Cardiovascular Disorders

Question 1

The most common congenital heart defect in children is:

a) tricuspid atresia
b) ventricular septal defect
c) aortic stenosis
d) pulmonary atresia

Answer 1

b) ventricular septal defect

Question 2

The mother of a 4 mo infant reports that he turned blue and seemed to have fast labored breathing after vigorous crying soon after awakening. He “fell asleep” and his color and breathing seemed to improve. On physical exam the lips and mouth appear mildly cyanotic. A systolic murmur is heard best at the left sternal border. VS are normal with normal peripheral pulses. There is no hepatomegaly. A likely diagnosis is:

a) congestive heart failure
b) apnea
c) coactation of the aorta
d) cyanotic spell related to tetralogy of fallot

Answer 2

d) cyanotic spell related to tetralogy of fallot

Question 3

Management of the infant with suspected heart disease and reported cyanotic spell should include:

a) prompt referral to a cardiologist
b) an apnea monitor
c) instructing the parent to keep a diary of the episodes
d) continuous administration of oxygen

Answer 3

a) prompt referral to a cardiologist

Question 4

Chest pain in young children is usually:

a) a symptom of congenital heart disease
b) noncardiac in origin
c) a sign of hypercholestermia
d) a symptom of congestive heart failure

Answer 4

b) noncardiac in origin

Question 5

A common cause of congestive heart failure in the first year of life is:

a) pulmonary stenosis
b) ventricular septal defect
c) rheumatic fever
d) complete heart block

Answer 5

b) ventricular septal defect

Question 6

The least likely physical finding in a 2 month old with congestive heart failure is:

a) tachypnea
b) tachycardia
c) hepatomegaly
d) pedal edema

Answer 6

d) pedal edema

Both tachypnea and tachycardia are initial, general signs of congestive heart failure. Hepatomegaly is a sign of right-sided failure and is found early in children. Pedal edema is less common in children than adults. There must be a 10% increase in body weight for edema to occur, and typically the face and eyelids are noted first.

Question 7

A vibratory systolic murmur is heard between the lower left sternal and the apex in a healthy 4 yo at her preschool physical. The cardiovascular exam is otherwise normal. A likely diagnosis is:

a) venous hum
b) still’s murmur
c) transposition of the great arteries
d) rheumatic heart disease

Answer 7

b) still’s murmur

Question 8

Characteristics of a venous hum include:

a) a systolic murmur
b) radiation over precordium
c) marked decrease or disappearance of murmur when child is supine
d) heard best at lower left sternal border

Answer 8

c) marked decrease or disappearance of murmur when child is supine

Question 9

Which of the following is true regarding innocent murmurs?

a) the murmur is often holosystolic
b) prompt referral to a cardiologist is indicated
c) a precordial thrill is present
d) the murmur is low intensity grade 1-3

Answer 9

d) the murmur is low intensity grade 1-3

Question 10

SBE prophylaxis is recommended for:

a) all children with congenital heart disease on a daily basis
b) all children with congenital heart disease before dental, GI, and GU procedures
c) children with repaired congenital heart disease with a residual defect at the repair site
d) five years after repair of all congenital heart defects

Answer 10

c) children with repaired congenital heart disease with a residual defect at the repair site

Based on current guidelines SBE prophylaxis is only recommended for children for 6 months after surgical repair and continued if they have residual defect, which would prohibit endothelialization of the surrounding tissue.

Question 11

A 12 yo girl seen at a routine visit has a blood pressure of 140/90. She denies any symptoms. The initial management would include:

a) intravenous pyelogram
b) return for two repeat blood pressure measurements
c) no follow up needed–blood pressure probably related to anxiety
d) diuretic therapy

Answer 11

b) return for two repeat blood pressure measurements

Question 12

A 9 yo boy presents with a fever of 102 and complaints of leg pains. His mother reports that he had an upper respiratory infection with a sore throat approximately 2 weeks ago, which subsided without therapy. On physical exam he has tender, swollen knees bilaterally. His heart rate is 120 and a blowing systolic murmur is heard at the apex. No murmur was noted at a previous well child visit. The most likely diagnosis is:

a) Kawasaki disease
b) rheumatic fever
c) sickle cell anemia
d) viral illness

Answer 12

b) rheumatic fever

symptoms such as new onset murmur associated with the mitral valve and polyarthritis of the joints particularly the knees, ankles, and elbows are two of the major criteria necessary for diagnosing rheumatic fever. Fever and arthralgia are minor criteria.

Question 13

The most useful test for evaluation of suspected acute rheumatic fever is:

a) antistreptolysin-O titer
b) electrocardiogram
c) hemoglobin electrophoresis
d) urinalysis

Answer 13

a) antistreptolysin-O titer

Question 14

The initial attack of acute rheumatic fever is preceded by:

a) a viral illness
b) a group A streptococcal infection
c) exposure to mites
d) exposure to chicken pox

Answer 14

b) a group A streptococcal infection

Question 15

A 3 wo infant has a one day history of irritability, pallor, and poor feeding. He is afebrile. On physical exam his heart rate is 240 while asleep. The most likely diagnosis is:

a) supraventricular tachycardia
b) premature ventricular contractions
c) sinus tachycardia
d) cyanotic heart defect

Answer 15

a) supraventricular tachycardia

Question 16

Congenital complete heart block may be associated with:

a) maternal lupus erythematosus
b) wolff-parkinson-white syndrome
c) maternal myocardial infarction
d) kawasaki disease

Answer 16

a) maternal lupus erythematosus

Question 17

The most common cause of myocarditis is:

a) bacterial
b) viral
c) drug reaction
d) radiation therapy

Answer 17

b) viral

While all of these can potentially cause an inflammatory process of the heart, which may progress to myocarditis, the most common cause is typically viral, with adenovirus, coxsackie B, cytomegalovirus, parvovitus B19, influenza, or respiratory syncytial virus being most frequently identified.

Question 18

Which of the following is not an expected finding in a child with myocarditis?

a) persistent tachycardia
b) history of antecedent “flu-like” illness
c) a gallop rhythm
d) a significant heart murmur

Answer 18

d) a significant heart murmur

Murmurs are soft, less common in myocarditis, and only noted if tricuspid or mitral insufficiency is present. The tachycardia is secondary to the inflammation and neurohormonal responses, and a preceding viral illness is very common. Often a gallop rhythm is noted because of rapid filling of a noncompliant, poorly contractile left ventricle.

Question 19

Hypercholesterolemia in children over 2 years old is defined as a total cholesterol at or above:

a) 100
b) 130
c) 160
d) 200

Answer 19

d) 200

Question 20

A potential childhood risk factor for development of atherosclerotic or coronary heart disease as adults is:

a) obesity
b) tachycardia
c) heart murmur
d) aerobic exercise

Answer 20

a) obesity

Question 21

Which of the following is not likely to cause secondary hypercholesterolemia?

a) nephrotic syndrome
b) hypertension
c) corticosteroids
d) obstructive liver disease

Answer 21

b) hypertension

Question 22

which of the following is a common cause of acquired coronary artery disease during childhood?

a) rheumatic fever
b) hypertension
c) systemic lupus erythematosus
d) kawasaki disease

Answer 22

d) kawasaki disease

Question 23

Kawasaki disease is most common in:

a) neonates
b) children less than 5 years of age
c) children over 6 years of age
d) females

Answer 23

b) children less than 5 years of age

Question 24

A principal clinical feature of Kawasaki disease includes:

a) low grade fever for 24 hours and a puritic rash
b) conjunctivitis with exudate and facial rash
c) arthritis and chorea
d) fever persisting at least 5 days and acute erythema and/or edema of hands and feet

Answer 24

d) fever persisting at least 5 days and acute erythema and/or edema of hands and feet

Question 25

An essential test in the evaluation of a 2 yo being managed for Kawasaki disease is:

a) an echocardiogram
b) electrolytes
c) cholesterol
d) streptococcal antibody titer

Answer 25

a) an echocardiogram

Question 26

A common cause of congestive heart failure in the first year of life is:

a) mild pulmonary stenosis
b) inflammatory heart disease
c) rheumatic fever
d) complete heart block

Answer 26

d) complete heart block

Complete heart block in a young child will lead to a heart rate less than acceptable, which will progress to heart failure as the heart cannot meet the body’s metabolic demand.

Question 27

Which of the following may cause volume overload leading to congestive heart failure if not appropriately followed and managed?

a) coarctation of the aorta
b) pulmonary stenosis
c) ventricular septal defect
d) supraventricular tachycardia

Answer 27

c) ventricular septal defect

A VSD with its left-to-right shunting will increase pulmonary blood flow and cause volume overload if large enough.

Question 28

A 4 yo caucasian female presents for a well child check. Her blood pressure by auscultation is 135/80. There is no significant family history of hypertension or heart disease. Besides wanting to repeat the blood pressures for accuracy, what are the potential differential diagnoses?

a) severe coronary artery disease
b) renal artery stenosis and coarctation of the aorta
c) rheumatic fever and ventricular septal defect
d) tetralogy of fallot and coarctation of the aorta

Answer 28

b) renal artery stenosis and coarctation of the aorta

Coarctation of the aorta would present with hypertension in the right upper extremity. Kidney diseases such as renal artery stenosis, congenital renal abnormalities, and renal parenchymal disease are a few of the most common causes of secondary hypertension in children younger than 10 years old.

Question 29

A 10 mo infant presents with a 3 day history of fever, runny nose, and coughing. Per the parent he has not been eating well, and only drinking a few cups of milk and juice over the last 2 days. On examination, he feels very warm, his RR is 45 and his HR is 210. He has no rashes. His abdomen is soft and round. Your initial diagnosis includes:

a) supraventricular tachycardia based on HR; needs immediate transfer
b) congestive heart failure based on HR and RR; needs immediate transfer
c) sinus tachycardia based on clinical history and HR; needs antipyretics and possibly fluids.
c) kawasaki disease based on clinical history and fever; needs transfer for IVIG

Answer 29

c) sinus tachycardia based on clinical history and HR; needs antipyretics and possibly fluids.

Question 30

Which of the following is not a component of metabolic syndrome, a clustering of factors in children shown to be associated with increased risk of coronary artery disease later in life?

a) obesity
b) elevated lipid levels
c) heart murmur
d) elevated fasting glucose level

Answer 30

c) heart murmur

Question 31

Initial management of a 4 yo overweight boy with a repeat total cholesterol level of 185 includes all the following except:

a) educate the family about decreasing saturated fat intake to less than 7% of calories and cholesterol intake to less than 200mg per day
b) blood sent for lipoprotein analysis
c) educate the family about decreasing/ eliminating daily intake of fruit juices, sugar sweetened food/ beverages, and salt intake
d) educate the family about increasing aerobic activities

Answer 31

a) educate the family about decreasing saturated fat intake to less than 7% of calories and cholesterol intake to less than 200mg per day

A repeat total fasting cholesterol level of 185 is considered borderline. Per AAP, the American Heart Association strict dietary changes such as dropping fat and cholesterol intake are not recommended at this time. Encouraging a heart-healthy diet and aerobic activities as well as simple dietary changes that decrease refined sugars, high fat, and salt intake are acceptable for children of any age. The lipoprotein profile will provide further guidance by documenting HDL and LDL values for future monitoring.

Question 32

The 4 defects present in tetralogy of fallot:

Answer 32

1) pulmonary stenosis
2) ventricular septal defect
3) overriding aorta
4) right ventricular hypertrophy
* cyanotic

Question 33

What congenital heart defect is acyanotic and causes narrowing of the aorta?

Answer 33

Coarctation of the aorta

Question 34

What congenital heart defect is cyanotic and causes the aorta to arise from the right ventricle, the pulmonary artery to arise from the left ventricle, and a patent ductus arteriosus?

Answer 34

Transposition of the great arteries

Question 35

What congenital heart defect causes the pulmonary veins to connect either directly or indirectly to the right atrium instead of the left atrium?

Answer 35

Total anomalous pulmonary venous connection

Question 36

What congenital heart defect causes a single arterial trunk (that did not divide into pulmonary artery and aorta in utero) that provides blood flow to both the pulmonary and systemic circulation? Blood bypasses the ventricles via a ventricular septal defect:

Answer 36

Truncus arteriosus

Question 37

What congenital heart defect causes a low atrial septal defect, a high ventricular septal defect, and abnormal mitral and tricuspid valves?

Answer 37

Atrioventricular canal (AV canal)

Question 38

Characteristics of innocent murmurs (5):

Answer 38

1) systolic
2) low intensity with “twanging” quality
3) not holosystolic; never associated with thrill
4) well localized (except neonatal peripheral pulmonary stenosis)
5) intensity varies with position change

Question 39

Hypertension definition:

Answer 39

Average SBP or DBP greater than or equal to the 95th pecentile on at least 3 occasions.

Question 40

Hypercholesterolemia definition:

Answer 40

Total cholesterol > 200 (170-199 is borderline)
Low density lipoprotein 130 or higher

Test children > 2 years of age with positive family history or other risk factors.

Question 41

When is drug therapy for hypercholesterolemina started?

Answer 41

At age 10 if completed an adequate trial of diet therapy (6 months to a year) and LDL > 190 OR LDL > 160 PLUS a positive family history of premature cardiovascular disease OR 2 other cardiovascular risk factors present in the child (obesity, HTN, diabetes).

Question 42

Antibiotic prophylaxis in order to prevent endocarditis is recommended if (6):

Answer 42

1) prosthetic cardiac valve
2) previous endocarditis
3) unrepaired congenital heart disease
4) repaired congenital heart disease with prosthetic material for the first 6 months post-op
5) repaired congenital disease with residual defects at or adjacent to the site of a prosthetic device or patch
6) following cardiac transplant