Pediatric Cardiology

Question 1

Which congenital heart defect causes severe cyanosis in the first days of life?
A) aortico-pulmonary fenestration
B) postductal coarctation of the aorta
C) atrioventricular septal defect
D) transposition of the great arteries
E) persistent ductus arteriosus

Answer 1

D) transposition of the great arteries
EXPLANATION
In transposition of the great arteries there are two separate circulations, the aorta arises from the right ventricle and receives deoxygenated blood from the body. Mixing is only possible via the fetal connections. Urgent balloon atrioseptostomy may be indicated. In A, B, C and E the shunt is from left to right, thus no cyanosis would occur.

Question 2

A cyanotic newborn has a chest x-ray, which shows decreased vascularisation of the lungs. Which of these congenital heart defects is the most likely diagnosis?
A) Transposition of the great arteries
B) Total anomalous pulmonary venous return
C) Pulmonary atresia

Answer 2

C) Pulmonary atresia
EXPLANATION
Vascularization of the lungs can only be judged on a good quality chest x-ray in newborns. In pulmonary atresia the vascularization of the lungs is decreased, pulmonary circulation is duct-dependent. The heart is usually not enlarged. In A and B, the configuration of the heart can be characteristic and vascularization is increased.

Question 3

One of these statements does not apply to an isolated ventricular septal defect in the newborn.
A) the left-to-right shunt increases with time
B) can lead to decompensation
C) there could be signs of pulmonary congestion
D) it always causes cyanosis, which disappears later

Answer 3

D) it always causes cyanosis, which disappears later
EXPLANATION
There is no cyanosis in an isolated ventricular septal defect in the newborn. Due to the higher pulmonary vascular resistance, the left-to-right shunt is not pronounced in the early phase, but will increase with time. Typical signs and symptoms would be present by 2-3 months of age. If the defect is big, decompensation can develop with signs of pulmonary congestion.

Question 4

A newborn showing signs of congestive heart failure has easily palpable upper extremity pulses, but the pulse can not be felt on the lower extremities. What is the most likely diagnosis?
A) hypoplastic left heart syndrome
B) severe, „critical” valvular aortic stenosis
C) coarctation of the aorta

Answer 4

C) coarctation of the aorta
EXPLANATION
Severe coarctation of the aorta can lead to circulatory decompensation in the newborn. If the ductus arteriosus is not widely open, the lower extremity pulse cannot be palpated. There is severe decompensation in hypoplastic left heart syndrome, but usually there is not a great difference in the pulses (they are usually weak everywhere). In severe (critical ) valvular aortic stenosis, the pulses are weak both in the upper and the lower part of the body.

Question 5

Which of the following maternal diseases increase the risk of congenital heart defect in the fetus?
1) alcoholism
2) phenylketonuria
3) Type 1-diabetes mellitus
4) hyperthyreoidismA) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

Answer 5

A) Answers 1, 2 and 3 are correct
EXPLANATION
There is an increased risk of congenital heart defect in the fetus if the mother drinks alcohol during the pregnancy (fetal alcohol syndrome can develop), or has certain metabolic diseases (e.g phenylketonuria, insulin dependent diabetes mellitus). Maternal hyperthyreosis can have an impact on the fetal circulation, but would not cause a congenital heart defect.

Question 6

Which of the following can be a sign of a congenital heart defect?
1) heart murmur
2) pulse difference
3) central cyanosis
4) heart failureA) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

Answer 6

E) All of the answers are correct
EXPLANATION
All these four can be manifestations of a congenital heart defect- in combination or alone. The congenital heart defect itself will determine which symptom is characteristic of the given disorder.

Question 7

Cardiac cause(s) of chest pain:
1) coronary artery malformation
2) tachy-arrhythmia
3) pericarditis/myocarditis
4) cardiac decompensation
A) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

Answer 7

A) Answers 1, 2 and 3 are correct
EXPLANATION
Malformations of the coronary arteries can lead to ischemia, which causes chest pain. In tachyarrhythmias, the diastolic filling of the heart is compromised, which causes shortness of breath and an uncomfortable feeling (dyscomfort). In peri/myocarditis it is the inflammation of the pericardium or the accumulation of the pericardial fluid which leads to pain. Decompensation in itself does not cause chest pain.

Question 8

Which of the following can be a sign/symptom of tetralogy of Fallot?
1) cardiac decompensation
2) cyanosis
3) tachypnea
4) polycythemia
A) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

Answer 8

C) Answers 2 and 4 are correct
EXPLANATION
Tetralogy of Fallot belongs to the cyanotic group of congenital heart defects, cyanosis and hypoxia increase the red blood cell production and can cause polycythemia. Decompensation is uncommon in Fallot. Bounding pulses are characteristic of the patent ductus arteriosus.

Question 9

In which of the following heart defects is it necessary to keep the ductus arteriosus open?
1) transposition of the great arteries
2) pulmonary atresia
3) critical coarctation of the aorta
4) common arterial trunk
A) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

Answer 9

A) Answers 1, 2 and 3 are correct
EXPLANATION
Keeping the ductus arteriosus open can be life-saving in the following situations: enhancing the mixture of the blood between the two otherwise separate circulations in transposition of the great arteries; or if the normal anterograde flow to the pulmonary (e.g pulmonary atresia) or the systemic circulation (e.g critical coarctation of the aorta) is not possible and the blood supply must come from the other circulation through the duct.

Question 10

Cardiac examinations which would cause radiation exposure:
1) CT-angiography
2) Cardiac MRI
3) cardiac catheterization
4) echocardiography
A) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

Answer 10

B) Answers 1 and 3 are correct
EXPLANATION
CT-angiography and angio-catheterization are examinations with x-ray-load (the latter needs contrast material in general). Cardiac MRI and ultrasound (echocardiography) do not involve exposure to irradiation.

Question 11

Synchronized cardiversion can effectively stop the following rhythm disturbancy (-ies):1) atrial ectopic tachycardia
2) atrial flutter
3) pulseless Torsades de pointes tachycardia
4) atrioventricular reentry tachycardia (AVRT)
A) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

Answer 11

C) Answers 2 and 4 are correct
EXPLANATION
Synchronized cardioversion can be effective in arrhythmias due to a reentry circuit, such as atrial flutter and atrioventricular reentry tachycardia (AVRT). If the arrhythmia is due to an ectopic focus, cardioversion is ineffective (atrial ectopic tachycardia or ventricular extrasystole, which in most cases originate from an ectopic focus).

Question 12

A bolus of adenosin can stop the following rhythm disturbancy(-ies):
1) Atrial flutter
2) Atrial fibrillation
3) Ventricular tachycardia
4) Atrioventricular reentry tachycardia
A) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

Answer 12

D) Only answer 4 is correct
EXPLANATION
Adenosine is effective in those rythm disturbancies, where the AV node is involved in the mechanism. Adenosine temporarily blocks the conduction of the AV node. Only atrioventricular reentry tachycardia fulfills this criteria of this list.

Question 13

Cardiac decompensation can cause the following in children:
1) loss of apetite
2) tachypnea
3) hepatomegaly
4) edema of the ankle
A) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

Answer 13

A) Answers 1, 2 and 3 are correct
EXPLANATION
In children common symptoms of heart failure include the following: loss of apetite, tachypnea (congestion of the lungs), hepatomegaly (congestion of the liver). Ankle or pretibial edema are unlikely in children (but are common in adults!)

Question 14

Characteristic of the AV reentry tachycardia:
1) The pulse gradually increases at the beginning of the episode and gradually decreases at the end
2) during the tachycardia the R-R distance is variable
3) never occurs under 1 year of age
4) adenosine can stop it
A) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

Answer 14

D) Only answer 4 is correct
EXPLANATION
In atrioventricular reentry tachycardia there is a sudden onset and sudden cessation of the tachycardia, the R-R distance is constant (regular tachycardia). The peak age is infancy, when an accessory pathway is likely to be present. Thus the first three answers are incorrect. The AV reentry tachycardia can be terminated with adenosine, because the AV node is a part of the pathologic circuit.

Question 15

1) Big subaortic ventricular septal defect
2) Aorta overriding the ventricular septal defect
3) Right ventricular outflow tract obstruction
4) Right ventricular hypertrophy
A) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

Answer 15

E) All of the answers are correct
EXPLANATION
Tetralogy of Fallot (as the name implies) consists of 4 characteristic disorders of the heart. These are the four listed in the test. The common origin is a malformation of the infundibulum of the right ventricular outflow tract.

Question 16

Characteristic of the hypoxic (also called: cyanotic) spell of the Fallot-patient:
1) It is caused by an increased right-to –left shunt through the ventricular septal defect, which results in more deoxygenated blood entering the systemic circulation.
2) It is contraindicated to give oxygen during the spell.
3) Calming the baby and putting him/her in a chest-knee position help to cease the spell.
4) Fortunately the spell never leads to a serious condition.
A) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

Answer 16

B) Answers 1 and 3 are correct
EXPLANATION
In some Fallot patients cyanotic spells (hypoxic spells) occur. They are always potentially life-threatening, thus have to be taken seriously. The spell typically starts with crying and anxiety, the right-to-left shunt increases, so more desaturated blood enters the aorta, worsening the cyanosis. It is essential to calm the patient and give them oxygen during the spell. A knee-to-chest position („folding” the patient) can be beneficial by elevating the systemic resistance and decreasing the right-to-left shunt

Question 17

The risk of infectious endocarditis increases in the following conditions:
1) Uncorrected cyanotic heart defect
2) Previous infectious endocarditis
3) Artificial heart valve
4) Uncorrected ventricular septal defect
A) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

Answer 17

A) Answers 1, 2 and 3 are correct
EXPLANATION
The list of the defects requiring endocarditis prophylaxes got much shorter in the last two decades. Most of the classic, non-cyanotic malformations (such as VSD) are no longer on the list. Endocarditis profilaxis is still essential if the patient has an uncorrected cyanotic heart defect; a previous endocarditis in the history; or an artificial valve.

Question 18

In endocarditis profilaxis…
1) the patient with a congenital heart defect needs antibiotics if he has fever.
2) the type of the heart defect is irrelevant.
3) is obligatory in endoscopic procedures.
4) a single dose of antibiotics is needed one hour prior to the procedure.
A) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

Answer 18

D) Only answer 4 is correct
EXPLANATION
Endocarditis profilaxis means that with certain heart defects the patient must be given a single dose of antibiotics one hour prior to some specific interventions (some oral procedures). Otherwise a patient with a congenital heart defect requires the same consideration regarding the use of antibiotics than a healthy individual.

Question 19

Which of the following heart auscultatory points are the punctum maximum of the listed murmurs or heart defects?
A) left parasternal 3rd-4th intercostal space
B) left parasternal 2nd intercostal space
C) between the left scapula and the spine on the back
D) right parasternal 2nd intercostal space
E) infraclavicular areaPED - 6.19 - Still’s murmur (vibratory murmur)
PED - 6.20 - Venous hum
PED - 6.21 - Valvular pulmonary stenosis
PED - 6.22 - Atrial septal defect
PED - 6.23 - Valvular aortic stenosis
PED - 6.24 - Ventricular septal defect
PED - 6.25 - Pulmonary flow murmur
PED - 6.26 - Coarctation of the aorta at the isthmus

Answer 19

PED - 6.19 - Still’s murmur (vibratory murmur) - A)
Still’s murmur (or vibratory murmur) is the most common innocent murmur in the pediatric population. The point of maximum is at the left lower parasternal area (3rd and 4th intercostal space), and the murmur often has a musical characteristic.
PED - 6.20 - Venous hum - E)
Venous hum is a type of innocent murmurs, it can be best heard in the infraclavicular region. It is a systolo-diastolic murmur, of which the intensity changes as the head turns.
PED - 6.21 - Valvular pulmonary stenosis - B)
Murmur of the valvular pulmonary stenosis has its punctum maximum in the second left parasternal intercostal space, the pulmonary area.
PED - 6.22 - Atrial septal defect - B)
Murmur of the atrial septal defect is heard in the pulmonary area, in the 2nd left parasternal intercostal space. The murmur is caused by a relative pulmonary stenosis, as more blood passes through a normal sized pulmonary valve.
PED - 6.23 - Valvular aortic stenosis - D)
The murmur of the valvular aortic stenosis has its punctum maximum at the right second parasternal intercostal space.
PED - 6.24 - Ventricular septal defect - A)
The murmur of a ventricular septal defect originates from the turbulent bloodflow through the defect from the high pressure left ventricle towards the low pressure pulmonary circulation. The murmur is best heard above the septal defect, so at the 3rd-4th left parasternal intercostal space.
PED - 6.25 - Pulmonary flow murmur - B)
Pulmonary ejection murmur/pulmonary flow murmur is an innocent murmur of childhood, originating from the bifurcation or the pulmonary branches, due to a relative stenosis which is physiologic in the first few months of life.
PED - 6.26 - Coarctation of the aorta at the isthmus - C)
The most typical type of coarctation of the aorta is a narrowing located at the isthmus of the vessel. This area is already closer to the back than to the anterior chest, so the murmur of the coarctation can be better heard with the patient sitting and listening to the area between the spine and the left scapula.

Question 20

Pair the different types of cardiomyopathies with their characteristics:
A) Hypertophic cardiomyopathy
B) Dilated cardiomyopathy
C) Restrictive cardiomyopathy
PED - 6.27 - Dominant inheritance with varied penetrance and expressivity
PED - 6.28 - The most common cause of death in young sportsmen
PED - 6.29 - It can be caused by a cardiothoxic chemotherapy
PED - 6.30 - The ventricles have normal sizes, the atria are markedly enlarged
PED - 6.31 - The most common form requiring heart transplant
PED - 6.32 - The thickened ventricular septum can impede the outflow from the left ventricle
PED - 6.33 - The systolic function of the left ventricle is reduced

Answer 20

PED - 6.27 - Dominant inheritance with varied penetrance and expressivity - A)
There is a genetic background in hypertrophic cardiomyopathy, this explains why it is important to screen the symptom-free family members. There can be a genetic cause in the pathogenesis of a dilated cardiomyopathy as well, but it is much less common.
PED - 6.28 - The most common cause of death in young sportsmen - A)
Hypertrophic cardiomyopathy is the most common cause of sudden death of young sportsmen. Rythm disturbance is the most common direct cause.
PED - 6.29 - It can be caused by a cardiothoxic chemotherapy - B)
Cardiothoxic chemotherapeutic agents (e.g anthracyclines) can lead to dilated cardiomyopathy, the risk is directly proportional to its cumulative dose.
PED - 6.30 - The ventricles have normal sizes, the atria are markedly enlarged - C)
In restrictive cardiomyopathy the ventricles are stiff, thus the diastolic function is impeded (the systolic function is preserved). Poor ventricular compliance leads to incompetence of the AV valves and the atria become remarkably dilated.
PED - 6.31 - The most common form requiring heart transplant - B)
Dilated cardiomyopathy is the most common type of cardiomyopathies. It can progress to severe decompensation, which necessitates heart transplantation.
PED - 6.32 - The thickened ventricular septum can impede the outflow from the left ventricle - A)
Thickening of the myocardium is a typical feature of the hypertrophic cardiomyopathy. Thickening can be diffuse or can be asymmetric, affecting e.g only a portion of the interventricular septum. If the subaortic part of the septum is very thick it can obstruct the outflow from the left ventricle (referred to as „hypertrophic cardiomyopathy with obstruction”).
PED - 6.33 - The systolic function of the left ventricle is reduced - B)
The systolic function of the left ventricle is decreased in dilated cardiomyopathy, but is typically preserved in the two other types.

Question 21

What is the most likely diagnosis?
A 2-month-old infant is taken to the emergency room with the following complaint: he seemed to be less active the day before and has taken less mothermilk. Today he is remarkebly sleepy, a little tachypneic and has only accepted little mothermilk. On physical examination his skin is a little colder and looks paler. Breath rate: 60/min, pulse: 290/min, liver is 2 cm below the costal margin.
A) Bacterial sepsis
B) Paroxismal supraventricular tachycardia
C) Ventricular tachycardia
D) Sinus tachycardia caused by thyrotoxicosis

Answer 21

B) Paroxismal supraventricular tachycardia
EXPLANATION
The upper limit of normal heart rate is 160/min in a two-month-old infant. Sinus tachycardia is not likely above 200/min. A heart rate of 290/min is obviously abnormal and at this age the most common cause is paroxismal supraventricular tachycardia (AV reentry tachycardia). Atrial flutter is rare at this age, being more common in the first few days of life. Ventricular tachycardia is also rare in infants without a congenital heart defect and the heart rate is usually not that elevated. Ventricular tachycardia is usually benign at this age and resolves spontaneously.

Question 22

An ECG is done. What is the most likely finding on the ECG?
A 2-month-old infant is taken to the emergency room with the following complaint: he seemed to be less active the day before and has taken less mothermilk. Today he is remarkebly sleepy, a little tachypneic and has only accepted little mothermilk. On physical examination his skin is a little colder and looks paler. Breath rate: 60/min, pulse: 290/min, liver is 2 cm below the costal margin.
A) F-waves with 2:1 conduction block to the ventricles
B) Wide QRS tachycardia
C) Reguler, narrow QRS tachycardia, p-waves can not be identified
D) Irregular narrow QRS tachycardia

Answer 22

C) Reguler, narrow QRS tachycardia, p-waves can not be identified
EXPLANATION
Typical ECG of the AV reentry tachycardia is a regular, narrow QRS tachycardia, where the p-waves are either not identified or appear after the QRS complex.

Question 23

What do the other signs on the physical examination refer to?
A 2-month-old infant is taken to the emergency room with the following complaint: he seemed to be less active the day before and has taken less mothermilk. Today he is remarkebly sleepy, a little tachypneic and has only accepted little mothermilk. On physical examination his skin is a little colder and looks paler. Breath rate: 60/min, pulse: 290/min, liver is 2 cm below the costal margin.
A) Cardiac decompensation
B) Dehydration (hypovolemia)
C) Infection
D) Patent arterial duct

Answer 23

A) Cardiac decompensation
EXPLANATION
When the physical examination reveals tachypnea and hepatomegaly, they indicate a longer-lasting tachycardia leading to cardiac decompensation. A one- or two-day-long tachycardia can already cause left ventricular dysfunction and as a consequence signs of decompensation.

Question 24

Which of the following drugs can stop the attack?
A 2-month-old infant is taken to the emergency room with the following complaint: he seemed to be less active the day before and has taken less mothermilk. Today he is remarkebly sleepy, a little tachypneic and has only accepted little mothermilk. On physical examination his skin is a little colder and looks paler. Breath rate: 60/min, pulse: 290/min, liver is 2 cm below the costal margin.
A) Antibiotics
B) Adenosine
C) Prostaglandin E1 (alprostadil)
D) Beta-blocker

Answer 24

B) Adenosine
EXPLANATION
In regular, narrow QRS tachycardia, the best therapeutic choice is adenosine, which can stop the AV reentry tachycardia. In the setting of atrial ectopic tachycardia or atrial flutter adenosine can help in the diagnosis by making the ectopic p-waves or the F-waves more visible during the blockage of the AV conduction.

Question 25

After the paroxysm stops, a new ECG is done. What is the most probable abnormality on the ECG?
A 2-month-old infant is taken to the emergency room with the following complaint: he seemed to be less active the day before and has taken less mothermilk. Today he is remarkebly sleepy, a little tachypneic and has only accepted little mothermilk. On physical examination his skin is a little colder and looks paler. Breath rate: 60/min, pulse: 290/min, liver is 2 cm below the costal margin.
A) Everything is normal
B) Prolonged QTc interval
C) 1st degree AV-block
D) Short PR interval and delta wave of the QRS

Answer 25

D) Short PR interval and delta wave of the QRS
EXPLANATION
The most common cause of paroxismal supraventricular tachycardia at this age is an accessory conduction pathway, so the resting ECG will likely show a short PR interval and a delta wave (not necessarily easy to see, though).

Question 26

Which congenital heart defect should be considered?
A 2-week-old baby girl is taken to the emergency department. She had no problems until today. Today she seems lethargic, could hardly be fed, she is tachypneic and had low urine output. On physical examination the baby’s skin is a little cold and pale. She has tachypnea (70-80/min breath rate), tachycardia (160/min), the liver is 2.5 cm-s below the costal margin. The brachial pulses are well palpable, but the femoral pulses are very weak.
A) Aortic stenosis
B) Patent ductus arteriosus
C) Coarctation of the aorta
D) Aortic atresia

Answer 26

C) Coarctation of the aorta
EXPLANATION
The key to the diagnosis is the weak femoral pulse. If the upper and lower body’s pulses have got different qualities, it must raise the suspicion of coarctation of the aorta. Severe (critical) coarctation of the aorta typically manifests at a few days-one month of age, when the closure of the ductus arteriosus leads to severe lower body circulation problems. It explains why the baby was doing well until now. The other symptoms (tachypnea, tachycardia, hepatomegaly, oliguria) are the consequences of cardiac decompensation and poor circulation distal to the coarctation.

Question 27

Which of the following simple diagnostic method can reinforce our suspicion?
A 2-week-old baby girl is taken to the emergency department. She had no problems until today. Today she seems lethargic, could hardly be fed, she is tachypneic and had low urine output. On physical examination the baby’s skin is a little cold and pale. She has tachypnea (70-80/min breath rate), tachycardia (160/min), the liver is 2.5 cm-s below the costal margin. The brachial pulses are well palpable, but the femoral pulses are very weak.
A) Blood gas analysis
B) Upper- lower body saturation measurement
C) Four extremity blood pressure measurement
D) Blood count, CRP test

Answer 27

C) Four extremity blood pressure measurement
EXPLANATION
If there is any uncertainty in the palpation of the femoral pulses, the most logical diagnostic procedure is the comparison of the upper/lower extremity’s blood pressure. If the blood pressure on the legs is 15-20 mmHg lower than the arms’, it needs further investigation.

Question 28

An echocardiography confirms our suspected diagnosis. Which drug can be life-saving in this situation?
A 2-week-old baby girl is taken to the emergency department. She had no problems until today. Today she seems lethargic, could hardly be fed, she is tachypneic and had low urine output. On physical examination the baby’s skin is a little cold and pale. She has tachypnea (70-80/min breath rate), tachycardia (160/min), the liver is 2.5 cm-s below the costal margin. The brachial pulses are well palpable, but the femoral pulses are very weak.
A) Steroid bolus
B) Peripheral vasoconstrictor
C) A drug for ductus arteriosus closure (indomethacin, ibuprophen or paracetamol)
D) Prostaglandin E1 (alprostadil)

Answer 28

D) Prostaglandin E1 (alprostadil)
EXPLANATION
In severe (critical) coarctation of the aorta the lower part of the body would only get adequate blood supply from the right ventricle, through the ductus arteriosus. Thus the arterial duct should be reopened with prostaglandin E1 (alprostadil). There is not sufficient collateral circulation in a newborn, so if the stenosis is severe, the closure of the duct is detrimental.

Question 29

We start the drug therapy. What shall we do now?
A 2-week-old baby girl is taken to the emergency department. She had no problems until today. Today she seems lethargic, could hardly be fed, she is tachypneic and had low urine output. On physical examination the baby’s skin is a little cold and pale. She has tachypnea (70-80/min breath rate), tachycardia (160/min), the liver is 2.5 cm-s below the costal margin. The brachial pulses are well palpable, but the femoral pulses are very weak.
A) The patient has to be transported to a cardiac surgery center as soon as possible.
B) Pediatric intensive care monitoring until the symptoms get better.
C) We reevaluate the patient in 24 hours and decide the next step then.
D) It is enough to monitor the patient in a normal infant ward.

Answer 29

A) The patient has to be transported to a cardiac surgery center as soon as possible.
EXPLANATION
Besides starting the prostaglandin E1 infusion, the patient needs urgent transport to a cardiac surgery centre, where the operation takes places as soon as possible (usually within 24 hours).

Question 30

Sometimes this type of congenital heart defect is only diagnosed later in childhood. What symptoms are the most common for the referral of these patients?
A 2-week-old baby girl is taken to the emergency department. She had no problems until today. Today she seems lethargic, could hardly be fed, she is tachypneic and had low urine output. On physical examination the baby’s skin is a little cold and pale. She has tachypnea (70-80/min breath rate), tachycardia (160/min), the liver is 2.5 cm-s below the costal margin. The brachial pulses are well palpable, but the femoral pulses are very weak.
A) Hypertension
B) Fatigue
C) Heart murmur
D) Cyanosis

Answer 30

B) Fatigue
EXPLANATION
Coarctation of the aorta manifesting at a later age usually causes no complaints. Most of these patients are referred to a cardiac specialist after a routine healthy check up reveals hypertension and/or heart murmur.