Neonate Cardiology

8/12/2019 Neonate Cardiology

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CASE 1: A NEONATE WITH A

HEART MURMUR

A full-term newborn delivered without complications isnoted to have a heart murmur at 1 day of age. The mur-

mur is described as a grade 2/6 systolic ejection-typemurmur heard best at the upper left sternal border. The

heart sounds are normal. The blood pressure is normal.The peripheral pulses are normal. The oxygen saturation

is 100% in room air. On day of life 3, the murmur haschanged from a systolic murmur to a continuous murmur

again heard best at the upper left sternal border. The babyappears well and is tolerating feeds without difficulties.

SELECT THE ONE BEST ANSWER

1. What is the likely cause of this heart murmur?(A) patent ductus arteriosus

(B) atrial septal defect

(C) ventricular septal defect(D) peripheral pulmonary stenosis

(E) aortic stenosis

2. This continuous murmur persists for more than72 hours. What is the most appropriate test to eval-

uate the etiology of this murmur?(A) chest radiograph

(B) electrocardiogram (ECG)

(C) complete blood count(D) echocardiogram(E) cardiac catheterization

3. The echocardiogram demonstrates a small patent

ductus arteriosus with no associated cardiac lesionsand no evidence of left heart volume overload.

What advice would you give to the family regardingfurther follow-up?

(A) no further cardiology follow-up is needed(B) there is a lifelong ongoing risk for endarteritis

and antibiotic prophylaxis should be given asneeded

(C) immediate surgical repair is required

(D) there is minimal risk for endarteritis and noantibiotic prophylaxis is needed

(E) B and C

4. At day of life 7, the neonate described developsincreased shortness of breath, decreased feeding,

and a chest radiograph shows pulmonary edema.What is the best first-line course of action?

(A) prostaglandin E1 infusion

(B) a course of indomethacin(C) supplemental oxygen

(D) nitric oxide(E) phenylephrine

5. A full-term newborn delivered via normal sponta-

neous vaginal delivery with Apgars of 9 at 1 minute

and 9 at 5 minutes is noted to have a grade 2/6 sys-tolic murmur at the left sternal border on day 1 of

life. Upon reexamination on day 4 of life, theneonate is found to have poor perfusion, poor

pulses, hypotension, and shock. What is the leastlikely cause of shock in this neonate?

(A) perinatal asphyxia(B) sepsis

(C) hypoglycemia(D) cardiogenic shock

(E) myocarditis

6. Which of the following congenital heart lesionsmay result in cardiogenic shock following sponta-

neous closure of the ductus arteriosus?(A) atrial septal defect

(B) hypoplastic left heart syndrome

(C) mitral valve prolapse(D) ventricular septal defect

(E) tetralogy of Fallot

Chapter 1

CARDIOLOGY

1

Chapter 1

CARDIOLOGY


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2 CHAPTER 1 CARDIOLOGY

7. What test would be most helpful to diagnose thecause of shock in this neonate?

(A) chest radiograph(B) arterial blood gas

(C) blood glucose level

(D) echocardiogram(E) a blood culture

8. What is the most appropriate acute treatment for

cardiogenic shock as a result of left heart obstruc-tive lesions?

(A) prostaglandin E1 infusion(B) indomethacin administration

(C) supplemental oxygen administration(D) nitric oxide

(E) intravenous (IV) furosemide (Lasix)

9. What are other courses of action to be taken forpatients with cardiogenic shock?

(A) inotropic medications such as epinephrine anddopamine

(B) ventilatory support(C) oxygen supplementation

(D) correct any metabolic abnormalities(E) all of the above

10. A neonate with a heart murmur on examination is

noted to have a bluish tinge to the lips. The baby issmiling and eating well in no apparent distress. The

baby is warm with good pulses. An oxygen satura-tion monitor was placed on the babys finger and the

oxygen saturation in room air is 80%. Which of the

following is (are) likely to account for this patientsfinding of cyanosis?

(A) cyanotic congenital heart disease(B) hemoglobinopathy

(C) respiratory disease(D) anemia

(E) A, B, and C

11. Which test would most likely help differentiate acardiac versus pulmonary cause for cyanosis?

(A) hyperoxia test(B) blood gas on room air

(C) chest radiograph(D) complete blood count

(E) chest computed tomography (CT)

12. What is the most common cyanotic heart lesion inthe neonate?

(A) transposition of the great arteries(B) total anomalous pulmonary venous return

(C) tricuspid atresia(D) tetralogy of Fallot

(E) Ebstein anomaly

13. What would be an appropriate first-line treatmentfor the management of cyanosis as a result of heart

disease?(A) prostaglandin E1 infusion

(B) indomethacin administration(C) nitric oxide

(D) surfactant

(E) dopamine

14. A 4-year-old with a known diagnosis of unrepairedtetralogy of Fallot becomes severely cyanotic while

crying. Which of the following is not indicated foracute management of this situation?

(A) administering of oxygen(B) placing the child in the knee-chest position

(C) administering morphine sulfate(D) rectal stimulation

(E) administration of phenylephrine

15. Which of the following is not a complication ofchronic cyanosis?

(A) polycythemia(B) stroke

(C) brain abscess(D) cognitive abnormalities

(E) cerebral aneurysms

ANSWERS

1. (A) The most likely cause of this murmur is a patent

ductus arteriosus (Figure 1-1). Atrial septal defects

are unlikely to cause a heart murmur at this early age,and ventricular septal defects cause a holosystolic

murmur that does not evolve into a continuousmurmur. Peripheral pulmonary stenosis is associated

with a systolic ejection murmur that radiates to theback and to both axillae. Aortic stenosis causes a

systolic murmur that does not evolve into a continuousmurmur. The ductus arteriosus is required for fetal

circulation; however, the structure usually closeswithin hours after delivery secondary to changes in

oxygen tension as well as other circulating factorssuch as prostaglandin. Persistence of the ductus

arteriosus occurs in approximately 0.8 per 1000 livebirths but is much more common in premature new-

borns with an incidence as high as 20% in neonatesweighing less than 1750 g. In patients with low oxy-

gen tension, pulmonary hypertension, or congenitalheart disease, there is delayed closure of the ductus

arteriosus.


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CASE 1 A NEONATE WITH A HEART MURMUR 3

2. (D) The ductus arteriosus in this neonate remains

patent after 72 hours. The most appropriate test toconfirm this is the echocardiogram. This test will

not only confirm the diagnosis of the patent ductusarteriosus but will also assess any associated car-

diac lesions. It is critical to check for pulmonaryartery hypertension or ductal-dependent cardiac

lesions when planning for closure of the patent duc-tus arteriosus.

3. (D) The presence of a small patent ductus arteriosus isunlikely to lead to significant left-to-right shunting orvolume overloading and therefore does not place the

child at risk for development of heart failure. There isabout a 5% incidence of spontaneous closure of a

small patent ductus arteriosus, and thus immediatesurgery is usually not required in that situation.

Recent review of subacute bacterial endocarditis has

suggested that the lifelong risk for bacterial endarteri-tis with the presence of a patent ductus arteriosus is

extremely low. Therefore, current guidelines do notrecommend antibiotic prophylaxis for this condition.

4. (B) In this situation, a large patent ductus arteriosus

has allowed significant left-to-right shunting result-ing in pulmonary edema, shortness of breath, and

left ventricular volume overload. The treatmentoptions at this point include administration of

indomethacin in an attempt to close the patent duc-tus arteriosus. Indomethacin is effective in approxi-

mately 80% of cases; however, its effectivenessdecreases if administered after 2 weeks of age. Other

treatment options for closure of a hemodynamicallysignificant ductus arteriosus include surgery or

transcatheter device closure. Both can be performed

safely and effectively with minimal morbidity andmortality. Administration of oxygen to the patient

with congestive heart failure because of a large left-to-right shunt would be detrimental in that it may

decrease pulmonary vascular resistance and increasethe degree of left-to-right shunting, exacerbating the

symptoms of heart failure. The same is true foradministration of nitric oxide in this situation.

Administration of phenylephrine would cause anincrease in systemic vascular resistance and result in

a greater degree of left-to-right shunting.

5. (A) The etiology of shock in the neonate includesthe following:

hypoglycemia asphyxia

sepsis intracranial bleeding

arrhythmias including tachyarrhythmias and

bradycardias cardiogenic shock because of left-sided obstruc-

tive lesions myocarditis

The least likely explanation of shock in this baby isasphyxia because there is no history of perinatal

asphyxia or distress based on the birth history.

6. (B) Cardiogenic shock may be the first presentation

in the neonate with congenital heart disease. Spe-

cific heart lesions causing left ventricular outflowtract obstruction may present in this manner. Theselesions include critical aortic stenosis, hypoplastic

left heart syndrome, and coarctation of the aorta. Apatent ductus arteriosus allows for blood to bypass

left-sided obstructions thus maintaining adequate

cardiac output. With closure of the ductus, cardiacoutput is diminished. The neonate with cardiogenic

shock from ductal-dependent lesions often presentsat 1-2 weeks of age with shock related to the spon-

taneous closure of the ductus arteriosus.

7. (D) An echocardiogram would be the most usefultest in this situation to determine if there is a ductal-

dependent cardiac lesion. It is also useful to detectprimary myocardial dysfunction related to other

causes of shock.

8. (A) The acute therapy for shock as a result ofductal-dependent cardiac lesions is infusion of

prostaglandin E1 in hopes of reestablishing patencyof the ductus arteriosus.

Patent ductusarteriosus (PDA)

FIGURE 1-1. Patent ductus arteriosus (PDA).


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9. (E) The other acute management strategies for shockin the neonate include inotropic support with

dopamine or epinephrine, ventilatory support incases of respiratory compromise, oxygen supple-

mentation, and correction of metabolic abnormalities

such as acidosis, hypokalemia, and hypocalcemia.

10. (E) Cyanosis can be divided into 2 clinical cate-gories: central or peripheral. Central cyanosis is a

result of a decrease in the oxygen saturation ofblood supplying the body. Peripheral cyanosis is a

benign finding caused by increased oxygen extrac-tion in distal capillary beds and is commonly seen

in normal neonates. The clinical detection ofcyanosis occurs when approximately 3-5 g/dL of

desaturated hemoglobin is present in the systemiccirculation. The most common causes of central

cyanosis in the neonate include cyanotic heart dis-ease, hemoglobinopathies, and respiratory distress.

11. (A) The test most likely to help differentiate cyan-

otic heart disease from respiratory disease is thehyperoxia test. In this test, 100% oxygen is adminis-

tered to the patient and the partial pressure of oxy-gen is measured. In patients with cyanotic heart

disease, the partial pressure of oxygen rarelyincreases above the level of 100 mm Hg, whereas in

lung disease there is usually some change from thebaseline PO2 with administration of oxygen. The

lack of response to oxygen in cyanotic heart diseaseis a result of fixed right-to-left shunting of desatu-

rated blood to the systemic circulation. Although the

other tests mentioned may be helpful in the evalua-tion of the cyanotic neonate, they are not as sensitive

in differentiating cardiac from respiratory etiologies.

12. (A) Cardiac lesions associated with cyanosisinclude

transposition of the great arteries (the most common) tetralogy of Fallot

truncus arteriosus tricuspid atresia

pulmonary atresia total anomalous pulmonary venous return

Other lesions include single-ventricle physiologysuch as hypoplastic left heart syndrome, or Ebstein

anomaly of the tricuspid valve with right-to-leftshunting across an atrial septal defect.

13. (A) In patients with cyanosis because of heart

disease, there is either obligate mixing of saturatedand desaturated blood reaching the systemic circu-

lation (eg, truncus arteriosus) or decreased effective

pulmonary blood flow. In either situation, promo-tion of increased pulmonary blood flow or

improved mixing of desaturated and saturatedblood would increase the systemic oxygen satura-

tion. This is acutely achieved by infusion of

prostaglandin E1 to reestablish (or maintain)

patency of the ductus arteriosus. Therefore, in situ-

ations where cyanosis because of heart disease issuspected, infusion of prostaglandin E1 should be

instituted as soon as possible.

14. (D) Patients with tetralogy of Fallot are at risk for ahypercyanotic tet spell. This usually occurs in

patients older than 2 years of age, although it hasbeen reported at younger ages. The mechanism of a

hypercyanotic spell is acute and progressive pul-monary stenosis, with increased right-to-left shunt-

ing of desaturated blood to the systemic circulation.This is precipitated by an increase in circulating

catecholamines, anxiety, hypoxia, or dehydration.Interventions to break this cycle include adminis-

tration of oxygen, morphine, propranolol, orphenylephrine, placing the child in a knee-chest

position. In extreme cases, muscle relaxation andintubation are required. Rectal stimulation would

serve to worsen the crisis by increasing the cate-cholamine levels (Figure 1-2).

FIGURE 1-2. Tetralogy of Fallot.


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CASE 2 A 10-YEAR-OLD BOY WITH PALPITATIONS 5

15. (E) Complications in patients with chronic cyanosisinclude polycythemia (compensation for decreased

systemic oxygen tension), and risk of strokesbecause of an increased propensity for forming

blood clots and increased blood viscosity. This is

particularly concerning when the hematocrit ishigher than 70%. A relative anemia may be seen in

patients with cyanosis with low hemoglobinindexes. Some have suggested that this increases

the risk of stroke because of increased blood viscos-ity. Another complication is brain abscess formation

because of poor venous blood flow and increasedsusceptibility to infection. Impaired cognitive func-

tion is associated with long-standing cyanosis. Thedegree of impairment is related to both the degree

and duration of cyanosis.

SUGGESTED READING

Allen HD, Driscoll DJ, Shaddy RE, Feltes TF, eds. Moss and

Adams Heart Disease in Infants, Children and Adolescents.

7th ed. Philadelphia, PA: Williams and Wilkins; 2007.

Braunwald E, Zipes DP, Libbey P, eds. Braunwald: Heart Dis-

ease: A Textbook of Cardiovascular Medicine. 7th ed.

Philadelphia, PA: WB Saunders; 2004.

Keane JF, Fyler DC, Lock JE, eds. Nadas Pediatric Cardiology.

2nd ed. Philadelphia, PA: Hanley and Belfus; 2006.

Park M, ed. Pediatric Cardiology for Practitioners. 5th ed. St.

Louis, MO: Mosby; 2007.

CASE 2: A 10-YEAR-OLD BOY

WITH PALPITATIONS

A 10-year-old boy presents to the clinic with a history ofintermittent episodes of palpitations occurring 1 time

per month. The episodes are not associated with exer-cise or activity, last for several minutes, and resolve

spontaneously. There is no significant past medical his-

tory, no new medications, and no dizziness or syncope.

A 12-lead ECG, performed while he is asymptomatic, isnormal.


1. Which test would best help to evaluate the etiology

of this patients palpitations?

(A) echocardiogram(B) chest radiograph

(C) event recorder monitor(D) exercise stress test

(E) tilt table test

Match the ECG rhythm strips in questions 2 through 5 to

one of the findings below.

(A) premature atrial contractions(B) premature ventricular contractions

(C) sinus tachycardia(D) sinus arrhythmia

2. Figure 2-1 shown below



6. What is the most likely cause of supraventricular

tachycardia in the patient whose history was pre-sented above?

(A) a concealed accessory bypass tract causing

orthodromic, reciprocating tachycardia(B) AV node reentry tachycardia

(C) ectopic atrial tachycardia(D) atrial flutter

(E) atrial fibrillation


FIGURE 2-1.

FIGURE 2-2.

FIGURE 2-3.

FIGURE 2-4.


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7. What is an appropriate acute therapy for sympto-matic orthodromic, reciprocating tachycardia?(A) DC cardioversion(B) IV adenosine(C) vagal maneuvers(D) all of the above(E) none of the above

8. Which of the following agents is considered first-line therapy for chronic control of orthodromic,reciprocating tachycardia?(A) propafenone(B) atenolol(C) sotalol(D) amiodarone(E) flecainide

9. What would be the most appropriate acute therapyfor symptomatic ectopic atrial tachycardia?(A) DC cardioversion

(B) IV adenosine(C) vagal maneuvers(D) IV esmolol(E) none of the above

10. A child with supraventricular tachycardia (SVT) isfound to have a resting ECG rhythm strip shown inFigure 2-5. What is the diagnosis?

(A) digoxin(B) verapamil

(C) propranolol(D) amiodarone

(E) flecainide

13. A 10-year-old child experiences palpitations associ-

ated with dizziness when standing. He has had oneepisode of syncope following standing in school.

What is the most common etiology of syncope inthis age group?

(A) seizures(B) cardiac disease

(C) hypoglycemia(D) neurocardiogenic (vasovagal)

(E) cardiac arrhythmia

14. Which of the following features would suggest neu-rocardiogenic syncope?

(A) urinary incontinence during episodes ofsyncope

(B) auditory aura preceding episode of syncope(C) transient right arm paralysis following episode

of syncope(D) symptoms of dizziness, blurred vision,

diaphoresis preceding syncope(E) prolonged episode of unconsciousness

15. Which of the following is true regarding tilt table

testing for syncope?(A) tilt table testing should be performed for every

patient with syncope

(B) the results of tilt table testing are highly repro-ducible for an individual

(C) tilt table testing is reserved for complicated caseswhere the diagnosis of syncope is uncertain

(D) tilt table testing is useful for predictingresponse to medications

(E) tilt table testing should be performed beforeinitiation of medical therapy

16. Anewborn baby presents with a heart rate of 45 beats

per minute (bpm), a normal blood pressure, andgood perfusion. The ECG rhythm strip is shown in

Figure 2-6. What is the diagnosis?(A) first-degree AV block

(B) second-degree AV block (type 1)(C) second-degree AV block (type 2)

(D) third-degree AV block(E) sinus bradycardia

(A) sinus tachycardia(B) ventricular preexcitation

(C) ventricular tachycardia(D) sinus rhythm(E) sinus arrhythmia

11. Which statement is true regarding the diagnosis ofWolff-Parkinson-White (WPW) syndrome?(A) there is an increased risk of sudden cardiac death(B) there is an increased risk of ectopic atrial

tachycardia(C) there is an increased risk of tachycardia-

induced cardiomyopathy(D) there is no risk of atrial fibrillation(E) there is no increased risk for associated struc-

tural heart disease

12. Which of the following agents is considered first-line therapy for chronic control of orthodromically,reciprocating tachycardia associated with ventricu-lar preexcitation (WPW syndrome)?

FIGURE 2-5.

P = p wave

FIGURE 2-6.


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17. What is the most likely cause of congenital AVblock in newborns?

(A) maternal lupus(B) maternal rubella

(C) maternal use of lithium

(D) maternal diabetes mellitus(E) maternal use of alcohol

18. The patient from Question 16 subsequently devel-

ops signs of heart failure with poor perfusion andtachypnea. What is the most appropriate long-term

therapy for this patient?(A) pacemaker implantation

(B) digoxin(C) theophylline

(D) atropine(E) caffeine

19. A 10-year-old patient with history of palpitations

is found to have the ECG rhythm strip shown inFigure 2-7. What is the most likely diagnosis of

the palpitations?

with acute arrhythmias in less than 5% of caseswhen evaluated with long-term monitoring. Palpita-

tions associated with symptoms of dizziness orsyncope or in the setting of a family history of

arrhythmias or sudden cardiac death would increase

the suspicion of an underlying cardiac disorder.Although the echocardiogram, stress test, and chest

radiograph are often used to exclude significant car-diac disease, they are unlikely to determine the spe-

cific etiology of the palpitations. In this situation, anevent recorder could be used to record an ECG

rhythm strip during symptoms. An event recorder isa long-term monitoring device used for patients

with symptoms that occur infrequently.

2. (B) The ECG in Figure 2-1 shows normal sinusrhythm with a premature ventricular contraction.

Premature ventricular contractions are a less com-mon finding in young patients occurring with an

incidence of approximately 5-10%. They are usu-ally benign; however, they may rarely be associated

with significant heart disease. Therefore, the evalu-ation often includes an echocardiogram or stress

test to assess for associated cardiac disease. Prema-ture ventricular contractions that are not associated

with significant heart disease do not require therapyunless they are associated with significant symp-

toms of discomfort or palpitations.

3. (C) The ECG in Figure 2-2 shows sinus tachycardia.The more common etiologies of sinus tachycardia

at this age include anxiety or emotion, hyperthy-

roidism, anemia, postural orthostatic tachycardiasyndrome, and dehydration. It is the most common

cause of palpitations in young patients.

4. (D) The ECG in Figure 2-3 shows sinus arrhythmia.This is a benign finding associated with an irregular

heart rhythm heard during auscultation. It mayvary with respiration and can be pronounced in

children. There is no association with significantheart disease.

5. (A) The ECG in Figure 2-4 demonstrates normal

sinus rhythm with a single premature atrial contrac-tion. Premature atrial contractions are a common

benign finding in young patients occurring with anincidence of approximately 17-25%. They are usually

not associated with significant cardiac disease.Because of the benign nature of the premature atrial

contractions, they do not require further investigation.

FIGURE 2-7.

(A) myocardial ischemia(B) orthodromic reentry tachycardia

(C) ventricular arrhythmias(D) premature atrial beat

(E) premature junctional beats

20. What are the known modes of inheritance for con-genital long QT syndrome?

(A) autosomal recessive inheritance(B) autosomal dominant inheritance

(C) spontaneous mutation(D) all of the above

(E) it is not known

21. Which of the following medications is associatedwith drug-induced long QT syndrome?

(A) propranolol

(B) amitriptyline(C) metoclopramide

(D) amoxicillin(E) ibuprofen

ANSWERS

1. (C) Palpitations are a common complaint in youngpatients reported by 16% of patients in a survey at a

large primary care clinic. Palpitations are associated


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6. (A) The most likely cause of supraventriculartachycardia at this age is orthodromic, reciprocating

tachycardia because of an accessory bypass tract(concealed in this case based on the normal

resting ECG). AV node reentry tachycardia is less

common at this age; however, this becomes the pre-dominant mechanism of tachycardia in young

adults. Ectopic atrial tachycardia and atrial flutteroccur with a low incidence throughout childhood.

Atrial flutter is more commonly seen in patientswho have congenital heart disease.

7. (D) Acceptable therapy for orthodromic, reciprocat-

ing tachycardia that occurs because of a concealedbypass tract includes DC cardioversion, IV adeno-

sine, or vagal maneuvers. Other antiarrhythmicdrugs such as calcium channel blockers can also be

used. The determination of which therapy is used isbased on the patients clinical status at the time of

presentation. In patients with stable tachycardia andvital signs, vagal maneuvers or IV adenosine may

be first-line treatment. In patients with unstabletachycardia, DC cardioversion may be the first-line

therapy. Of note, IV calcium channel blockers are notrecommended for use in infants younger than 1 year

of age secondary to an increased risk for hypotension.

8. (B) First-line medical therapy for chronic treatmentof supraventricular tachycardia includes digoxin,

calcium channel blockers, and beta-blockers (eg,

atenolol). Amiodarone, flecainide, and sotalol are

all effective drugs for treatment of SVT but have a

higher incidence of side effects (proarrhythmia).Thus they are usually reserved for cases that are

resistant to first-line medications.

9. (D) Ectopic atrial tachycardia is a rare tachycardiaoccurring with an incidence of approximately 10%

throughout childhood. In some cases of automaticatrial tachycardia, the heart rate is only slightly

higher than sinus rhythm; thus the tachycardia maygo undetected for months to years. Patients with

ectopic tachycardia are at risk for ventricular dys-function and heart failure. Unlike orthodromic,

reciprocating tachycardia and AV node reentrytachycardia, ectopic atrial tachycardia arises from a

single atrial focus that does not depend on the AVnode. Therefore, adenosine, vagal maneuvers, and

DC cardioversion are ineffective. Appropriate acutetherapy includes IV beta-blockers, calcium channel

blockers, or amiodarone.

10. (B) The ECG in Figure 2-5 demonstrates ventricu-lar preexcitation (short PR interval and slurred

upstroke of the QRS or delta wave) because of an

accessory bypass tract. When associated with

tachycardia, this is known as the Wolff-Parkinson-

White (WPW) syndrome. The incidence of WPWsyndrome is approximately 0.3%. The mechanism

of tachycardia is orthodromic, reciprocating tachy-cardia similar to that in patients with concealed

bypass tracts.

11. (A) There is an increased risk of sudden cardiacdeath (approximately 1 in 1000 patient-years) in

WPW syndrome. Sudden cardiac death occursbecause of rapid conduction over the accessory

bypass tract during atrial tachycardia resulting inventricular compromise. Patients with WPW syn-

drome are not at increased risk for ectopic atrialtachycardia or tachycardia-induced cardiomyopa-

thy, but there is an increased incidence of atrial fibril-lation in this population.

12. (C) First-line chronic medical therapy for patients

with WPW syndrome includes beta-blockers orother antiarrhythmic medications such as flecainide

or amiodarone. The use of digoxin and/or verapamilis contraindicated in these patients because these

agents may potentiate rapid antegrade conductionover the accessory pathway and may increase the

risk for sudden death.

13. (D) The most common etiology of syncope in

young patients is neurocardiogenic or vasovagalsyncope. This occurs in up to 20% of the popula-

tion. Other possibilities include neurologic (eg,seizures), metabolic (eg, anemia, dehydration), or

cardiac (eg, arrhythmia) causes.

14. (D) Typical features of neurocardiogenic syncopeinclude preceding symptoms of nausea, dizziness,

diaphoresis, or blurred vision. The episodes arerelated to position and usually occur when sitting or

standing. Loss of consciousness is brief and resolvesspontaneously. The other features mentioned are

suggestive of syncope because of a seizure.

15. (C) Tilt table testing (Figure 2-8) is a simple test usedto reproduce neurocardiogenic syncope. However,

the low sensitivity and specificity as well as the lackof reproducibility of the test limit its usefulness. It is

usually reserved for difficult situations where theetiology of syncope is uncertain. It is not recom-

mended for all patients with syncope and is not usefulfor predicting response to medications.


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16. (D) The ECG rhythm strip in Figure 2-6 demon-

strates third-degree AV block. This is defined ascomplete dissociation of the atrial and ventricular

electrical activity.

17. (A) The most common cause of congenital com-plete AV block is maternal lupus. The pathogenesis

is thought to be a result of maternal transfer of lupusantibodies (anti-Ro or anti-La) to the fetus and sub-

sequent damage of the developing conduction sys-tem. In patients with congenital AV block, the

chances of their mother having anti-Ro or anti-Laantibodies are approximately 60-70%. For mothers

with lupus, there is a 5% incidence of having a childwith complete AV block. Other causes of AV block

in the neonate include structural heart disease,myocarditis, and metabolic disease.

18. (A) In patients with congenital AV block and symp-

tomatic bradycardia, the treatment of choice ispacemaker implantation. No other therapy has been

shown to affect long-term outcome in thesepatients. Medications are not consistently effective

for prevention of bradycardia and therefore are not

recommended for chronic therapy. Other indica-tions for pacemaker implantation in patients with

complete heart block include a wide QRS escaperhythm, a prolonged QT interval, an asymptomatic

infant with a heart rate less than 55 bpm, an asymp-tomatic child with a heart rate less than 50 bpm, and

asymptomatic complete heart block with a heartrate less than 75 bpm associated with congenital

heart disease.

19. (C) The ECG in Figure 2-7 demonstrates a pro-longed QT interval. The QT interval is usually cor-

rected for heart rate (QTc) and normal limits varywith age and gender. Palpitations associated with

this ECG would most likely be a result of a poly-

morphic ventricular tachycardia known as torsadesde pointes. The ventricular arrhythmia may be self-

limited or result in symptoms of dizziness, syncope,or death. The occurrence of torsades de pointes is

associated with exercise or activity; however, insome cases it may occur at rest.

20. (D) Congenital long QT syndrome is most com-

monly inherited in an autosomal dominant manner(Romano-Ward). It is a result of a defect in 1 of 5

known genes that affect potassium and sodium ionchannels in the heart. A specific gene defect has

been identified in 50% of patients with clinical longQT syndrome. A less common mode of inheritance

is autosomal recessive (Jervell and Lange-Nielsensyndrome). In these patients, there is often associ-

ated conductive hearing loss and a higher risk forventricular arrhythmias and sudden death. Finally,

some cases of congenital long QT syndrome occurbecause of spontaneous mutations. The risk for sud-

den death in patients with long QT syndrome whopresent with symptoms of dizziness or syncope is

approximately 20% in 1 year, if untreated. The use

of beta-blockers decreases the risk of sudden deathto < 5%. Therefore, beta-blockers have become the

mainstay of therapy as well as avoidance of strenu-ous activities, especially swimming. Pacemaker

implantation is occasionally required for patientswith either baseline bradycardia or those who

develop bradycardia following treatment with beta-blockers. Implantable cardioverter defibrillators are

now used more frequently in patients who continueto become symptomatic despite medical therapy

with a beta-blocker or for those in whom there is astrong family history of sudden death.

21. (B) Acquired long QT syndrome can occur with

head trauma, myocardial infarction, cardiomyopa-thy, or as a result of drugs or medications. This list

of drugs/medications associated with the acquiredprolonged QT syndrome is extensive and updated

regularly. Some of the more common medicationson this list include amitriptyline or other tricyclic

antidepressants, erythromycin, and cisapride.

FIGURE 2-8. (Reproduced, with permission, from Prystowsky

EN, Klein GT. Cardiac Arrhythmias: An Integrated Approach for

the Clinician. New York: McGraw-Hill; 1994:353.)


10/24


SUGGESTED READING







Keane JF, Fyler DC, Lock JE, eds. Nadas Pediatric Cardiology.




CASE 3: A 7-YEAR-OLD CHILD

WITH A HEART MURMUR

A 7-year-old boy with no significant past medical his-

tory is noted to have a heart murmur heard for the firsttime during a routine physical evaluation by his lifelong

pediatrician. The physical examination is significant fora grade 2/6 vibratory systolic murmur heard best at the

lower left sternal border with no radiation. The remain-der of the physical examination is normal.


1. What is the most likely diagnosis of this heartmurmur?

(A) Still murmur(B) ventricular septal defect

(C) atrial septal defect(D) peripheral pulmonary stenosis

(E) venous hum

2. What supporting evidence would help to confirmthe diagnosis of an innocent Still murmur?

(A) a fixed, split second heart sound

(B) the murmur is louder in the supine positioncompared to the sitting or standing position

(C) a diastolic rumble(D) the murmur is associated with a precordial

thrill (grade IV)(E) radiation to the back and axilla

3. Which test would not be indicated for this patient atthis time?

(A) ECG(B) echocardiogram

(C) chest radiograph

(D) cardiac catheterization(E) four extremity blood pressure determination

4. What would be the appropriate advice to give the

family regarding a child with this murmur?(A) endocarditis prophylaxis is needed

(B) rigorous sports should be avoided(C) sports participation is allowable

(D) annual echocardiograms are required(E) other family members should be screened for

heart disease

5. A 6-week-old infant presents with a heart murmurand a history of poor feeding, diaphoresis, and

tachypnea. On examination, he is playful buttachypneic with a grade 2/6 systolic regurgitant

murmur heard best in the mid left sternal border,with hepatomegaly, rales, intermittent wheezes,

cool extremities with normal distal pulses, and anactive precordium. What is the likely diagnosis of

this patient?(A) chronic lung disease

(B) acute upper respiratory infection(C) congestive heart failure

(D) dehydration

(E) hyperthyroidism

6. Which test would not be helpful in evaluating thispatient?

(A) an echocardiogram(B) an ECG

(C) a chest radiograph(D) a throat culture

(E) a physical examination

7. What heart lesion is least likely to present withcongestive heart failure symptoms and signs at age

6 weeks?(A) ventricular septal defect

(B) patent ductus arteriosus(C) atrial septal defect

(D) aortopulmonary window(E) large coronary artery fistula


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CASE 3 A 7-YEAR-OLD CHILD WITH A HEART MURMUR 11

8. Which feature may help to distinguish heart failurefrom an anomalous left coronary artery arising from

the pulmonary artery versus heart failure secondaryto a ventricular septal defect?

(A) extreme irritability and crying at the onset of

feeding(B) diaphoresis

(C) weight loss(D) decreased frequency of wet diapers

(E) tachypnea

9. Which therapy would not be indicated for the acutemanagement of congestive heart failure because of

a left-to-right shunting lesion?(A) digoxin

(B) oxygen supplementation(C) dobutamine

(D) furosemide (Lasix)(E) enalapril

10. A 2-year-old patient presents with symptoms of

heart failure. An echocardiogram demonstrates nointracardiac lesion but a dilated, poorly functioning

left ventricle. What is the least likely cause of thedilated cardiomyopathy in this patient?

(A) myocarditis(B) metabolic disease

(C) idiopathic(D) myocardial ischemia

(E) tachycardia-induced cardiomyopathy

11. Which of the following would be supportive evi-

dence of the diagnosis of myocarditis?(A) an endomyocardial biopsy showing fibrosis

(B) an endomyocardial biopsy showing fibrosisand inflammation

(C) systolic flow murmur(D) an S3 gallop on cardiac examination

(E) hepatomegaly

12. What is the most common cause of myocarditis?(A) Staphylococcus aureus

(B) streptococcus infection(C) Epstein-Barr virus

(D) enterovirus(E) influenza A virus

13. Which of the following should be avoided for the

treatment of acute myocarditis?(A) dopamine

(B) IV immunoglobulin(C) supportive therapy with diuretics

(D) IV digoxin(E) dobutamine

14. Which therapy would not be considered standardmanagement for patients with chronic congestive

heart failure?(A) beta-blockers

(B) diuretics

(C) angiotensin-converting enzyme (ACE) inhibitors(D) calcium channel blockers

(E) digoxin

15. A 7-year-old patient presents with heart murmur,chest pain, and shortness of breath. The murmur is a

continuous type that varies in quality with positionchanges. The patient seems to have relief when

sitting and leaning forward. The heart sounds aredistant. What is the likely diagnosis for this patient?

(A) pulmonary embolus(B) pericarditis

(C) myocardial infarction(D) gastroesophageal (GE) reflux

(E) pleural effusion

16. What is the most common cause of this 7-year-oldsdiagnosis among young patients in the United States?

(A) viral infection(B) acute rheumatic fever

(C) bacterial infection(D) collagen vascular disease

(E) drug induced

17. While in the office, the 7-year-old patient becomeslethargic with poor perfusion and hypotension.

What is the most likely explanation?

(A) pulmonary embolus(B) myocardial infarction

(C) stroke(D) cardiac tamponade

(E) arrhythmia

ANSWERS

1. (A) The patient most likely has a Still murmur. This

is described as a vibratory or musical systolic ejec-tion murmur occurring at the left sternal border with

no other associated cardiac findings. It is the mostcommon innocent heart murmur in children and

usually presents at 2-7 years of age. Other innocentmurmurs include pulmonary outflow tract murmur,

the peripheral pulmonary stenosis murmur of thenewborn, and a venous hum. The incidence of inno-

cent heart murmurs in young patients after infancyis approximately 17-66%.


12/24


2. (B) Features of innocent heart murmurs include notonly the quality and location of the heart murmur

but also the fact that the first and second heartsounds are normal. The murmurs are usually well-

localized without much radiation and are usually

graded between 1 and 3 with no associated precor-dial thrill. The murmurs are usually described as

vibratory, musical, or blowing, and they are louderin the supine position compared with the sitting or

standing position. This is not the case with the inno-cent venous hum that is often louder in the sitting

position. A venous hum can be distinguished by thegreat amount of variability in quality with position

changes and with turning the head. Innocent mur-murs do not typically have a diastolic component. A

split, fixed second heart sound is associated with anatrial septal defect.

3. (D) Common tests performed in evaluation of inno-

cent heart murmurs include 12-lead ECGs, chestradiographs, 4-extremity blood pressure (to rule out

coarctation of the aorta) and echocardiograms. Theuse of these tests is at the discretion of the examin-

ing physician and depends on the findings on phys-ical examination as well as the past medical and

family history. An invasive procedure such as a car-diac catheterization is usually not recommended for

evaluation of an innocent heart murmur if the testsjust listed have been normal.

4. (C) Innocent heart murmurs are not associated with

any increased risk for bacterial endocarditis; thus

prophylaxis is not needed. Sports participation isnot restricted because there is not an increase in car-

diac events associated with innocent heart mur-murs. Innocent murmurs, by definition, are not

associated with structural heart disease, so yearlyechocardiograms are usually not recommended.

There is no familial association with structural heartdisease. Depending on the age at time of diagnosis,

a follow-up visit is occasionally recommended foryounger patients.

5. (C) The child presents with evidence of congestive

heart failure. In addition to the clinical findingsdescribed, other findings associated with congestive

heart failure include edema, usually of the eyelidsor in dependent areas, jugular venous distention, an

S3 or S4 gallop on examination, and cardiomegaly orpulmonary edema demonstrable on chest radiograph.

6. (D) Evaluation of a young patient with congestive

heart failure requires a physical examination toassess for edema, hepatomegaly, pulmonary con-gestion, and cardiac gallop. An echocardiogram is

used to assess cardiac function and any associated

cardiac structural abnormalities. A 12-lead ECG isused to assess for any ischemic changes, arrhyth-

mias, or bradycardia. A chest radiograph is helpfulto assess for cardiomegaly or pulmonary edema as

well as to assess for any obvious primary pul-monary disorders. A throat culture would not be

useful at this age for evaluation of heart failure.

7. (C) Cardiac lesions that present with heart failure atthis age are usually a result of an increase in the

amount of left-to-right shunting secondary to thenatural decrease in the pulmonary vascular resist-

ance. Common left-to-right shunting lesionsinclude a ventricular septal defect, a patent ductus

arteriosus, a large coronary artery fistula, and anaortopulmonary window. Atrial septal defects do

not usually present with heart failure at this agemainly because the amount of left-to-right shunting

depends on the compliance of the right ventriclerather than the drop in pulmonary vascular resistance.

8. (A) An anomalous left coronary artery arising from

the pulmonary artery is a rare congenital heartdefect. The right coronary artery arises normally

from the aorta. A number of collateral vesselsdevelop between the right and left coronary arteries,

and thus, when there is a drop of pulmonary vascu-

lar resistance, a steal phenomenon occurs withcoronary blood essentially flowing from the aorta to

the right coronary artery across the collaterals to theleft coronary artery and into the pulmonary artery.

Because of this, certain areas of the myocardium areat risk for ischemia. Patients with this lesion often

present at 6-8 weeks of age with extreme irritability(especially at the onset of feeding) that is a result of

angina. Other symptoms such as diaphoresis,weight loss, hepatomegaly, and decreased fre-

quency of wet diapers occur at a similar frequencyto other conditions associated with heart failure.


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CASE 3 A 7-YEAR-OLD CHILD WITH A HEART MURMUR 13

9. (B) Acute management of heart failure because ofleft-to-right shunting lesions include inotropic

support with digoxin, dopamine, dobutamine, orepinephrine. Diuretics are also used to relieve

symptoms of edema. Milrinone is an inotrope and

afterload-reducing agent that is commonly employedfor the treatment of heart failure. Use of oxygen

may exacerbate symptoms of heart failure in thepresence of left-to-right shunting lesions by decreas-

ing pulmonary vascular resistance and increasingthe degree of left-to-right shunting. The ultimate

long-term therapy is to eliminate the left-to-rightshunting lesion either with surgery or interventional

cardiac catheterization.

10. (D) Myocardial dysfunction resulting in dilatedcardiomyopathy is uncommon in the pediatric popula-

tion. The causes include myocarditis, metabolic dis-eases, idiopathic, familial dilated cardiomyopathy,

or tachycardia-induced cardiomyopathy. Ischemicheart disease is a rare cause of dilated cardiomyopa-

thy at this age but is the most common cause in theadult population.

11. (B) Myocarditis is defined as inflammation of the

myocardium thought to occur because of a cell-mediated immunologic reaction. The diagnosis is

confirmed by endomyocardial biopsy showingfibrosis and inflammation. A biopsy showing fibro-

sis alone may be seen with any form of cardiomy-opathy and is not specific for acute myocarditis.

Other features suggestive of myocarditis include

tachycardia out of proportion to the symptoms of heartfailure, cardiac dysfunction, a pericardial effusion,

and a preceding history of upper respiratory illness.A systolic flow murmur, hepatomegaly, and S3 gallop

may occur with myocarditis but are nonspecificfindings for this diagnosis.

12. (D) The most common cause of myocarditis is

infection due to enterovirus (eg, coxsackievirus andechovirus) as demonstrated by polymerase chain

reaction (PCR) of endomyocardial biopsies. Otheragents include other viruses (such as adenovirus and

epstein barr), bacteria, rickettsia, fungi, protozoa,and parasites. Other etiologies include immune-

mediated diseases, toxic myocarditis, and collagenvascular diseases.

13. (D) The acute management of myocarditis includesthe use of steroids, IV immunoglobulins, diuretics,

and inotropic support. Use of IV digoxin during theacute phase of myocarditis is associated with

increased occurrence of arrhythmias and is thus typ-

ically not recommended. There are conflicting dataregarding the efficacy of these therapies, and no sin-

gle therapy has shown to significantly improve thelong-term outcome in patients with myocarditis.

Generally, most patients improve and have com-plete recovery, although the acute mortality rate

may be as high as 75% in neonates.

14. (D) In patients with ventricular dysfunction andchronic congestive heart failure, therapy includes

the use of diuretics and digoxin. The use ofafterload-reducing agents such as ACE inhibitors

has been shown to reduce morbidity and mortality.The recent use of beta-blockers has been shown to

decrease myocardial oxygen demand and alsodecreases morbidity and mortality among adults

with ventricular dysfunction. The use of calciumchannel blockers is not recommended because of the

significant negative inotropic effect. The long-termmanagement for patients with ventricular dysfunc-

tion and heart failure includes medical therapy androutine monitoring with exercises tests to obtain

objective data regarding cardiac performance.

15. (B) The most likely diagnosis of this patient is acute

pericarditis. This is defined as inflammation of the

parietal and visceral pericardium and results in

serous hemorrhagic or purulent pericardial effusion.The clinical manifestations include a pericardial

friction rub and the presence of a dull substernalchest pain that improves when leaning forward.

There may be a history of upper respiratory tractinfection and fever.

16. (A) The most common agents causing pericarditis

in the United States are viruses. Acute rheumaticfever is a common cause in certain parts of the world.

Other causes include bacterial infection, collagenvascular disease, tuberculosis, oncologic disease,

and uremia.


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15/24

CASE 4 A 5-YEAR-OLD CHILD WITH A HEART MURMUR AND FEVER 15

7. The heart murmur in this patient is most likely aresult of which of the following?

(A) mitral valve regurgitation(B) mitral valve stenosis

(C) tricuspid valve regurgitation

(D) pulmonary valve regurgitation(E) aortic valve stenosis

8. A 2-year-old child presents with a 10-day history of

fever, a heart murmur, bilateral nonexudative con-junctivitis, swollen and erythematous lips and

strawberry tongue with erythematous and edema-tous hands and feet, and a polymorphous rash on the

face, trunks, and extremities. The most likely diag-nosis in this patient is

(A) Kawasaki disease(B) measles

(C) viral upper respiratory tract infection(D) group A beta-hemolytic streptococcal pharyngitis

(E) Hodgkin lymphoma

9. What acute finding would not be expected to beassociated with this 2-year-olds diagnosis?

(A) sterile pyuria(B) hydrops of the gallbladder

(C) cervical adenopathy with at least one nodelonger than 1.5 cm

(D) thrombocytosis(E) A and B

10. Which of the following statements is true regarding

coronary artery involvement with this 2-year-olds

disease?(A) there is a 50% incidence of coronary artery

aneurysms if untreated(B) the peak incidence for coronary artery

aneurysms is 6-12 months following the onsetof fever

(C) patients with giant coronary artery aneurysms(>8 mm in diameter) are at highest risk for late

stenosis and myocardial infarction(D) coronary artery rupture is the most common

cause of mortality within the first 7 days of theonset of fever

(E) coronary artery aneurysms are not at risk fordevelopment of thrombus

11. The 2 drugs most commonly used for the acute

management of this disease are(A) aspirin and intravenous immunoglobulin

(IVIG)(B) penicillin and IVIG

(C) steroids and aspirin

(D) steroids and penicillin(E) penicillin and aspirin

12. A 5-year-old boy presents with a fever of 10 days,

weight loss, night sweats, a new heart murmur,splenomegaly, joint pains, and a history of having

had his teeth cleaned by the dentist 1 month prior to

this visit. On examination, he was found to have agrade 2/4 diastolic murmur heard best at the mid-

left sternal border radiating to the apex. There is anS3 gallop rhythm. What is the most likely diagnosis

for this patient?(A) infective endocarditis

(B) juvenile rheumatoid arthritis(C) Kawasaki disease

(D) acute rheumatic fever(E) mononucleosis

13. Which of the following laboratory evidence most

likely supports the diagnosis of infective endocarditis?(A) two positive blood cultures with the same

organism and no other source other than theheart

(B) increased white blood cell count(C) increased C-reactive protein (CRP)

(D) throat culture positive for group A beta-hemolytic streptococcus

(E) increased erythrocyte sedimentation rate (ESR)

14. Which bacterial species would be the most commoncause of infective endocarditis in this 5-year-old

child?

(A) alpha-hemolytic streptococcus(B) Staphylococcus aureus

(C) Staphylococcus epidermidis(D) Enterococcus faecalis

(E) Bacteroides fragilis or other anaerobe

15. What percentage of cases of infective endocarditisare blood culture negative?

(A) 10%(B) 20%

(C) 30%(D) 40%

(E) 50%

16. Which of the following heart lesions would not beconsidered an increased risk for the development of

infective endocarditis?(A) mitral regurgitation

(B) aortic insufficiency(C) aortic stenosis

(D) atrial septal defect(E) ventricular septal defect


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17. What is the most common antibiotic regimen thatshould be started in cases of suspected infective

endocarditis before knowing the results of the bloodculture?

(A) vancomycin or oxacillin and gentamicin

(B) ampicillin and gentamicin(C) ampicillin and ceftriaxone

(D) vancomycin alone(E) none of the above

18. Which of the following procedures does not require

endocarditis prophylaxis in patients who are sus-ceptible to endocarditis?

(A) tonsillectomy(B) urinary tract surgery

(C) professional dental cleaning(D) endotracheal intubation

(E) cardiac surgery

ANSWERS

1. (A) The most common cause of this constellation of

symptoms is acute rheumatic fever, a multisysteminflammatory disease. The disease occurs among

children 5 to 15 years of age with a peak inci-dence at 8 years and is rarely seen in children

younger than 2 years of age.

2. (A) Acute rheumatic fever is triggered by group Abeta-hemolytic streptococcal infection of the upper

respiratory tract. Acute rheumatic fever does not

occur following streptococcal skin infections, staphy-lococcal infections, or enterococcal infections. The

pathogenesis of rheumatic fever is thought to be sec-ondary to an immune response to antigens in the M

protein of the capsule of the group A beta-hemolyticstreptococcus, which occurs in susceptible hosts and

cross-reacts with similar epitopes in human joint tis-sue, heart, and brain tissue. Pathologic findings

include inflammatory lesions that include perivascu-lar granulomas consisting of infiltrates of cells and

fibrin that are also known as Aschoff bodies.

3. (B) The incidence of acute rheumatic fever isapproximately 0.3-3% in untreated patients with

Streptococcus pyogenes pharyngitis. The onset ofdisease occurs 1-5 weeks later with a mean of 18

days following the onset of pharyngitis. The typicalcourse includes pharyngitis with improvement of

symptoms. Two weeks later the patient begins todevelop a low-grade fever and the inflammatory

response of rheumatic fever.

4. (A)

5. (D) Although there is no specific diagnostic labora-tory test for rheumatic fever, the diagnosis is based

on the Jones criteria. These are separated into majorand minor manifestations. Major criteria include

polyarthritis, carditis, erythema marginatum, subcu-

taneous nodules, and chorea. The most commonmanifestation is polyarthritis occurring in up to 70%

of patients, typically a migratory arthritis involvingthe large joints (knees, hips, ankles, elbows), which

characteristically responds dramatically to salicylatetherapy. Carditis occurs in approximately 50% of

cases and includes myocarditis, pericardial effu-sions, arrhythmias, and valvular heart disease. Ery-

thema marginatum (Figure 4-1) occurs in less than10% of patients and is a nonpruritic serpiginous rash

that occurs on the torso and is almost never seen onthe face. The rash is evanescent and becomes more

apparent following hot baths or being wrapped inwarm blankets. Subcutaneous nodules are non-

tender, freely mobile nodules occurring usually overthe bony surfaces of the elbows, wrists, shins, knees,

ankles, and spine. They occur in 2-10% of cases.Chorea occurs in up to 15% of cases and is a neu-

ropsychiatric disorder that may include choreic

movements, hypotonia, emotional lability, anxiety,and an obsessive-compulsive disorder. Chorea usu-

ally occurs late, after the initial pharyngitis with theaverage time to onset of about 6-7 months. It may

last as long as 18 months. Recent evidence suggeststhat chorea is associated with the presence of anti-

neuronal antibodies. Minor manifestations includefever, arthralgias (when polyarthritis is not present),

increased acute phase reactants such as CRP, and aprolonged PR interval on ECG (in the absence of

other evidence of carditis). The diagnosis of acuterheumatic fever is made with either 2 major mani-

festations or with a single major manifestation and2 minor manifestations. If made by 1 major manifes-

tation and 2 minors, the diagnosis should be sup-ported by evidence of a preceding streptococcal

infection either by a positive throat culture or by

rising streptococcal antibody titers (eg, antistrep-tolysin O). There are 3 exceptions to the Jones crite-

ria for diagnosis of acute rheumatic fever:1. Chorea may be the only manifestation of rheu-

matic fever.

2. Indolent carditis may be the only manifestationin patients following the initial infection.

3. Recurrences often do not strictly fulfill the Jones

criteria. Therefore, a presumptive diagnosis ofrecurrent rheumatic fever may be made with

fewer than the usual number of criteria. Recur-rent disease should only be diagnosed if there is

supporting evidence of a recent streptococcalinfection.


17/24

CASE 4 A 5-YEAR-OLD CHILD WITH A HEART MURMUR AND FEVER 17

for at least 5 days, no other reasonable etiology, and4 of 5 of the following:1. A nonexudative conjunctivitis that is usually

bilateral2. Erythema of the lips, oral mucosa, and pharynx,

including a strawberry tongue and cracking orpeeling of the lips later into the disease

3. A polymorphous rash of the face, trunk, andextremities that later can involve the perinealarea and is characterized by desquamation at 5-7days (Figure 4-2)

4. Cervical adenopathy greater than 1.5 cm indiameter that is usually unilateral

5. Changes in the extremities, including edema anderythema of the hands and feet followed by periun-gual desquamation at 11-25 days into the disease

FIGURE 4-1. Erythema marginatum. (Reproduced, with per-

mission, from Fuster V, ORourke RA, Walsh RA, et al. Hursts

the Heart. 12th ed. New York: McGraw-Hill; 2008:1694.)

6. (C) Treatment for acute rheumatic fever includestherapy directed at the streptococcal infection with

penicillin followed by prevention of recurrenceswith either twice-daily oral penicillin or once-

monthly IM benzathine penicillin injections.Although some advocate prophylaxis to be contin-

ued at least until the patient is 21 years of age, othersrecommend that prophylaxis be lifelong. In patients

who are penicillin allergic, prophylaxis can be sub-stituted with either oral sulfadiazine or erythromy-

cin. Recurrences of acute rheumatic fever usuallyoccur within the first 5 years after the initial diagno-

sis and are characterized by more severe cardiacvalve involvement. It is estimated that approxi-

mately 10-25% of patients with heart valve involve-

ment will have complete resolution by 10 years.

7. (A) The mitral valve is the most commonly affectedfollowed by the aortic valve and, rarely, the tricuspidor pulmonary valves. Initially, the affected valvesdevelop regurgitation as a result of inflammationand valve dysfunction. However, with healing ofthe inflammation, long-term development of mitralvalve stenosis can occur. This may be seen as earlyas 2-3 years following the acute episode but usuallyoccurs 10-20 years later.

8. (A) The child described most likely has Kawasakidisease, an acute vasculitis of unknown etiology.

Kawasaki disease is the leading cause of acquiredheart disease in children in the United States. Theincidence ranges from 2-6/100,000 children and ishighest in Asian American children. The peak inci-dence is at 1-2 years of age with 85% of the casesoccurring in children younger than 5 years of age.The disease is uncommon in patients older than8 years of age or younger than 3 months of age. Theclinical manifestations include the presence of fever

FIGURE 4-2. Kawasaki disease. Desquamation of the tissue of

the palm. (Reproduced, with permission, from Wolff K, Johnson

RA. Fitzpatricks Color Atlas & Synopsis of Clinical Dermatol-

ogy, 6th ed. New York: McGraw-Hill; 2009: Fig. 14-45.)

Incomplete Kawasaki disease can be diagnosedwhen fever is present for 5 or longer days in the

presence of 2 to 3 of the preceding criteria when theCRP is 3.0 mg/dL or more and/or the ESR is 40

mm/hour or more. In this instance, certain labora-tory criteria should be met including hypoalbumine-

mia, anemia, increased serum alanine aminotrans-ferase, thrombocytosis, leukocytosis, and sterile

pyuria.


18/24


9. (D) Other supportive findings of the acute phase ofKawasaki disease include urethritis with sterile

pyuria, aseptic meningitis, abdominal pain, hydropsof the gallbladder, and arthritis that usually involves

the small joints but may involve the large joints

2-3 weeks into the disease. Also, mild carditis andarrhythmias may occur. The subacute phase occurs

11-25 days following the onset of fever and is char-acterized by a decrease in the rash and fever with

the onset of desquamation of the fingers and toes.Thrombocytosis peaks at 2-4 weeks into the course

of the disease. It is during this phase that coronaryartery aneurysms usually become evident.

10. (C) Coronary artery aneurysms are the most feared

complication of Kawasaki disease and occur in upto 15-25% of untreated patients (Figure 4-3). They

are a result of the panvasculitis resulting in aneurys-mal transformation of the coronary arteries and may

be seen as early as 7 days after the onset of thefever. Their incidence peaks at 3-4 weeks and they

are seldom found after 8 weeks into the course ofthe disease. Giant aneurysms are described as hav-

ing a diameter greater than 8 mm and are associatedwith increased mortality and morbidity. Patients

with aneurysms also have a higher incidence ofdeveloping stenoses leading to myocardial ischemia

and infarction with long-term follow-up. Coronary

artery rupture is the most common cause of mortalityin the subacute phase; myocarditis, heart failure,

and arrhythmias are the most common causes ofmortality in the acute phase within the first 10 days

after the onset of fever. Risk factors for the devel-opment of coronary artery aneurysms include male

gender, age younger than 1 year, hemoglobin lessthan 10 g/dL, white blood cell count greater than

30,000/mm3, ESR greater than 101 mm/hour, andprolonged fever. Echocardiography is recommended

for assessment of coronary artery involvement withKawasaki disease. The most recent recommenda-

tions suggest that an echocardiogram is obtained atdiagnosis, and if no coronary disease is seen, then it

should be repeated in 6-8 weeks. If coronary arteryinvolvement is documented, then follow-up should

be more frequent based on the extent of disease.

11. (A) The management of Kawasaki disease includeshigh-dose aspirin, which is given during the acute

phase, followed by low-dose aspirin for 6-8 weeks.This is given for antithrombotic effect while throm-

bocytosis is present. IVIG is administered withinthe first 10 days of the disease at a dose of 2 g/kg

and may be repeated if fever persists following thistherapy. The use of IVIG decreases the incidence of

coronary artery aneurysms to less than 5%. If

aneurysms persist past 2 months, then aspirin orother anticlotting agents should be continued. The

use of steroids is not indicated for uncomplicatedKawasaki disease.

12. (A) The most likely diagnosis in this situation is

infective endocarditis. This is an inflammatory dis-order of the heart resulting from an infection. The

signs and symptoms of infective endocarditisinclude acute findings of fever, anorexia, weight

FIGURE 4-3. Parasternal short axis images (echocardiograms)

of cronan artery aneurysms associated with Kawasaki disease.

(Reproduced, with permission, from Fuster V, ORourke RA,

Walsh RA, et al. Hursts the Heart, 12th ed. New York: McGraw-

Hill, Fig. 55-38.)

A

B


19/24


20/24


aureus is now, overall, the leading cause.Enterococ-

cus spp. is also a common cause of endocarditis, par-

ticularly in adults. Among patients with burns or IVdrug abusers, staphylococci are the most common

causative organisms. Infants with sepsis are also at

higher risk for endocarditis with no underlying cardiacdisease.

15. (A) Up to 10% of cases of endocarditis are culture

negative. However, in 90% of cases, the causativeagent may be identified by obtaining at least 3 blood

cultures during the first 24 hours of hospitalization.

16. (D) Valvular heart lesions are most commonly asso-ciated with predisposition for the development of

infective endocarditis; however, other forms of con-genital heart disease, including tetralogy of Fallot,

ventricular septal defect, patent ductus arteriosus,coarctation of the aorta, and pulmonary valve steno-

sis, have also been implicated. Congenital heartdefects such as atrial septal defect, peripheral pul-

monary stenosis, and mitral valve prolapse withoutmitral regurgitation are not considered to be high-

risk lesions for development of infective endocardi-tis, and thus bacterial endocarditis prophylaxis is

not indicated in these situations.

17. (A) In patients with suspected infective endocardi-tis, a course of either vancomycin or oxacillin and

gentamicin is started because the most likely organ-isms include streptococci and staphylococci,

depending on the rate of methicillin-resistant S.

aureus in the patients geographic area. Once theorganism is identified, the antibiotic regimen can be

adjusted. Surgical intervention is occasionallyrequired for removal of foreign bodies that are diffi-

cult to sterilize with IV antibiotics or repair of valvesthat have been damaged by infection. Other compli-

cations associated with endocarditis include mycoticaneurysms, localized cardiac abscesses, and autoim-

mune phenomena such as nephritis and arthritis.

18. (D) Procedures in which there is a risk for signifi-cant bacteremia would require bacterial endocardi-

tis prophylaxis. These procedures include dentalprocedures where bleeding is anticipated, tonsillec-

tomy, cardiac surgery, incision of infected sites,urologic surgery, and Foley placement in the pres-

ence of a urinary tract infection. Endotracheal intu-bation is not associated with a high incidence of

bacteremia and thus antibiotic prophylaxis is notrequired.

SUGGESTED READING







Keane JF, Fyler DC, Lock JE, eds.Nadas Pediatric Cardiology.


Newburger J, Takahashi M, Gerber M, et al. Diagnosis, treatment

and long-term management of Kawasaki disease. A statement

for health professionals from the Committee on Rheumatic

Fever, Endocarditis, and Kawasaki Disease, Council on Car-

diovascular Disease in the Young, American Heart Associa-

tion. Pediatrics. 2004;114:1708-1733.



CASE 5: A 10-YEAR-OLD BOY

WITH ELEVATED BLOOD

PRESSURE ASCERTAINED AT A

ROUTINE PHYSICAL

EXAMINATION

A 10-year-old boy presents for a preparticipation sports

physical examination. He has previously been well

with no significant past medical history. His vital signsindicate a heart rate of 101 bpm and a blood pressure of

130/85 mm Hg (greater than the 95th percentile for age).The remainder of his examination is normal.


1. What is the most appropriate next step?(A) recheck the blood pressure with a smaller

blood pressure cuff(B) recheck the blood pressure on at least 2 other

separate occasions before beginning further

evaluation(C) begin medical therapy with antihypertensive

medications

(D) order a renal ultrasound(E) check serum catecholamine levels


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CASE 5 A 10-YEAR-OLD BOY WITH ELEVATED BLOOD PRESSURE ASCERTAINED AT A ROUTINE PHYSICAL 21

2. At this age, what is the most common cause ofhypertension?

(A) primary familial or idiopathic hypertension(B) coarctation of the aorta

(C) renal artery thrombosis

(D) renal parenchymal disease(E) adrenal hyperplasia

3. Renal parenchymal hypertension is caused by all

except which of the following mechanisms?(A) salt retention

(B) water retention(C) increased renin levels

(D) excess levels of catecholamines(E) none of the above cause renal parenchymal

hypertension

4. On the third visit, the patient continues to demon-strate evidence of systemic hypertension with ele-

vated blood pressure recordings. Of the following,which is the most appropriate testing at this time?

(A) urinalysis(B) serum catecholamine levels.

(C) renal CT scan(D) urine 17-hydroxy steroids or 17-ketosteroids

(E) all of the above

5. Which of the following interventions would not berecommended at this time?

(A) stop all exercise(B) weight reduction

(C) stop tobacco use

(D) salt reduction(E) all of the above should be recommended

6. Beta-blockers are useful for treatment of hyperten-

sion but are contraindicated in all but which of thefollowing?

(A) patients with asthma(B) patients with diabetes

(C) hyperthyroidism(D) bradycardia

(E) severe bee sting allergy

7. The patient returns with a hypertensive crisis with asystolic blood pressure greater than 180 mm Hg and

a diastolic blood pressure greater than 110 mm Hgassociated with headache, vomiting, and pulmonary

edema. What is the most commonly used medica-tion in this setting?

(A) nitroprusside sodium(B) captopril

(C) phentolamine(D) Aldactone

(E) clonidine

8. A 10-year-old child presents for a sports physical

examination. The examination and blood pressure

are completely normal. There is a family history of

hypercholesterolemia in his mother. What is themost appropriate advice for this patient?

(A) total serum cholesterol should be evaluated(B) total serum cholesterol and lipoprotein analysis

should be evaluated(C) exercise stress test should be performed

(D) total serum cholesterol should be evaluated at21 years of age

(E) none of the above

9. At what serum cholesterol level would lipoproteinanalysis be indicated?

(A) 120 mg/100 mL(B) 140 mg/100 mL

(C) 180 mg/100 mL(D) 200 mg/100 mL

(E) 220 mg/100 mL

10. Which of the following is not a common cause ofsecondary hypercholesteremia in children?

(A) obesity(B) isotretinoin

(C) oral contraceptive pills

(D) hyperthyroidism(E) anorexia

11. What is the most common form of inherited

hyperlipidemia?(A) familial hypercholesterolemia

(B) familial combined hyperlipidemia(C) mild hypertriglyceridemia

(D) severe hypertriglyceridemia(E) none of the above

12. Which of the following is a true statement regarding

the treatment for hypercholesterolemia in children?(A) a Step 1 diet is recommended for children

younger than 2 years of age(B) bile acid sequestrants are recommended for

children older than 10 years of age when dietmodification alone is not effective

(C) lovastatin may be used in selected cases forchildren younger than 2 years of age

(D) none of the above(E) all of the above


22/24


13. A 10-year-old child with physical features of tallstature, a long thin face, scoliosis, pectus excava-

tum with a family history of sudden death at ayoung age (an uncle who died while playing basket-

ball) presents for a preparticipation sports physical.

What is the likely diagnosis in this patient?(A) Marfan syndrome(B) hypertrophic cardiomyopathy

(C) Turner syndrome

(D) Down syndrome(E) homocystinuria

14. What is the most common cardiac lesion associated

with this 10-year-olds diagnosis?(A) mitral valve prolapse

(B) aortic stenosis(C) coarctation of the aorta

(D) left ventricular outflow tract obstruction(E) atrial septal defect

15. An echocardiogram is ordered and demonstrates

mild aortic root dilation. What is the recommenda-tion for further management?

(A) participation in sports is unrestricted(B) follow-up echocardiogram in 10 years

(C) return to clinic only if symptoms of chest painoccur during activities

(D) treatment with beta-blockers may decrease therisk of further aortic root dilatation

(E) aortic root replacement

16. What findings during routine pre-participation

physical examination would place a child at risk forsudden death during sports?

(A) a harsh systolic murmur at the upper right ster-nal border

(B) family history of diabetes(C) respiratory sinus arrhythmia

(D) a single elevated blood pressure reading thatreturns to normal on subsequent visits

(E) arm span less than height

ANSWERS

1. (B) Hypertension is defined as a systolic or diastolic

blood pressure recording greater than the 95th per-centile for age and gender, recorded on 3 separate

occasions. Severe hypertension is defined as bloodpressure recordings greater than the 95th percentile

by 8-10 mm Hg. Accurate blood pressure record-ings are crucial for this diagnosis and should be

taken in a quiet, nonthreatening manner. The widthof the blood pressure cuff should be 40-50% or

more of the arm circumference. Smaller blood pres-

sure cuffs result in erroneously high blood pressurerecordings. Ideally, blood pressure recordings

should be taken in all 4 extremities. Anxiety leadingto transient elevations in blood pressure (white coat

hypertension) accounts for up to 40% of elevatedblood pressure recordings in children. Because the

diagnosis of hypertension should not be based on asingle reading, medical therapy and testing for sec-

ondary causes of hypertension are not appropriateduring this first visit.

2. (A) Hypertension is classified as either primary

(essential or idiopathic) or secondary. Primaryhypertension is the most common syndrome in

older patients such as this child, whereas secondaryhypertension is common in younger patients with

more severely elevated blood pressure recordings.Ninety percent of secondary causes are because of

renal parenchymal disease, renal artery disease, andcoarctation of the aorta.

3. (D) Renal parenchymal hypertension causes salt

and water retention and in some cases elevatedrenin levels leading to increased vascular resist-

ance. Increased serum catecholamine levels causinghypertension are seen with pheochromocytomas or

with congenital adrenal hyperplasia (11-hydroxy-lase deficiency or 17-hydroxylase deficiency).

Hypertension is also seen with use of certain drugsor medications and in cases of hypercalcemia.

4.(A)

The initial evaluation of hypertension is guidedby both the examination and the family history. The

important features of the history include a past med-ical history of urinary tract infections, cardiovascu-

lar surgeries, weakness or cramps, medication use,and tobacco use. Important features of the family

history include history of hypertension or prema-ture heart disease. The important features of the

physical examination include accurate blood pres-sure recordings in 4 extremities, assessment for

heart murmurs or bruits, assessment of peripheralpulses, assessment of renal tenderness, and a thor-

ough eye examination. The usual initial laboratoryevaluation includes a urinalysis, serum electrolytes

including blood urea nitrogen (BUN) and creatinine,and possibly a 12-lead ECG, chest radiograph, and

echocardiogram. A cholesterol level and a lipopro-tein analysis are indicated in select cases. If there is

severe hypertension and end-organ involvement or

hypertension refractory to therapy, then tests evalu-ating for secondary causes of hypertension can be

performed.


23/24

CASE 5 A 10-YEAR-OLD BOY WITH ELEVATED BLOOD PRESSURE ASCERTAINED AT A ROUTINE PHYSICAL 23

5. (A) Additional management in patients with hyper-tension includes nonpharmacologic interventions

such as exercise proscription, weight reduction,avoidance of tobacco or oral contraceptive pills,

and reduction of dietary salt intake. In severe hyper-

tension or persistent hypertension despite nonphar-macologic interventions, pharmacologic agents are

often used that include diuretics, beta-blockers, andvasodilators.

6. (C) Beta-blockers are often used in conjunction

with diuretics or in conditions where hyperthy-roidism results in hypertension. However, they are

contraindicated for use in patients with asthma (canprecipitate bronchospasm), diabetes (prevents man-

ifestation of symptoms of hyperglycemia), and inpatients with bradycardia.

7. (A) A hypertensive emergency requires immediate

reduction of blood pressure usually within minutesto hours. Hypertensive crises can be associated with

neurologic signs or congestive heart failure. Admin-istration of parental medications is important for the

acute treatment of hypertensive emergencies.These medications include diazoxide, nitroprusside

sodium, diuretics, IV nifedipine, hydralazine, orlabetalol. Phentolamine is usually reserved for

patients with pheochromocytomas.

8. (A) Hypercholesterolemia is a major risk factor forcoronary artery disease. Several long-term prospec-

tive studies have shown that lowering serum cho-

lesterol levels decreases the risk for coronary arterydisease in the future. This has prompted a more

aggressive approach to screening and therapy forhypercholesterolemia in young patients. The current

recommendations for serum cholesterol screeninginclude the child of a single parent with a cholesterol

level greater than 240 mg/100 mL or if the history isunobtainable but there is a suspicion of hypercho-

lesterolemia. The recommendations to perform aserum cholesterol level and lipoprotein analysis

include children with parents or grandparents with ahistory of coronary angioplasty or coronary artery

bypass surgery, men younger than 55 years of age,women younger than 65 years of age, and children

with parents or grandparents with a documentedmyocardial infarction among men younger than 55

years of age or women younger than 65 years ofage.

9. (D) Serum cholesterol levels can be measured in the

nonfasting state anytime after the age of 2 years. If

serum cholesterol levels are higher than 200 mg/dL,

lipoprotein analysis is indicated. Lipoprotein analy-

sis requires the patient to be fasting for 12 hoursbefore the testing.

LDL = (total serum cholesterol) (HDL)

(triglyceride concentration 0.2)

Low-density lipoprotein (LDL) levels


24/24


12. (B) In patients with hypercholesterolemia, therapy isnot indicated for children younger than 2 years of age.

In children older than 2 years of age, initial treatmentincludes the Step 1 diet recommended for approxi-

mately 3 months. If the serum cholesterol level

remains elevated, then a Step 2 diet is recommendedfor 6-12 months. If the level continues to be elevated

and the child is older than 10 years of age with an LDLhigher than 190 or an LDLhigher than 160 and a fam-

ily history of hypercholesterolemia, then bile acidsequestrants such as cholestyramine are the first-line

choice of medical therapy. In selected cases, lovastatinhas been reported to be of beneficial use. However, it

is currently not recommended for routine use.

13. (A) The patient in this scenario most likely carriesthe diagnosis of Marfan syndrome, an autosomal

dominant genetic disorder due to a mutation in thefibrillin gene on chromosome 15. This leads to

defective connective tissue disease. The clinicalfeatures include a long thin face, tall stature with the

arm span greater than the height, pectus excavatumor carinatum, scoliosis, lens subluxation, and high

arched palate (Figure 5-1). There is a family historyof Marfan syndrome in 70-85% of cases. Patients

with hypertrophic cardiomyopathy do not have thephysical stigmata described in this case, but it is an

important diagnosis in cases where there is a family

history of sudden unexpected death. Patients withTurner syndrome have physical stigmata consistent

with short stature and webbed neck; patients withDown syndrome also have short stature with char-

acteristic facial features.

14. (A) The most common heart lesions occurring in

patients with Marfan syndrome include mitral valveprolapse and dilatation of the aortic root due to

abnormalities in the connective tissue as a result ofa mutation in the fibrillin gene. Aortic stenosis

because of a bicuspid aortic valve and coarctationof the aorta are commonly seen in patients with

Turner syndrome, and left ventricular outflow tractobstruction may be seen in patients with severe

forms of hypertrophic cardiomyopathy. The typicalexamination feature in a patient with mitral valve

prolapse includes a systolic ejection click thatvaries in timing when the patient is standing versus

when he is squatting. A diastolic murmur of mitralregurgitation may be heard in conjunction with

more severe cases of mitral valve prolapse.

15. (D) In patients with Marfan syndrome, participationin activities is limited to mildly aerobic low-impact

sports. This is to avoid precipitation of further aor-tic root dilation or rupture and to avoid retinal

detachment. It is recommended that patients withMarfan syndrome undergo routine echocardio-

graphic evaluation to assess aortic root dilation aswell as routine ophthalmologic examinations. Beta-

blockers are effective to decrease the progression ofaortic root dilation.

16. (A) The American Heart Association recommends

that a preparticipation physical examination screen-ing include obtaining a family history for sudden

cardiac death and a review of systems looking for a

history of dizziness or syncope. Physical findings ofMarfan syndrome, hypertension, decreased periph-

eral pulses, or a pathologic murmur such as theharsh systolic murmur described would indicate the

need for further evaluation. The finding of a familyhistory of diabetes, respiratory, sinus arrhythmia, or

a single elevated blood pressure returning to normalat subsequent visits would not place this patient at

risk for sudden death during sports.

SUGGESTED READING

Allen HD, Driscoll DJ, Shaddy RE, Feltes TF, eds. Moss andAdams Heart Disease in Infants, Children and Adolescents.





Keane JF, Fyler DC, Lock JE, eds.Nadas Pediatric Cardiology.




FIGURE 5-1. Pectus excavatum. (Reproduced, with permis-

sion, from Doherty G. Current Diagnosis & Treatment: Surgery,

13th ed. New York: McGraw-Hill; 2010: Fig. 43-4.)

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