoring, and an exer-cise stress test. An echocardiogram and at times apostoperative cardiac catheterization are often in-cluded in the evaluation. Patients who are found tohave an excellent repair, with normal or near normalright-heart pressures, only mild RV volume overload(from pulmonary or tricuspid insufficiency), no signif-icant residual shunt and no rhythm abnormality on24-hour electrocardiogram monitoring or exercisestress testing may be allowed to participate in allcompetitive sport activities. Similarly, in asymptom-atic children who are postatrial repair for transposition

of the great arteries, unrestricted athletic participationis permitted in those with no cardiac enlargement onchest x-ray, no history of atrial flutter or ventriculararrhythmia, no history of syncope, and a normalexercise stress test. All other patients require carefulevaluation and individualized consideration prior toformulating a recommendation for participation inathletics. For the increasing number of patients whohave undergone the Fontan operation for single ven-tricle complex, in general only class IA athletics arepermitted. Specific recommendations for children withother congenital heart defects, including unoperatedcyanotic heart disease, Marfan’s syndrome, and coro-nary artery anomalies (discussed later in this article),are also contained in the 26th Bethesda Conferencereport.

Children with significant systemic hypertension arebelieved potentially to be at increased risk for compli-cations including cerebral vascular accidents withexercise. To address this concern the 26th BethesdaConference set forth recommendations concerning theeligibility for sports participation of children andadolescents with mild, moderate, severe and verysevere degrees of systemic hypertension.66 Theseguidelines were subsequently revised by the Commit-tee on Sports Medicine and Fitness of the AmericanAcademy of Pediatrics in 1997 to more closely link thedefinition of severe hypertension in children greaterthan 12 years of age with blood pressure valuesreported in the Second Task Force on Blood PressureControl in Children.67 According to the 1997 AAPrecommendations, children and adolescents with nomore than ‘significant’ hypertension (136-143/86-91in a 13-15 year old), in the absence of evidence oftarget organ damage, may be allowed full participationin athletics without restriction. Children whose hyper-tension meets the criteria of “severe” hypertension(greater than 143/91 in a 13-15 year old) should berestricted from exercises that are highly static (classesIIIA, IIIB, and IIIC) until their hypertension is con-trolled, and there is no evidence of end organ damage.

Children with common, benign rhythm disturbancesmay participate in athletic activities without restric-tion.68 These include asymptomatic children withisolated premature atrial premature beats and childrenwith premature ventricular contractions who by his-tory and physical examination have a normal heart.For children with premature ventricular contractions,some recommend either a formal exercise stress test,or an informal office-based “exercise test,” to docu-


ment that the premature ventricular contractions do notincrease with exercise, and ideally are suppressed athigh heart rates. Similarly, asymptomatic children(without structural heart defects) with sinus bradycar-dia, junctional escape beats, or sinus pauses of lessthan 3 seconds, or mild first degree atrioventricularblock are allowed full athletic participation. For ath-letes with a history of supraventricular tachycardia,asymptomatic patients with exercise-induced su-praventricular tachycardia that is well controlled bymedical therapy, or who have sporadic episodes oftachycardia that appear suppressed by therapy, mayparticipate in all athletic activities.

There are cardiac conditions associated with hemo-dynamically significant and potentially lethal arrhyth-mias for which clearance for participation in athleticsshould be withheld by the primary care physician,pending evaluation by a pediatric cardiologist. Theseinclude children with ventricular pre-excitation(Wolff-Parkinson-White Syndrome), congenital heartblock, congenital prolonged QT syndrome, hypertro-phic cardiomyopathy, dilated cardiomyopathy, myo-carditis, and those with documented episodes of sus-tained, or nonsustained ventricular tachycardia.Evaluation by the pediatric cardiologist may include24-hour electrocardiogram monitoring, exercise stresstesting, echocardiography, cardiac catheterization,MRI imaging and, in some instances, intracardiacelectrophysiologic testing. Final recommendationsmay then be formulated. Complete guidelines forathletic participation for children with cardiac rhythmdisturbances are contained in the 26th Bethesda Con-ference.62

Conditions Associated With Structural HeartDisease

The conditions described below (as well as aorticstenosis, dilated cardiomyopathy, and myocarditis)share in common the presence of an anatomic orfunctional abnormality of the heart. Hypertrophic car-diomyopathy is the leading cause of sudden cardiacdeath in young athletes in the United States. Thisdisorder is characterized by left ventricular hypertro-phy that is the result of dominantly inherited genemutations of elements of the cardiac sarcomere, that is,the contractile proteins. Mutations in the myosinheavy chain, troponin T, and myosin binding proteinaccount for almost two thirds of all cases.69 Cardiachypertrophy in this disorder typically involves the

interventricular septum, but may be more concentricor even confined to apical portions of the left ventricle.Hypertrophy of the interventricular septum may beassociated with varying degrees of left ventricularoutflow track obstruction, which may be manifest onphysical examination as a harsh systolic murmur alongthe left sternal border. In other patients, minimaloutflow track obstruction occurs and the disorder maybe difficult to detect simply by auscultation. Theprevalence of hypertrophic cardiomyopathy may be ashigh as 1 in 500 adults. The true prevalence in thepediatric age group is not known, but it has beenidentified even in young infants. It is now recognizedthat, in addition to patients who have marked, life-threatening symptoms due to outflow tract obstruction,ischemia, and arrhythmias, a significant number ofpatients are asymptomatic and have a relatively benignclinical course. A family history of hypertrophic car-diomyopathy or unexplained sudden cardiac death in ayoung family member should alert the clinician to thisdiagnosis. Of equal importance, the child should bequestioned concerning exertional chest pain or syn-cope, and exercise intolerance. As mentioned earlier, amurmur may or may not be appreciated, depending onthe degree of left ventricular outflow obstruction. Theelectrocardiogram is abnormal in over 90% of infantsand children with hypertrophic cardiomyopathy, withleft ventricular hypertrophy and abnormal ST-T wavesbeing evident in most. The definitive diagnosis ismade by echocardiography, demonstrating either seg-mental or global left ventricular hypertrophy (Figure8). Mortality rates reported for hypertrophic cardio-myopathy range from 0% to 50%.

In a recent report of 99 pediatric patients withhypertrophic cardiomyopathy, sudden death was notedin 18 patients over a follow-up period of 5 years, foran overall death rate of 2.7% after age 8.70 In thisseries, predictors of sudden death included ventriculartachycardia on 24-hour electrocardiogram recording,myocardial bridging of the left anterior descendingcoronary artery, and increased QT dispersion on the12-lead electrocardiogram. In contrast to other reportsin the pediatric age group, age at diagnosis, familyhistory, and symptoms were not predictive of suddendeath in this series. For any child with known orsuspected hypertrophic cardiomyopathy, participationin competitive or strenuous athletic activities is for-bidden. Treatments available include medical therapywith �-blockers and, for those who remain symptom-atic from outflow obstruction, surgical excision of the


obstructing hypertrophied septum. Other therapies in-clude implantation of an automatic internal cardio-verter-defibrillator for those with refractory ventriculararrhythmias, and cardiac transplantation.

Coronary artery anomalies are the second leadingcause of sudden cardiac death in the young athlete.(Table 4) These anomalies include origin of either theleft or right coronary artery from the opposing aorticsinus, with a course that “wedges” the aberrant coro-nary artery between the aorta and pulmonary artery(Figure 9).71 These are the most common coronaryanomalies encountered in the context of sudden deathin the competitive athlete. Other anomalies, such ascoronary ostial stenosis and aberrant origin of the leftanterior descending coronary artery from the pulmo-nary artery are less common, or present earlier in life.The prevalence of significant coronary artery anoma-lies ranges from 0.17% in autopsy series, to as high as1.2% by angiographic series.72 In a recent report, 4cases of anomalous origin of the coronary arterieswere detected out of 2,388 children (0.17%) undergo-ing echocardiographic study for various indications.72

The mechanism of sudden death in children andadolescents with coronary artery anomalies is pre-sumed to be exercised induced ischemia with orwithout regional infarction, and resultant ventriculararrhythmia. The physical examination of the childathlete with an aberrant origin of a coronary artery willtypically be normal, however, upon close questioningas many as 40% may report exertional chest pain orsyncope, which should serve to alert the clinician to

the possibility of a coronary artery anomaly.71 Theutility of the routine electrocardiogram, exercise stresstesting, and echocardiographic screening for wall mo-tion abnormalities in the evaluation of patients withsuspected anomalous origin of the coronary arterieshas been questioned, because in 1 series these testswere normal in all patients with anomalous coronaryorigins in whom the tests were performed.71 Whenanomalous origin of a coronary artery is suspectedbecause of a history of recurrent or exertional syncopeor angina, definitive diagnosis should be aggressivelypursued. This requires imaging of the coronary arterieseither by echocardiography, MRI, or, if necessary,coronary artery angiography. Surgical correction ofthe anomaly represents the definitive treatment.

Marfan’s syndrome with aortic rupture representsthe third leading cause of sudden death in the compet-itive athlete. Phenotype can vary greatly in Marfan’ssyndrome, with some children and adolescents beingeasily diagnosed by physical examination (pectus,scoliosis, typical arm length to height ratio), whereasin others the diagnosis may be more difficult. Acareful family history may be useful when consideringthe diagnosis of Marfan’s syndrome in the “ tall”athlete without obvious stigmata. Evaluation by ageneticist, cardiologist, and ophthalmologist can behelpful in establishing the diagnosis. Most children

FIG 8. Echocardiogram in a child with hypertrophic cardiomy-opathy. Note thickened, hypertrophic interventricular septum(IVS). LA, Left atrium; LV, left ventricle; Ao, aorta.

FIG 9. Schema of anomalous origin of left main coronary arteryfrom right aortic sinus. A, Normal origin of left coronary artery(LAD and LCCA) from left sinus, and right coronary artery(RCA) from right aortic sinus. B, Left coronary artery arises fromthe right coronary artery and courses between the aorta (Ao)and the pulmonary artery (PA). LAD, Left anterior descendingcoronary artery; LCCA, left circumflex coronary artery; Ant,anterior; Post, posterior; L, left; R, right. Reproduced withpermission from Berger S, Dhala A, Friedberg DZ. Pediatr ClinNorth Am 1999;46:221-34.


and adolescents with Marfan’s syndrome (30%-60%)have accompanying cardiac abnormalities, includingaortic root dilatation, aortic insufficiency, aortic rootaneurysms, mitral valve prolapse, and mitral insuffi-ciency (Figure 10). While echocardiographic screen-ing of “ tall” athletes, for example, basketball players,for aortic root dilation has been advocated by some,73

this approach is has not been adopted in the UnitedStates. Sports participation for patients with Marfan’ssyndrome is guided by the extent of cardiac disease,though activities associated with chest collision shouldprobably be avoided.

As described earlier, arrhythmogenic right ventricu-lar dysplasia is the leading cause of sudden cardiacdeath in competitive athletes in Italy. This entity ischaracterized by fatty and fibrous infiltration of theright ventricular myocardium that provides an ana-tomic and electrical substrate for the development ofventricular arrhythmias. Ventricular tachycardia inthese patients is often exercise-induced, and has atypical left bundle branch morphology on electrocar-diogram. Therefore, a history of exercise-inducedsyncope, lightheadedness, or palpitations may be elic-ited. A history of syncope may be elicited in 23.5% ofchildren with arrhythmogenic right ventricular dyspla-sia.74 The resting electrocardiogram typically showsabnormal T wave inversions in the precordial leads.The entity may be dominantly inherited, and has beenlinked to multiple chromosomal loci, including chro-mosomes 1,2,3,10 and 14.75 A careful family historylooking for young persons with ventricular arrhythmiaor sudden death, therefore, may alert one to the

diagnosis. In severely affected patients, the diagnosiscan be based on demonstration of abnormalities ofright ventricular wall motion by echocardiography orby cineangiography. In many cases, however, thedegree of fibro-fatty infiltration is subtler and MRI orbiopsy may be required to establish the diagnosis.Arrhythmogenic right ventricular dysplasia may be theunderlying etiology in over 30% of cases of ventricu-lar arrhythmia and a seemingly “normal” heart, asassessed by echocardiography. Treatment may consistof medical therapy (sotalol preferred), and radiofre-quency catheter or surgical ablation of the arrhythmo-genic substrate. Implantation of an automatic internalcardioverter-defibrillator is also an option.

Conditions Without Structural Heart Disease

Causes of sudden cardiac death in the young athletewhere no structural heart defect or functional abnor-mality is present include children with arrhythmiasassociated with entities such as Wolff-Parkinson-White syndrome and long QT syndrome, as well aslethal arrhythmias induced on the athletic field inassociation with chest trauma, so-called commotiocordis.

The Wolff-Parkinson-White syndrome (presence ofan accessory atrioventricular connection) is a verycommon substrate for supraventricular tachycardia ininfants and children. The classical electrocardiogrampattern of the Wolff-Parkinson-White syndrome (shortPR interval, wide QRS complex, delta wave) may bepresent in up to 0.1% of the population (Figure 7). Thespectrum of symptoms varies greatly with many indi-viduals remaining symptom-free, some experiencingpalpitations or well-tolerated episodes of supraventric-ular tachycardia, and a few experiencing life-threaten-ing arrhythmias. Sudden death in association with theWolff-Parkinson-White syndrome is uncommon, esti-mated to occur in 1.5 per 1000 patient-years.64 Deathis thought to occur secondary to ventricular fibrilla-tion, initiated in response to rapid conduction of rapidatrial rhythms, such as atrial fibrillation, to the ventri-cles over the accessory connection. While rare, suddendeath or near sudden death episodes have been de-scribed in children with the Wolff-Parkinson-Whitesyndrome, including sudden death as the initial clini-cal presentation.76,77 Treatment of children withsymptomatic Wolff-Parkinson-White syndrome in-cludes antiarrhythmic therapy and, increasingly com-mon, radiofrequency catheter ablation of the accessoryconnection, which can be performed in children and

FIG 10. Magnetic resonance image in an adolescent withMarfan syndrome. Note significant dilatation of aortic root(arrowhead). Moderate aortic insufficiency was also present.


adolescents with a very high rate of success and highdegree of safety.78

Long QT syndrome should be considered in anychild or adolescent with a history of unexplained,recurrent, seizures or syncope, particularly when thesesymptoms are related to activity. A family history oflong QT syndrome or sudden unexplained cardiacdeath in young family members should alert thepediatrician to this diagnosis. It may be as common as1 in 7000 children.64 The disorder is the result ofcardiac ion channel gene mutations (potassium orsodium ion channel mutations75) that cause prolonga-tion of the action potential plateau, a delay in therepolarization process, and a propensity to developlife-threatening, polymorphic ventricular tachycardia.Sudden death associated with long QT syndromeoccurred in 8% of children followed for 5 years in 1series.79 The diagnosis is readily made from theelectrocardiogram (Figure 6) when the calculated cor-rected QT interval (QT interval divided by the squareroot of the RR interval) exceeds the upper limit ofnormal of 0.45 seconds. Genetic testing may becomemore widely available to assist in the diagnosis ofsuspected patients or family members when QT inter-val prolongation on electrocardiogram is borderline.Treatment consists of �-blockade, and, in patients inwhom symptoms persist, implantation of an automaticinternal cardioverter defibrillator.

Sudden cardiac death following a blow to the chestwall has become known as commotio cordis. Althoughrare in children, it is most commonly observed duringbaseball or hockey games.80 Collapse occurs immedi-ately following impact. Resuscitation efforts are oftenunsuccessful. It has been recently shown that bluntchest trauma, delivered at a critical phase of thecardiac cycle, can induce ventricular tachycardia andventricular fibrillation.81

Screening Children and Adolescents forPotentially Life-Threatening CardiovascularAbnormalities

Many of the conditions associated with sudden deathin the young athlete can be detected by electrocardio-gram (hypertrophic cardiomyopathy, long QT syn-drome, Wolff-Parkinson-White syndrome), or byechocardiography (hypertrophic or dilated cardiomy-opathy, aortic root dilatation). As discussed earlier,mass electrocardiogram screening of student athletesin the Veneto region of Italy has resulted in an

apparent reduction in the incidence of sudden cardiacdeath from hypertrophic cardiomyopathy among Ital-ian athletes.61 While a few US-based studies do reportcost-effective detection of “at risk” athletes throughuniversal electrocardiogram or limited-view echocar-diographic screening programs,82–84 other studies donot support this approach.85 In spite of this, in theUnited States the routine screening of all athletes byelectrocardiogram or echocardiography is currentlyconsidered impractical, expensive, and has not beenproven to prevent sudden death. The cost of detectinga single case of hypertrophic cardiomyopathy byroutine echocardiographic screening has been esti-mated to be between $200,000 and $250,000, andscreening for life-threatening conditions by electrocar-diogram, $44,000 per detected case. (57,82) Further-more, consideration of the costs and burdens associ-ated with false-positive results, and the sheermagnitude of resources required to provide nationwidescreening, make a universal screening approach basedon noninvasive testing impractical.

In 1996 a consensus statement was issued by theAmerican Heart Association, and endorsed by theAmerican Academy of Pediatrics Section on Cardiol-ogy, detailing the critical components of cardiovascu-lar screening of young competitive athletes.86 Thecurrently recommended and most cost-effective ap-proach for screening children and adolescents forpotentially life-threatening cardiovascular abnormali-ties is through a careful personal history, a focusedfamily history, and a physical examination. Cardiovas-cular screening is recommended for all athletes, andshould be performed prior to participation in highschool and college sports. For high school students,screening is recommended every 2 years, with aninterim history obtained each year. A careful history isof paramount importance. Of particular concern to thepediatrician should be children who report exertionalangina, exertional syncope, near syncope, excessivefatigue, or shortness of breath. These exertional symp-toms should not be confused with the very commonand quite benign sharp intermittent chest pain thatoccurs primarily at rest, or simple vasovagal syncope,both of which occur in many children and adolescents.The history should also include a past diagnosis ofeither hypertension or a heart murmur. The familyhistory should include questions of premature death ordisability from cardiovascular disease in close rela-tives younger than 50 years of age, or known familymembers with any of the conditions described above,


such as long QT syndrome, hypertrophic cardiomyop-athy, or Marfan’s syndrome. The focus of the physicalexamination should include checking for stigmata ofMarfan’s syndrome, measurement of blood pressure,palpation of femoral pulses (to exclude coarctation ofthe aorta), and auscultation of the heart for a murmur.A positive finding during the cardiovascular screenshould prompt a referral to a pediatric cardiologist forfurther evaluation. If such a referral is not practical,obtaining an electrocardiogram to screen for left ven-tricular hypertrophy, Wolff-Parkinson-White syn-drome or long QT syndrome, and an echocardiogrammay provide some guidance. It should again be em-phasized that the echocardiographic diagnosis of enti-ties such as anomalous origin of the coronary arteriesor arrhythmogenic right ventricular dysplasia is quitespecialized and technically challenging, and, there-fore, whenever possible, evaluation by a pediatriccardiologist is recommended.

Finally, community-based approaches to aid in theprevention of sudden cardiac death on the athletic fieldhave been proposed. These include education of thepublic concerning sudden cardiac death, the morewidespread teaching of CPR, and an increase in thepublic awareness and access to automatic externaldefibrillators during high school and college athleticevents. The use of chest protectors to prevent commo-tio cordis has also been proposed.

Insurance and Employment inAdolescents and Young Adults WithCongenital Heart Disease

It is a testament to the benign course of the mostcommon congenital heart defects and the improvingsurvival of those with even the more complex defectsthat the problems of insurability of these adolescentsand young adults have come to the forefront. There areat least half a million adults living with congenitalheart disease in the United States, a number that isgrowing by more than 20,000 per year.87,88 The factthat most of these adults are not severely disabled andare capable of working does not appear to improvetheir access to health insurance, which is frequentlydenied, limited, or set with prohibitive premiums thatmay exceed standard premiums by as much as 50%.For those with insurance, lifetime caps on coverage,underinsurance, and disapproval of an ever-broaden-ing list of certain services by managed care organiza-

tions can be near insurmountable. As minors, theirmedical expenses are usually covered by a parent’sprivate insurance (up to age 19) or through a publicprogram such as Bureau for Children with MedicalHandicaps, up to the age 21. For the low-incomepatient, Medicaid coverage may continue throughadulthood.

The severe limitations to health insurance coveragefaced by young adults with congenital heart diseaserepresents a direct drain on society and places signif-icant burdens upon a potentially highly productivesegment of society, as aptly illustrated by the results ofthe First and Second Natural History of CongenitalHeart Disease (NHS-1 and NHS-2).89,90 The initialNatural History Study of Congenital Heart Disease(NHS-1) funded by the National Heart, Lung andBlood Institute89 was not purely a natural historystudy, because it included surgically repaired patients.The average follow-up period was 8 years. They applyto patients with aortic valve stenosis, pulmonary valvestenosis and ventricular septal defects. To determinethe long-term status and outcome, the patients in thisstudy were re-contacted and studied from 1985 to1988 in NHS-2. Data were obtained from almost 85%of the NHS-1 cohort. At the time of entry into theinitial study, virtually all the patients were childrenand by the time of follow-up in the second study(NHS-2), the average age of the patients was justunder 30 years. The results are summarized as follows:

● More than 90% of the patients considered theirhealth good or excellent during the period of the study,no different than the average American.

● The patients were generally better educated thanthe general population. More than 90% graduatedfrom high school, compared with 80% in the generalpopulation. 60% either graduated from or were attend-ing college, compared with 40% for the generalpopulation. Of import, those patients with severedisease and in less than optimal health attained aneducational level similar to those with mild disease.

● The employment record of patients with ventricu-lar septal defects, aortic, or pulmonary stenosis wassimilar to that of the general population with approx-imately 93% working or still attending college.

● By the age of 30 to 40 years, 80% of the women inthis study were married, similar to the general popu-lation except that the women in the study tended tomarry later. The women in the study gave birth tofewer children, with 60% having children by the age


of 40 years compared to 85% for the general popula-tion of women.

● The frequency of marriage and rates of divorceand separation for the male cohort was similar to thegeneral population of men.

This NHS is especially pertinent in this discussionbecause the vast majority of young adults with thelabel of “congenital heart disease” will have (or havehad) an atrial, ventricular septal defect, or stenosis ofthe pulmonary or aortic valve, lesions which are thecommonest. The other end of the spectrum, a minority,albeit a growing one, will include patients with vary-ing degrees of palliated complex heart disease, such astetralogy of Fallot, transposition of the great arteries,and various anatomic substrates with a single ventriclephysiology. These are patients whose need for contin-ued consumption of medical and surgical servicesshould be expected to be higher than those of thegeneral population and will have, therefore, highermedical and health insurance expenses.

Fewer than 10% of adults with congenital heartdisease are considered disabled,91 yet their ability tosecure gainful employment is often markedly hinderedby their diagnosis even in the absence of any residualcardiac defects. Many employers balk at hiring suchadults because of often ill-informed concerns aboutfrequent absenteeism due to illness, an exaggeratedsense of the potential employee’s frailty and subse-quent ability to perform, and the real “dollar andsense” worry about increasing health care costs borneby the employer. Even a minor disability may have anegative impact on employability.92 Although theAmericans with Disabilities Act of 1990 prohibitsdiscrimination in employment and makes it unlawfulto question applicants about their disabilities unless itis prudent to their ability to perform the job, the factremains that the full protection of the law is somethingthe young adult with congenital heart disease can findextremely difficult to achieve.

There is an ongoing crisis in American health care,which is heightened, in the vulnerable populationssuch as adults with congenital heart disease. Inflation-ary pressures created by an ever-increasing demandfor services and rapidly evolving but costly technolog-ical advances will remain with us in the foreseeablefuture. In the interim, health insurance companiescontinue to adopt more restrictive measures and im-pose higher premiums to offset expenses. Employersin turn avoid hiring those with preexisting conditions

in an attempt to keep their health care costs manage-able.

Life insurance is another matter altogether. In 1study, which polled insurance companies to determinewhich cardiac conditions were considered insurable,there was a marked disparity from company to com-pany for no discernable reason. The factors consideredby the companies in making their decisions were oftennot provided. Some conditions such as transposition ofthe great arteries and ventricular septal defects withpulmonary hypertension were considered uninsur-able.93

Pregnancy, Delivery, andContraception in the Young AdultWith Congenital Heart Disease

As in other aspects of the medical care of the patientwith congenital heart disease, a multidisciplinary ap-proach involving an appropriately trained pediatric oradult cardiologist, obstetrician, perinatologist, geneti-cist, and anesthesiologist is necessary to provide opti-mal care to these patients. Ideally, a pre-pregnancyevaluation should be performed to assess the severityof the patient’s heart disease and allow discussionbetween the medical team and the patient concerningthe anticipated impact of pregnancy induced hemody-namic changes on the patient’s condition. The mater-nal risk depends on the type and severity of the heartlesion, ventricular function, presence of pulmonaryhypertension, and the patient’s exercise capacity. Thesame factors that determine pregnancy outcome inheart disease in general apply to this group of patientswith congenital heart disease. Severe systemic ventric-ular dysfunction, significant oxygen desaturation, andpulmonary hypertension are associated with high mor-bidity and mortality for the mother and fetus.94,95 Athorough prepregnancy evaluation of all patients withcomplex congenital heart disease (preoperatively orpostoperatively) should therefore include cardiaccatherization. A review of the patient’s medicationsfor potential teratogens, such as angiotensin convert-ing enzyme inhibitors, which may cause renal dyspla-sia in the fetus, or other possible toxicities should becarried out prior to a planned pregnancy. Finally,genetic counseling with the patient and the infant’sfather is often appreciated by the patient. Both menand women are concerned about the risk of congenitalheart disease in their offspring.


Genetics

In the general population, the risk of congenital heartdisease is 0.4% to 0.6%. If the mother is affected, theaverage risk is 6.7% (range 2.5% to 18%). If the fatheris affected, the risk is 2.1% (range 1.5% to 3.0%). If 1offspring is affected, the risk of having a second childwith congenital heart disease is 2.3% (range 1.5% to5%). If 2 offspring are affected, the risk of having athird child with congenital heart disease rises to 7.3%(range 5% to 10%).96

The risks are higher when there is a chromosomalanomaly; and some conditions such as velocardiofa-cial syndrome show an autosomal dominant inheri-tance.

Physiology

Plasma volume begins to rise in the first trimester,leveling off in the second trimester. The red blood cellcount also rises (20%), but to a lesser extent than theplasma volume which almost doubles. The entireblood volume increases by 40% to 50% reaching amaximum by 30 to 32 weeks of gestation. In addition,the heart rate increases by 10% to 20%, contributing toan increase in the cardiac output, which rises byapproximately 30%. Concomitantly, there is a signif-icant fall in peripheral vascular resistance and thevenous pressure in the lower extremities, which is whyup to 80% of healthy pregnant women develop pedaledema. The pregnant woman therefore has a hyperdy-namic cardiovascular physiology. Because of thephysiologic decrease in peripheral vascular resistancein pregnancy, obstructive cardiac lesions, such asaortic stenosis, are not well-tolerated as their gradientsincrease, whereas regurgitant legions, such as mitralregurgitation, are well-tolerated because of the de-crease in afterload.

The majority of women with congenital heart diseasewho become pregnant have, or have had, simplelesions, are acyanotic, and have normal systemicventricular function and no pulmonary hypertension.The fetal and maternal outcome in these patients isfavorable with no significant difference in labor out-come, or fetal or maternal morbidity and mortality,although the fetuses tended to be smaller.97 In thepregnant woman with high-risk heart disease, maternaland fetal outcomes are significantly worse. Womenwith cyanotic heart disease have high fetal loss, withup to a 45% chance of spontaneous abortion when theoxygen saturation is below 82%.98,99 As maternal

cyanosis worsens, as reflected by an increasing hemo-globin concentration, there is a near linear increase inthe incidence of spontaneous abortion, and fetalgrowth retardation is worsened. When the hemoglobinis higher than 18g/dl, the chance of infant survivalbecomes remote.100 Certain cardiac lesions are asso-ciated with such a high risk of maternal and fetalmorbidity and mortality that pregnancy termination isrecommended. Marfan’s syndrome with a dilated aor-tic root greater than 40 mm is at increased risk fordissection and rupture during pregnancy. Patients withpulmonary vascular obstructive disease (Eisenmengerphysiology) have a 50% mortality with pregnancy, andtermination of pregnancy, especially in the first trimes-ter, is a safer option. Women with symptomaticobstructive lesions, such as aortic and pulmonarystenosis, fare poorly in pregnancy, because the after-load reduction exaggerates their obstructive gradient.Patients with symptomatic ventricular dysfunctiontend to deteriorate during pregnancy as the volumeexacts a hemodynamic burden.

Labor and Delivery

There are rapid changes in hemodynamics duringlabor. With each uterine contraction, 300 to 500 mL ofblood is pumped into the circulation, with progres-sively increasing cardiac output. Following delivery,with the relief of IVC obstruction by the fetus, there isan increase in venous return to the heart. The imme-diate postpartum period is especially fraught withdanger for patients with Eisenmenger syndrome andpulmonary hypertension, and those with hereditarylong QT syndrome. Recent studies show a high ma-ternal mortality rate primarily in the postpartum periodfor patients with Eisenmenger syndrome101 and exclu-sively in the postpartum period in those with heredi-tary long QT syndrome.102

Hemodynamics return to baseline approximately 4 to6 weeks after delivery. In the vast majority of patientswith congenital heart disease, vaginal delivery is saferunless there is an obstetrical complication; blood lossis significantly greater with a caesarian section. Themanagement of labor and delivery in the patient witha moderate to high risk cardiac lesion is very complex,and requires a multidisciplinary approach to minimizematernal stress, shorten the second stage of labor, andprovide intensive monitoring with judicious use ofanesthesia. Routine antibiotic prophylaxis is not usu-ally recommended for labor and delivery unless infec-tion is suspected or in high-risk patients with pros-


thetic valves, previous history of endocarditis,complex congenital heart disease, or surgically createdsystemic to pulmonary conduits.

Contraception

The prevention of pregnancy is desired by patientswith congenital heart disease for diverse reasons thatare similar to the general population and is the beststrategy for those with high-risk cardiac disease. Lowestrogen contraceptives are effective and safe exceptin patients with right-to-left shunting, polycythemia,or a prior thromboembolic event where there is anincreased risk of thromboembolism. Estrogen mayalso exacerbate pulmonary hypertension. In thosepatients with significant left ventricular dysfunction(ejection fraction � 30%), there is an increased risk ofthrombus formation that may be aggravated by use ofestrogen containing contraceptive. Medoxyprogester-one acetate (Depo-Provera) and levonorgestrel implant(Norplant) are the preferred methods of contraception,because they are long-lasting and have a highercompliance. Progesterone-only contraceptive pills areless effective and do not prevent ovulation. Intrauter-ine devices are usually avoided because of the risk ofinfection. Sterilization (tubal ligation) is usually rec-ommended for the very high risk patient where preg-nancy would be life-threatening, but is not recom-mended for the patient under the age of 21 years.

Summary of Key Points● Innocent heart murmurs are quite common in

children. Most physicians caring for children are“comfortable” diagnosing innocent murmurs. If thereis any doubt about the innocent nature of a heartmurmur, or associated symptoms of concern, referralto a pediatric cardiologist is the preferred route forfurther evaluation.

● Chest pain is quite common in children, and rarelysignifies a serious underlying cardiac condition. Inter-mittent sharp, stabbing chest pain is particularly com-mon (precordial catch syndrome), is benign, andrequires only reassurance. Laboratory evaluation ofchest pain in children should be guided by clinicalpicture and examination. Thus, a routine chest x-ray isnot necessary for every child, but would be indicatedin the child with chest pain, fever, and cough. Exer-tional chest pain should be evaluated by a cardiologist.

● Palpitations in children are most often associatedwith non-life-threatening events, such as sinus tachy-

cardia or isolated extrasystoles. Children who reportlightheadedness, syncope or near syncope, or exer-tional symptoms, should be evaluated by a cardiologistand monitored to document the nature of the underly-ing cardiac rhythm.

● Children with known congenital heart disease,either repaired or unrepaired, may participate in ath-letic activities as guided by the recommendations ofthe 26th Bethesda Conference.62 Children with mildforms of congenital heart disease, or those with fewhemodynamic residua following surgical repair mayparticipate in most athletic activities.

● Children without known cardiovascular diseaseoften request of their primary care physician “clear-ance” to participate in athletic activities. The pre-participatory screening for these children includes acareful history and family history, looking for exer-tional chest pain, syncope, or fatigue, or a familyhistory of sudden cardiac death in persons youngerthan 50 years, or a known inheritable syndrome suchas Marfan syndrome, and a focused cardiovascularexamination. Routine laboratory testing (electrocar-diogram, echocardiogram) is not recommended forpre-participatory screening of all children.

● Most children with congenital heart disease growto become highly productive and functional adults.Although at times they may encounter difficulty inobtaining insurance, the majority are, in fact, inexcellent health. Most young women with congenitalheart disease are able to become pregnant and delivernormal newborns. A small number, such as those withpersistent cyanosis, or pulmonary hypertension, areconsidered at high risk and require careful multidisci-plinary approaches to issues such as employability,pregnancy, and contraception.

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