Cadiology 1 PDF

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Work smart: MRCPCH Part 1 B

70.17%
Question: 4 of 10 / Overall score: 60%

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A 7 day old baby presents with cyanosis. This is

pathophysiologically due to abnormal mixing in the following
conditions:
True / False
Times answered
173
Average user rating

3
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Tetralogy of Fallot.
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Transposition of the great arteries.

Correct
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Total anomalous pulmonary venous

drainage.
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Univentricular heart.
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Aortic coarctation.
selected
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Correct
Incorrect answer
B, C, D 'Abnormal mixing' refers to those conditions where

systemic and pulmonary circulations mingle. In Fallot's,
cyanosis is caused by decreased pulmonary blood flow.
Aortic coarctation does not cause cyanosis.
Question supplied by Colin Melville Consultant Paediatrician
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57.61%
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The following may be found in atrioventricular septal defects:
Questions
True / False
176
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RSR1 pattern in V1.
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Mild cyanosis in the early stages.
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A pan-systolic murmur at the apex.

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Wide but variable splitting of the second heart

sound.
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An ejection murmur at the upper left sternal

border.
Incorrect answer selected
Correct
4
Correct
An RSR pattern is seen in primum ASD. An apical pan-systolic

murmur is common, and is caused by mitral regurgitation.
The second heart sound has wide fixed splitting. A
pulmonary flow murmur is usual.
eMedicine article
Question supplied by Colin Melville Consultant
Paediatrician
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Regarding cardiogenic shock:
Questions
True / False
Times answered
175
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Increased firing of neurones in the carotid

body stimulates the vagus nerve.
Correct
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Renal cortical necrosis is a recognised

complication.
Correct
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Constriction of the renal afferent arterioles

stimulates renin production.
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Angiotensin II production is increased in the

liver.
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ANP production is increased.

answer selected
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Incorrect
B, C The vagus slows the heart, and angiotensin II is

produced in the lungs. ANP production is increased in
response to atrial stretching, and generally the effects are
opposite to those of the renin-angiotensin-aldosterone
system.
Question supplied by Colin Melville Consultant Paediatrician
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The following are recognised treatments for Fallot's spell:
65.56%
Times answered
180
Questions
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True / False
Scores
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Noradrenaline infusion
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knee-chest position.
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Morphine 0.1mg/kg.
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Prostaglandin E2 50mg/kg/min.
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Propranolol 0.1mg/kg IV.
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Incorrect answer
Correct
Correct
The Fallot's spell is caused by a sudden increase in right

ventricular outflow tract obstruction caused by spasm of the
infundibulum. There is an acute decrease of blood circulating
around the lungs and the child becomes cyanosed, hypoxic,
and may loose consciousness. Treatment is 100% oxygen via
face mask, Morphine intramuscularly or intravenously, and/or
Propranolol. The knee-chest position is designed to increase
systemic vascular resistance and decrease shunting in the
heart, and Noradrenaline performs the same function. Although
Prostaglandin infusion may be useful in maintaining duct
patency during transfer to an acute cardiac centre, it is
unlikely to be of benefit in the acute situation.
Copyright 2002 Colin Melville
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In the fetal circulation
54.65%
Times answered
172
Questions
Average user rating
True / False
Scores
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Approximately equal volumes of blood are

pumped by the left and right ventricles.
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The saturation of blood returning from the

placenta is about 80%.
Incorrect answer
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Approximately 25% of cardiac output traverses

the fetal lung.
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The ductus venosus is probe-patent in 75% of

cases.
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Pulmonary vascular resistance is

suprasystemic.
Correct
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In the fetal circulation, the vast majority of blood bypasses

the lungs through the ductus arteriosus, but the right and left
ventricles pump approximately equal volumes. The fetus is
relatively hypoxaemic, with fetal haemoglobin being used to
increase oxygen carriage to the tissues. Only 10% of cardiac
output traverses the fetal lung, and the ductus venosus
remains patent until the postnatal period. Approximately 50%
of umbilical venous catheters can be passed through it into the
right atrium postnatally. The pulmonary-vascular resistance
remains suprasystemic until the time of the first breath, when
the combination of lung aeration reducing pulmonary blood
pressure, and umbilical ligation increasing systemic blood
pressure reverses the situation.
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The following lesions can be diagnosed antenatally on a routine 4

chamber view at 18 weeks gestation:
Scores
True / False
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Tetralogy of Fallot.
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Hypoplastic left heart syndrome.
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VSD.
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Pulmonary stenosis.
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Transposition of the great arteries.

answer selected
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Incorrect answer
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Correct
Correct
Incorrect
The routine 4 chamber view is likely to pick up only severe

lesions causing small right or left ventricles, such as
hypoplastic left heart syndrome or pulmonary atresia.
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Times answered
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An infant is noted at postnatal examination to have a slow pulse

rate. ECG confirms congenital complete heart block. The following
statements are true:
Times answered
167
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True / False
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Echocardiography is usually normal.
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It is usually associated with a presence of antiRo antibodies in the mother.

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Heart failure is uncommon.

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Mother's thyroid function should be checked.

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The underlying defect may be due to prolonged

QT Syndrome.
Correct
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The commonest cause is maternal SLE, which may be

asymptomatic, and is associated with anti-Ro antibodies.
These were found in 34/41 mothers who delivered children with
CHB. Echocardiography is usually normal (>60% of cases), and
heart failure is uncommon. Maternal hyperthyroidism can cause
abortion, preterm labour, and fetal tachycardia. Prolonged QT
may occur with hypokalaemia, hypocalcaemia, antihistamines
(e.g. Terfenadine). Congenital prolongation presents with
syncope in late childhood.
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The following are features of aortic coarctation in infancy:
56.02%
Times answered
166
Questions
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True / False
Scores
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Rib-notching.
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Inverted T wave in V6.
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Ejection systolic murmur between the shoulder

blades.
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Radio-femoral delay.
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Plateau pulses.
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Correct
Correct
B, C, D Rib notching is a late sign in adolescents, and is rarely

seen these days. An inverted T wave in V6 suggests left
ventricular strain. Plateau pulses are a feature of aortic
stenosis. There may be a systolic murmur along the left sternal
border with a loud 2nd heart sound.
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The following are recognised features of cardiac failure in the

infant:
Scores
True / False
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Jaundice.
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Splenomegaly.
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Clinically detectable elevation jugular venous

pulse.
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Pallor.
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Grunting.
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Correct
Cardiac failure in the infant usually presents with feeding

difficulties and respiratory distress. On examination, there may
be sweating, tachypnoea, nasal flaring, recession, grunt,
hepatomegaly, and splenomegaly. Pallor may reflect poor
peripheral perfusion, and there may be a murmur from the
underlying heart lesion. The squat neck of the infant makes
the jugular venous pulse impossible to assess reliably, and
jaundice does not occur.
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73.26%
Times answered
178
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Interventional catheterisation can be used to repair the following

defects:
Scores
True / False
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Ostium primum ASD.

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Ostium secundum ASD.
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Muscular VSD.
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PDA.
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Dysplastic pulmonary valve.

selected
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Incorrect answer
Correct
Correct
Correct
Incorrect answer
B, D Ostium primum ASD is at the minor end of the spectrum of

AV canal defects. Perimembranous VSDs require surgical
closure, but muscular ones often close themselves. Dysplastic
pulmonary valves can be palliated by balloon dilatation, but
usually require surgical repair later. Secundum ASDs and PDA,
can by closed using umbrella devices.
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60.70%
Times answered
171
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4
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65.17%
Times answered
174
Myocarditis is a recognised association of:

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Salmonella infection.
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Mycoplasma infection.
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Lyme Disease.
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Measles.
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Hurler's Syndrome.
selected
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5
Incorrect answer
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Incorrect answer
A, B, C, D Hurler's is associated with a cardiomyopathy.

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Recognised features of a large uncomplicated patent ductus

arteriosus include:
Scores
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Narrow pulse pressure.
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Pulmonary plethora.
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Decreased noise of murmur on squatting.

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Left ventricular hypertrophy on ECG.

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Soft pulmonary second heart sound.

Correct
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Incorrect answer
B, D A large uncomplicated patent ductus arteriosus results in

a bounding pulse and wide pulse pressure, because of
increased cardiac output on the left side of the heart draining
to a low resistance pulmonary circulation. Because of
increased left to right shunt, pulmonary plethora and
cardiomegaly are characteristic. Squatting will increase
systemic arterial resistance, but this is unlikely to have any
effect on the murmur. Because the left ventricle pumping at
excessive volume, left ventricular hypertrophy and strain may
be seen on the ECG. The second pulmonary heart sound is loud
because of pulmonary hypertension, but may not be audible
beneath a loud murmur.
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65.43%
Times answered
173
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Do the following suggest pulmonary rather than aortic

Questions
Scores
valve stenosis?
66.43%
Times answered
199
Average user rating
True / False
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A deep S wave in V2
selected
Incorrect answer
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An ejection click best heard in the second and

third left intercostal spaces
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An ejection murmur radiating to the neck

Correct
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An upright T wave in V1
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Prominence in the left upper mediastinum on

chest x ray
Incorrect answer
An ejection murmur radiating to the back (along the

direction of the pulmonary arteries) is found in pulmonary
stenosis. The ejection click is found at the apex in aortic
stenosis.
A deep S wave in V2 and large R wave in V6 suggests left
ventricular hypertrophy (LVH), while an upright T wave in
V1 suggests right ventricular hypertrophy (RVH).
A prominent left upper mediastinum on chest x ray may be
caused by post-stenotic aortic dilatation in aortic stenosis.
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The following are indications for immediate transfer to a tertiary

cardiology centre in a neonate:
Scores
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Suspected large VSD.

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Suspected ASD.
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A diagnosis of Down's Syndrome.
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A diagnosis of Kawasaki Disease.

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Suspected aortic coarctation.
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Incorrect answer
Correct
Correct
E Urgent transfer to a tertiary cardiac centre is required for

life-threatening conditions, particularly those dependent on the
ductus arteriosus for survival. These include: 1. Hypoplastic
left heart syndrome. 2. Critical aortic valve stenosis. 3.
Coarctation of the aorta. 4. Interruption of the aortic arch.
The other conditions mentioned require more routine cardiac
evaluation.
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77.14%
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182
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73.30%

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Broad complex tachycardias:
Questions
True / False
Times answered
182
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May be associated with shock.
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May be associated with pulselessness

Correct
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In the shocked patient are usually treated

with synchronous DC shock.
Correct
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Are uncommon in childhood.

answer selected
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May be due to supraventricular tachycardia.

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Correct
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Incorrect
Wide complex tachycardias may be due to ventricular

fibrillation, ventricular tachycardia, or supraventricular
tachycardia with associated ventricular conduction defect.
It may therefore be associated with shock and
pulselessness. Unstable patients should therefore be treated
as if they have ventricular tachycardia. Adenosine will
distinguish the rare SVT with conduction defect, and
Lidocaine can be tried in VT if no shock is present.
Otherwise (and usually) synchronous DC shock 0.5J/kg is
used.
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Regarding infective endocarditis:
59.12%
Times answered
182
Questions
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True / False
Scores
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The commonest causative organism is Group A

Streptococcus.
Correct
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Lesions are most frequent in areas of high

velocity blood flow.
Correct
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Amoxicillin is first choice for prophylaxis in

procedures done under local anaesthetic.
Correct
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Presentation in infancy is extremely rare.

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Splenomegaly is a subtle early sign.

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B, C, D Infective endocarditis is usually caused by Strep.

Viridans (Group D), but Staphylococcus aureus is becoming
increasingly common. The portal of entry is usually the teeth.
The organisms settle usually on congenital or rheumatic heart
lesions, particularly in areas of high velocity blood flow. Early
symptoms and signs are usually mild, and include prolonged
fever and weight loss, which may last for several months
before diagnosis. A rare presentation is with high fever and
prostration, but the usual course is somewhere between these
two. Fever, fatigue, myalgia, changing heart murmurs, heart
failure, splenomegaly and petechia are common. Serious
complications include cerebral abscess and mycotic aneurysms.
Osler's nodes, Janeway lesions and splinter haemorrhages may
occur due to vasculitis.
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The following findings suggest that a murmur is innocent:
76.63%
Times answered
184
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True / False
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It is grade 3/6
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It has a vibratory quality.
Correct
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It is loudest at the apex.
Correct
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It is only heard when the patient is febrile.

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It changes in intensity with posture.

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Correct
About 30% of children can have innocent murmurs. These are

usually ejection generated by the outflow tracts of the left of
right side of the heart, or venous hums due to turbulent flow in
the head and neck veins. The hallmarks are their localised
nature; the fact they are confined to systole; the fact they
are soft; and that they are associated with no symptoms or
other signs.
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The following are recognised causes of cyanosis in the newborn:
66.02%
Times answered
186
Questions
Average user rating
True / False
Scores
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Persistent fetal circulation.
Community
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Severe anaemia.
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Congenital pneumonia.
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Transient tachypnoea of the newborn.

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Hypoplastic left heart syndrome.
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Correct
4
Correct
Incorrect answer
Correct
Cyanosis can be caused by non-cardiac or cardiac lesions. The

former include: persistent fetal circulation, hyaline membrane
disease, congenital pneumonia and transient tachypnoea of
the newborn. The latter include lesions due to abnormal mixing
(TGA, univentricular heart) or to decrease pulmonary blood
flow (pulmonary atresia, Fallot's Tetralogy). Hypoplastic left
heart syndrome can lead to severe congestive cardiac failure,
shock and secondary cyanosis.
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Nitric oxide (endothelial relaxation factor):
Questions
True / False
Scores
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Times answered
186
Average user rating
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Unlike prostacyclin, promotes platelet

aggregation.
Correct
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Can be enzymatically synthesised from

glyceryl trinitrate.
Correct
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Has a half life of 1-2 minutes in vivo.

Correct
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Can be synthesised by macrophages.

Correct
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Is synthesised from L-arginine.
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Correct
Prostaglandin I2 inhibits platelet aggregation via cAMP, while

nitric oxide works via cGMP. Glycerol trinitrate and
nitroprusside are converted to nitric oxide before having
their pharmacological effects. Nitric oxide is synthesised by
macrophages, lymphocytes, endothelial cells, and is a
neurotransmitter and vasodilator. When given by inhalation it
is a specific pulmonary vasodilator because of its short half
life.
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Paradoxical (reverse) splitting of the second heart sound is typical

of:
Scores
True / False
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Ostium primum ASD.
Correct
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Pulmonary stenosis.
Correct
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Aortic stenosis.
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Left bundle branch block.
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Ostium secundum ASD.
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Correct
Correct
Correct
C, D Paradoxical (reverse) splitting of the second heart sound

occurs when splitting sounds larger in expiration than
inspiration. Normally, inspiration increases right-sided filling and
delays pulmonary valve closure. In situations such as aortic
stenosis, or left bundle branch block, delayed closure of the
aortic valve results in reverse splitting.
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54.92%
Times answered
181
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3
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Times answered
Diastolic dysfunction of the left ventricle may be a dominant

feature of:
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True / False
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192
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Hypertrophic obstructive cardiomyopathy.

Correct
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Endocardial fibroelastosis.
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Doxorubicin toxicity.
selected
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Anomalous left coronary artery.
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Myocardial infarction after Kawasaki Disease.

Correct
Incorrect answer
Correct
A, B, C, D, E Diastolic dysfunction is a particular feature of

anthracycline chemotherapy, iron overload, and heart
rejection following transplantation. It may also be seen in
cardiac ischaemia.
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Regarding supraventricular tachycardias:
Questions
True / False
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192
Average user rating
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Rates of 120-180 beats per minute are typical

in adolescence.
Correct
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They are usually associated with structural

cardiac defects.
Correct
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Lown-Ganong-Levine Syndrome is the most

commonest cause.
Correct
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J waves may be seen.

selected
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DC shock is most commonly required.

Correct
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Times answered
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Incorrect answer
All answers are false. SVT is usually due to re-entry within

the AV node due to an accessory pathway, and is
characterised clinically by abrupt onset and cessation. The
heart rate exceeds 180 beats per minute, and can
occasionally be as fast as 300 beats per minute. The heart
is usually anatomically normal, but there may be an
associated bypass tract in WPW or Lown-Ganong-Levine
Syndromes. It may also occur in relation to Ebstein's
anomaly or corrected transposition of the great arteries. In
older children it can be precipitated by sympathomimetics
such as cold cures. Vagal manoeuvres or facial immersion in
ice may abort the attack. Adenosine may be used in the
non-shocked patient, or DC cardio-aversion 0.5J/kg in the
child with congestive heart failure.
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Regarding systemic hypertension in childhood:
59.79%
Times answered
192
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True / False
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Sodium nitroprusside is useful for the long-term

treatment of severe cases.
Correct
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Headache is the usual presenting feature.

Correct
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It is defined as systolic blood pressure above

the 99th centile for age.
Correct
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Abnormalities are frequently seen on DMSA

scan.
Correct
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Aortic coarctation is the commonest secondary

cause.
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D Sodium nitroprusside is useful only in the short term, as

cyanide levels accumulate with time. Hypertension is usually
diagnosed incidentally, and is defined as systolic blood
pressure >95th centile for age. Secondary causes are usually
due to renal abnormalities, with reflux associated scarring
being the commonest renal disease. This will cause
abnormalities on DMSA scan. Coarctation of the aorta is the
commonest non-renal cause, with
pheochromocytoma/neuroblastoma, congenital adrenal
hyperplasia, Cushing Syndrome and steroid therapy being rarer
causes.
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A 3 month old girl presents with apnoea. She had been well
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that morning, but had become unsettled, crying

inconsolably and gradually more mottled. Mother was
207
bringing her to A and E when she stopped breating. She

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responded to physical stimulation. She was born at
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40+3/40 weighing 3.6kg and there were no neonatal

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problems.
On examination she has a temperature of 36.3&degC
(tympanic), RR 30/min and HR of 240/min. Her pulse in
thready. She has a 3 cm liver and gallop rhythm.
What is the most likely diagnosis?
(Please select 1 option)
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Acute life-threatening event
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Cardiac dysrhythmias
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Seizures
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Sudden infant death syndrome
Correct
The history suggests apnoea precipitated by

tachyarrhythmia. This is most likely to be a supraventricular
tachycardia. This can be confirmed by ECG monitoring, and
is usually successfully reverted by adenosine with digoxin
maintenance therapy. An echocardiogram will exclude the
rare possibility of an underlying structural defect.
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A 2-year-old child has an uncomplicated coarctation of the aorta.

The constriction is located distal to the left subclavian artery.
Which of the following would be decreased in this patient?
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64%
3%
12%
15%
5%
Times answered
Scores
260
Community
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Blood flow in the lower body
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Blood flow in the upper body
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Blood pressure in the upper limbs
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Vascular resistance in the lower limbs

correct answer
This is the
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Vascular resistance in the upper limbs

answer selected
Incorrect
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This patient with a fully compensated coarctation blood flow is

normal in both the lower and upper limbs despite increased
pressure in the upper limbs compared to lower body. Thus as
resistance=pressure/blood flow, resistance must be lower in
the lower limbs.
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Theme: Genetics - cardiac abnormalities in genetic disorders
79.44%
Times answered
201
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Angelman's syndrome
Beckwith-Wiedemann syndrome
Congenital Rubella syndrome
Down syndrome
Foetal alcohol syndrome
Glycogen storage disease
Marfan's syndrome
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4
H Noonan's syndrome
I
Turner's syndrome
Williams syndrome
Match each of the following cardiovascular abnormalities to the

single most likely associated genetic disorder.
Dilation of the aorta with aneurysms.
Angelman's syndrome
Incorrect - The correct answer is Marfan's syndrome
In Marfan's syndrome dilatation of the ascending aorta is often

seen with or without aneurysms. Less commonly the thoracic
abdominal aorta or pulmonary arteries are affected with
secondary aortic regurgitation and mitral valve prolapse.
Supra-valvular aortic stenosis.
Williams syndrome
Correct
In Williams syndrome supra-valvular aortic stenosis is the most

common cardiac lesion. Septal defects also occur as well as
peripheral branch pulmonary artery stenosis.
Pulmonary stenosis.
Noonan's syndrome
Correct
In Noonan's syndrome pulmonary valve stenosis due to a

dysplastic or thick valve is seen often associated with left
ventricular hypertrophy. Branch stenosis of the pulmonary
artery also is found in Noonan's syndrome.
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64.16%
Times answered
203
Theme: Congenital cardiac defects

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Angelman's syndrome
Beckwith-Wiedemann syndrome
Congenital Rubella syndrome
Down syndrome
Foetal alcohol syndrome
Glycogen storage disease
Marfan's syndrome
Average user rating

4
H Noonan's syndrome
I
Turner's syndrome
Williams syndrome
Match each of the following cardiovascular abnormalities to the

single most likely associated disorder.
Endocardial cushion defect.
Angelman's syndrome
Incorrect - The correct answer is Down syndrome
In Down syndrome approximately 40% of children have a

congenital heart disease. The most common being endocardial
cushion defects although VSDs, ASDs and PDA also occur.
Coarctation of the aorta.
Turner's syndrome
Correct
In Turner's syndrome cardiac defects are common. 30% include

bicuspid aortic valves with the second most common heart
defect being coarctation of the aorta. Aortic stenosis, mitral
valve prolapse and hypertension are also found.
Septal defects.
Down syndrome
Incorrect - The correct answer is Foetal alcohol syndrome
In foetal alcohol syndrome individuals have poor growth,

developmental delay and usually characteristic facial features
including microcephaly and a short smooth philtrum. The most
common cardiac lesion in these children are septal defects
primarily ventricular septal defects.
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78.40%
Times answered
213
Theme: Emergency medicine

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Adenosine
Adrenaline
Atropine
DC shock
Dobutamine
Diving reflex
Endotracheal intubation
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4
H Intraosseous line
I
IV Morphine
Naloxone
Select the most appropriate emergency treatment for the following

children:
A 4-year-old child is rescued from a house fire. She is admitted
tachypnoeic and tacchycardic. She has soot in her nostrils.
Correct
This relates to burns. Most deaths following house fires occur

secondary to smoke inhalation. Amongst the indicators of
inhaled smoke injury is deposits around the mouth and nose.
Oedema follows thermal injury and therefore any suspicion of
airway compromise should result in endotracheal intubation.
A 3 month old baby is admitted with a history of poor feeding. On

arrival he has a pulse rate of 220 beats per minute.
DC shock
Incorrect - The correct answer is Diving reflex
Supraventricular tachycardia is the diagnosis. Vagal stimulation

is the treatment of choice and the diving reflex is the simple
procedure elicited by submerging the baby's face in to ice or
placing an ice bag over the face. The diving reflex increases
vagal tone, slows AV conduction interrupting the tachycardia.
A 13-year-old boy is admitted with meningococcaemia. He is in

shock. Peripheral cannulation is difficult.
Intraosseous line
Correct
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This relates to a child in shock. In many life-threatening

conditions venous cannulation is difficult. It is important to
obtain vascular access very quickly and therefore intraosseous
infusion is recommended.
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68.62%
Times answered
222
Theme: Emergency treatments

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Adenosine
Adrenaline
Atropine
DC shock
Dobutamine
Diving reflex
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4
H Intraosseous line
I
IV morphine
Naloxone
Select the most appropriate emergency treatment for the

following children.
A 5-year-old boy is brought to the hospital with 15% scalds to his
chest.
Incorrect - The correct answer is IV morphine
This relates to a child with serious burns. Children who have

been burnt are in severe pain and therefore IV morphine is the
analgesic of choice.
A 14-year-old girl with a history of previous overdoses is admitted

to the Emergency Department apnoeic and unconsciousness. ECG
shows ventricular fibrillation. CPR is commenced.
DC shock
Correct
This case describes a child in ventricular fibrillation. This is

uncommon in childhood although may occur as a result of
tricyclic antidepressant overdose and hypothermia. If the
arrest is witnessed a precordial thump is carried out otherwise
electrical de-fibrillation at 4 joules per kilogram.
A term baby is born in poor condition. Apgar scores 3 at 1 minute

and 5 at 5 minutes. CPR is commenced. At 10 minutes he remains
bradycardic.
Atropine
Incorrect - The correct answer is Adrenaline
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This case describes a baby born in poor condition. A

bradycardia in an unstable newborn requires oxygenation,
ventilation and cardiac compressions. IV adrenaline is
administered, as atropine is ineffective in this age group.
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A 3 month old girl presents with liver enlargement of 4 cm.

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She is slightly breathless and not gaining weight well. Full

term normal delivery, noted to have Down's facies,
confirmed as trisomy 21 on karyotype. Immunisations up
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to date. No family or social history of note.
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On examination:
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Times answered
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4
Temperature
36.5C
Respiratory rate
45/min (mild recession),
Pulse
140/min
She has cutaneous stigmata of Downs. Pulses are normal

in all 4 limbs. She has an active praecordium with loud S2
and pansystolic murmur loudest at the lower left sternal
border and apex. Liver is 4 cm and spleen 3 cm.
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Aortic coarctation
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Atrial septal defect, primum

answer
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Atrial septal defect, secundum
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Hypoplastic left heart syndrome
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Ventricular septal defect

selected
This is the correct
Incorrect answer
The key points are the Down's syndrome, heart failure, with
loud murmurs. 50% of Down syndrome patients have structural
congenial heart disease. The commonest abnormalities is atrioventricular canal defect followed by VSD. In the former (ostium
primum ASD) there is a defect in the lower part of the atrial
septum, the medial part of the tricuspid and mitral valves, and
in the upper part of the ventricular septum. Hence there are
overlapping murmurs of ASD, VSD, tricuspid and mitral
regurgitation together with heart failure. After detailed
evaluation by echocardiography, surgical repair is undertaken.
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An 8-year-old girl presents with a 2 week history of

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headache. This has been severe, worst in the mornings,

accompanied by effortless vomiting, and has kept her off
school the past 4 days. Her school performance has been
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Times answered
256
deteriorating over the past term. Full term normal delivery

with no neonatal complications. Immunisations up to date.
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There is no FH/SH of note.

On examination she is on the 50% for height and weight,
apyrexial, with pulse 70/min, respiratory rate 15/min and
blood pressure is 125/100 mmHg. Fundoscppy reveals
blurring of the disk margins bilaterally.
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Cerebral abscess
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Medulloblastoma
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Migraine
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Sinusitis
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Tension headache
Correct
The brief duration of headache, its severity and

characteristics, the absence of family history of migraine, and
findings of slow pulse, raised BP and papilloedema all point to
raised intracranial pressure. The most likely diagnosis is a
tumour. In childhood 2/3 are in the posterior fossa, often
presenting with ataxia or headache. 1/3 are supratentorial,
where raised intracranial pressure and decline in school
performance are commoner presentations.
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4%
4%
79%
A 4-month-old boy presents with heart murmur,

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breathlessness and failure to thrive.
Times answered
He has always breathed a little fast, but this has gradually

worsened. He has been falling through the centiles. He was
born at 40+2/40 weighing 3.22 kg and there were no
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neonatal problems. Immunisations are up to date. There is

no family or social history of note.
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40/min (minimal recession) and pulse is 120/min. He is

below the 3% for weight and on the 50% for head
circumference.
He has an active praecordium, loud P2 and a 3/6
pansystolic murmur maximal at the lower left sternal edge,
but heard all over the praecordium.
The liver is 3 cm.
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Mitral regurgitation
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Tricuspid atresia
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Ventricular septal defect (VSD)
537
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4
On examination the temperature is 36.9C, respiratory rate
Correct
This history suggests heart failure, worsening over the first

few weeks of life, with a pansystolic murmur.
This suggests a VSD causing haemodynamically significant
left-to-right shunt. The shunt volume usually increases in
the first few weeks as the pulmonary vascular resistance
drops. The murmur goes from ejection systolic in the first
few days, gradually becoming pansystolic. Diuretics are
often needed.
Some VSDs (particularly of the muscular septum) close
spontaneously. Others (especially of the membranous
septum) require surgical closure.
Antibiotic prophylaxis is essential to reduce the risk of
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endocarditis.
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A 14-year-old boy falls awkwardly while swinging from a

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rope in an adventure playground. He lands head first,

causing violent flexion of his neck. An ambulance is called.
He has previously been fit and well. Full term normal
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No family or social history of note.

On examination the temperature is 36.5C. He has been
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Times answered
268
delivery, no neonatal problems. Immunisations up to date.
Help
intubated and is being bag ventilated in 100% Oxygen.

Heart rate is 60/min and pulse is thready, though the
peripheries are warm and pink. Blood pressure is 75 mmHg
systolic by dynamap. He is paralysed from the neck down,
and has a GCS of 8.
What is the most mechanism of shock?
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Anaemic
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Cardiogenic
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Distributive
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Hypovolaemic
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Septic
Correct
The history suggests spinal cord transection causing

neurogenic shock. Severe head injury or cord transection can
prevent autonomic control of blood vessels, leading to fluid
loss into the tissues (distributive shock). Unusually for shock
the pulse is slow and the peripheries warm. Fluid replacement
and treatment of the underlying cause (if possible) is
indicated.
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A 2-week-old girl presents with a history of poor feeding,

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worse over the last six hours. 38+3/40, 2.95 kg, no

problems at birth. First pregnancy of healthy Caucasian
mother. No FH/SH of note. No drugs nor immunisations.
Times answered
263
Community
On examination temperature is 35.4C (tympanic), RR

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60/min, HR 160/min on monitor (all pulses impalpable).

Cold mottled peripheries, capillary refill time six seconds.
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Oxygen saturations 45% in air. Gallop rhythm, 4 cm

hepatomegaly, no spleen. No bruising or rash.
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Congenital adrenal hyperplasia
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Escherichia coli septicaemia
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Galactosaemia
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Group B streptococcal infection
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Correct
The history is of shock, hepatomegaly and hypoxia in a 2week-old child.

The most likely diagnosis is duct-dependent congenital
heart disease. Left heart lesions causing this presentation
include:
aortic coarctation/hypoplastic arch

truncus arteriosus
critical aortic stenosis
hypoplastic left heart syndrome.
Right-sided lesions include:
transposition
severe Fallot's
pulmonary atresia +/- VSD.
After intubation and ventilation the key step is to open the
duct using prostaglandin E2 infusion.
Acidosis may require separate correction.
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Urgent transfer to a cardiac centre should then follow so a

specific diagnosis can be made.
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74.33%
Times answered
274
Theme: Syncope.
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Arrhythmia
Breath-holding, blue
Breath-holding, white
Hypertrophic cardiomyopathy
Hypoglycaemia
Hypotention, vasovagal
Hypotension, orthostatic
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H Seizure
I
Tumour, brain
For each scenario choose the most likely diagnosis:

An 8-year-old girl presents with loss of consciousness and
occasional awareness of heartbeat. She has been deaf from birth.
Arrhythmia
Correct
The 8-year-old girl has deafness and palpitations, followed by

syncope. The most likely diagnosis is the Jervell-Lange-Neilsen
variant of long QT syndrome. The milder form is the RomanoWard syndrome.
A 13-year-old girl was found unconscious one Sunday morning

whilst in bed. She had wet herself and took 30 minutes to recover
completely.
Seizure
Correct
The 13-year-old girl has had a generalised seizure as

suggested by incontinence and the prolonged recovery.
An 18 month old boy is referred with loss of consciousness on 6

occasions. Each was preceded by a tantrum.
Tumour, brain
Incorrect - The correct answer is Breath-holding, blue
The 18 month old boy has blue breath-holding episodes. These

should be distinguished from white breath-holding, which is an
extreme vagal response resulting in transient asystole.
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9%
48%
25%
3%
15%
An 16-year-old female is found to be hypertensive at 26

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weeks of her first pregnancy.
Times answered
Blood pressure
144/92 mmHg
Community
Dipstick urine
+++ proteinuria
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Serum creatinine
80 mol/L
24 hour urine collection
1.2 g of protein/d
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What is the most appropriate management?

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Atenolol
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Methyldopa
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Nifedipine
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Ramipril
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Re-measure blood pressure in one week
Correct
This patient has Pre-eclampsia, which is pregnancy induced

hypertension with proteinuria with or without oedema; a
multisystem disorder originating in the placenta. As BP is
>140/90 with associated proteinuria she needs admission for
observation and monitoring of BP, biochemistry, daily weight,
fluid balance and monitoring of urinary protein. Methyldopa
would be the agent of choice to control hypertension. ACEi are
contraindicated in pregnancy due to teratogenicity.
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22%
54%
10%
8%
6%
A 17-year-old boy whose brother had hypertrophic

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cardiomyopathy was referred for a cardiological

assessment. His echocardiogram confirmed the condition.
Which one of the following echocardiographic features is
the most important risk factor for sudden cardiac death?
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Times answered
164
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A gradient of 10 mmHg across the left ventricular

outflow tract
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Significant thickening of the interventricular septum

Correct
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An enlarged left atrium
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Systolic anterior motion of the mitral valve
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The presence of mitral regurgitation
In hypertrophic obstructive cardiomyopathy the cause of

death is usually ventricular tachycardia or ventricular
fibrillation and therefore the thicker the muscle the more
abnormal the cardiac architecture and the higher the risk of
arrhythmia and sudden death.
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148
The following are true of cardiac transplantation:

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the donor heart is provided to the recipient with

the best HLA match
Incorrect answer
selected
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long term hypotension is a problem

answer selected
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recipients suffer less angina but accelerated

coronary artery disease
This is the correct
answer
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the histology of atheroma in the transplanted

heart is the same as that of typical
atherosclerosis
Correct
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75% 5 year survival
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Incorrect
Correct
a-This is true of kidney transplants but hearts go to the most

needy. b-Hypertension is a significant problem related to use
of cyclosporin and denervation of the heart. c-The heart is
denervated. d-The pathology is different being more diffuse
and symmetrical. e-For most centres with conventional
selection criteria.
Transplantation
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Cerebral abscess in the absence of endocarditis is a recognised

complication of:
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True / False
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tetralogy of Fallot
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persistent ductus arteriosus
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tricuspid artresia
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transposition of the great arteries
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atrial septal defect of the ostium primum type

Correct
Correct
Correct
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Times answered
162
Average user rating

1
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Correct
Correct
This is paradoxical embolization seen in right to left shunts. It

is not associated with left to right shunts except if
Eisenmenger's supervenes.
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50.53%
Times answered
169
A short PR interval on the ECG is associated with:

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True / False
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Duchenne muscular dystrophy

answer selected
Community
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dystrophia myotonica
Help
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Friedrich's ataxia
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rheumatic carditis
selected
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hypertrophic cardiomyopathy
answer selected
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Incorrect
Correct
Correct
Incorrect answer
Incorrect
a-Commonest change is large R/S ratio in V1, but short PR

interval can also occur. b-Associated with a prolonged PR
interval which may manifest before the overt clinical
manifestations of the disease. (Am J Med 1978(61), 452). c-In
a quarter of cases (JACC 1986(7), 1370-8). d-Long PR. e-In
some cases HOCM is associated with a short PR. Commoner
causes are WPW and Lown-Ganong-Levine syndromes.
ECG changes in Duchenne

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The following are recognised causes of pericardial disease:
58.44%
Times answered
154
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True / False
Scores
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Hepatitis B virus
Correct
Community
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Mulibrey nanism
Help
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rheumatic fever
Correct
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hypereosinophilic syndrome
answer selected
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ankylosing spondylitis
selected
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Incorrect
Incorrect answer
a-And Coxsackie B (the commonest viral cause), ECHO, rubella,

mumps and influenza viruses. b-An autosomal recessive
condition affecting MUscle LIver BRain and EYes and includes
pericardial fibrosis. c-A small effusion accompanies almost all
cases of acute rheumatic fever. d-This affects the
endomyocardium causing fibrosis but may also cause a
pericarditis. e-Aortic regurgitation and AV block are recognised
consequences of ankylosing spondylitis.
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Theme: Syndromes associated with congenital heart disease
78.21%
Times answered
179
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Charcot-Marie-Tooth syndrome
Down syndrome
Fragile X syndrome
Marfan's syndrome
Noonan syndrome
Sturge-Weber syndrome
Tetrology of Fallot
Average user rating

5
H Tourette syndrome
I
Turner's syndrome
William's syndrome
For each of the cardiac lesions described below, choose the

most commonly associated syndrome from the above list
of options. Each option may be used once or not at all.
Supravalvular aortic stenosis
Sturge-Weber syndrome
Incorrect - The correct answer is William's syndrome
William's syndrome is characterised by
short stature
characteristic facies
supravalvular aortic stenosis
mild to moderate learning difficulties
transient neonatal hypercalcaemia.
Coarctation of aorta
Turner's syndrome
Correct
This is characterised by
45,X genotype
ovarian dysgenesis leading to infertility
short stature
webbing of the neck
wide carrying angles
wide spaced nipples.
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However they have normal intellectual development.

Dilatation of aortic root/aortic regurgitation
Marfan's syndrome
Correct
This is an autosomal dominant disorder. The clinical

features are
tall stature
arachnodactyly
high arched plate
increase in length of the lower segment of the body
compared to the upper segment.
The cardiac manifestations include
dilated aortic root

aortic incompetence
mitral valve prolapse
mitral incompetence.
Cardiac cushion defects (leading to ASD, VSD)
William's syndrome
Incorrect - The correct answer is Down syndrome
Features include
characteristic facies
hypotonia
severe learning difficulties
small stature.
About 40% of patients have cardiac anomalies, mainly
endocardial cushion defects leading to ASD and VSD.
Infundibular pulmonary stenosis
Noonan syndrome
Incorrect - The correct answer is Tetrology of Fallot
This is a cyanotic heart disease and the cardinal features

include
infundibular pulmonary stenosis

VSD
right ventricular hypertrophy
over-riding of the aorta.
Charcot-Marie-Tooth syndrome: autosomal dominant
peroneal muscular dystrophy.
Fragile X syndrome: moderate learning difficulty,
macrocephaly, characteristic facies (long face, large ears,

prominent mandible and forehead).
Noonan syndrome: facies, mild learning difficulties, short
webbed neck, short stature and congenital heart disease
(pulmonary valvular stenosis, ASD).
Sturge-Weber syndrome: haemangiomas in the distribution
of trigeminal nerve and in the brain. Sometimes can have
intractable epilepsy.
Tourette syndrome: tics, compulsive utterances of obscene
words (coprolalia).
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The following regarding Atrial Natriuretic Peptide are correct
Times answered
215
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True / False
Scores
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It is secreted in response to right atrial

distension
Correct
Community
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It inhibits aldosterone release
Help
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It is a direct inotrope
selected
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It is a direct vasodilator
selected
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It is inactivated by endopeptidase
Correct
Incorrect answer
Incorrect answer
Correct
ANP is a 28 aa peptide that is synthesised and released by

atrial myocytes in response to distension, angiotensin II and
endothelin. It causes salt and hence water losses, is a
vasodilator (through possible direct and indirect mechanisms)
and inhibits aldosterone release. Such actions reduce blood
volume and cardiac output. ANP is degraded by neutral
endopeptidase.
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Which one of the following is a recognised feature of

abetalipoproteinaemia?
Scores
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a high serum cholesterol

selected
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palmar xanthomas
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advanced atherosclerotic vascular disease
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abnormal red blood cell morphology

correct answer
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Severe mental retardation
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Incorrect answer
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12%
11%
12%
49%
16%
Times answered
190
Average user rating
This is the
Acanthocytes are seen in abetalipoproteinaemia.

Retinitis pigmentosa is seen in abetalipoproteinaemia.
Neurodegenerative changes are seen such as ataxia but IQ is
normal.
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39%
10%
8%
31%
13%
A post-marketing surveillance study of a new heart failure

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therapy to the market was carried out on 10,000 subjects

who had completed clinical trials. Which one of the following
most accurately reflects the information generated from
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such a study?
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Times answered
204
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Adverse events profile
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Cost benefit analysis
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Cost effectiveness
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Comparative therapeutic efficacy
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Drug potency
This is the correct answer
Post-marketing surveillance/observational studies (phase IV

studies) generally are designed to assess the potential side
effects of new drugs but under everyday conditions and
with a minimum of intervention. In contrast to the
randomized controlled trials, PMS typically include patients
from more extreme age groups, patients with comorbidity
or other risk factors. In order to cover a wide spectrum of
patients and to observe rare events with sufficiently high
probability, PMS enroll a large number of patients, typically
several thousands. Comparative efficacy has already been
undertaken in Phase III studies (RCTs) but can also be
undertaken as part of specific RCT studies later in the
drugs development and potency usually in phase I and II
studies.
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46.67%
Times answered
180
Theme: Congenital heart disease

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Ostium secundum atrial septal defect
Transposition of the great arteries
Total anomalous pulmonary venous drainage
Atrioventricular septal defect
Patent ductus arteriosus
Pulmonary valve stenosis
H Coarctation of the aorta

I
Tetralogy of Fallot
Which of the above is the most likely diagnosis in the

following cases?
A 7-week-old infant presents with breathlessness on feeding and
failure to thrive. On examination his femoral pulses are difficult to
feel but present. Chest radiograph shows cardiomegaly and
increased vascular markings.
Incorrect - The correct answer is Coarctation of the aorta
Absent or weak femoral pulses suggest coarctation.

Remember association with Turner's syndrome in females.
A 13-year-old girl is referred for evaluation of her short stature.
She is pre-pubertal. On auscultation she has an ejection systolic
murmur in the second and third left intercostal spaces radiating to
the back, but is asymptomatic.
Coarctation of the aorta
Incorrect - The correct answer is Pulmonary valve stenosis
The murmur describes pulmonary stenosis which could also

be a left peripheral pulmonary stenosis. She is short and
has delayed puberty, and coupled with the cardiac findings,
would suggest Noonan's syndrome.
An infant is seen for his 6-week-check and found to have a loud
ejection systolic murmur in the third left intercostal space and a
single second heart sound on examination. There is no obvious
cyanosis but a suggestion of mild desaturation.
On the chest x ray there is a concavity on the left heart border
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and decreased pulmonary vascular markings.
Tetralogy of Fallot
Correct
Tetralogy of Fallot may present later than in the neonatal

period. The ejection systolic murmur is from the
infundibular stenosis. The desaturation results from the
right to left shunt across the VSD.
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14%
4%
7%
11%
64%
A 16-year-old boy is admitted after a blackout at the

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dentist. His mother describes how he blacked out as the

dentists began performing a filling and that he jerked his
arms a few times and was then incontinent. He awoke after
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Times answered
224
a minute or so and was oriented but nauseous.There were

no similar episodes in the past and he is totally unaware of
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what happened.
Examination was normal and his ECG was normal. Which
one of the following is the most likely diagnosis?
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Complex partial seizure
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Pseudoseizure
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Stokes-Adams attack
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Tonic-clonic seizure
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Vasovagal syncope
Correct
Vasovagal syncope is common during dental procedures,

mainly induced by pain (as the dentist started drilling). The
fact that he recovered very quickly supports the diagnosis
of syncope. It is common to have jerking of limbs due to
brain hypoxia.
ECG is always normal. Incontinence of urine can occur, but
not biting of the tongue.
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5%
0%
92%
0%
3%
A 16-year-old male is brought to emergency admissions

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with alcohol intoxication. An initial ECG reveals atrial

fibirillation but a repeat ECG after 12 hours when he has
sobered up, shows sinus rhythm. An echocariogram is
Community
patient?
213
Average user rating

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Times answered
normal. What is the most appropriate management for this
Help
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Aspirin for 3 months
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Bisoprolol for 3 months
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Lifestyle advice
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Sotalol for one month
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Warfarin for one month
Correct
Excessive Alcohol is a recognized cause for atrial fibirillation

and is the likely cause here as the rhythm has reverted to
sinus after 12 hours. There is also no evidence of
structural heart disease as the echocardiogram was normal.
Therefore this patient needs advice regarding moderation
of alcohol consumption and needs to be warned of the
toxic effects that alcohol can have on the heart and other
organs.
There is no indication for short term aspirin. Atenolol
provides rate control, which is not an issue.
Sotalol/amiodarone and Flecainide can be used in
paroxysmal AF. Short term Warfarin is used for 4-6 weeks
prior to elective cardioversion to protect against embolic
complications.
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1%
14%
6%
3%
76%
A 16-year-old male presents with acute severe asthma. On

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examination his peripheral pulse volume fell during

inspiration.
Which one of the following is the most likely explanation for
this clinical sign?
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Times answered
220
Average user rating
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The cardiac effect of high dose beta agonist

bronchodilator drugs
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A falling heart rate on inspiration
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Myocardial depression due to hypoxia
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Peripheral vasodilatation
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Reduced left atrial filling pressure on inspiration

Correct
This patient is demonstrating pulsus paradoxus. The right

heart responds directly to changes in intrathoracic
pressure, while the filling of the left heart depends on the
pulmonary vascular volume. At high respiratory rates, with
severe air flow limitation (eg acute asthma) there is an
increased and sudden negative intrathoracic pressure on
inspiration and this will enhance the normal fall in blood
pressure.
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In disorders of cardiac conduction:
46.33%
Times answered
218
Questions
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True / False
Scores
Community
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right bundle branch block and left axis deviation

indicate bifascicular block
Correct
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i
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left anterior hemiblock causes right axis

deviation
Correct
i
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left posterior hemiblock causes left axis

deviation
i
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n
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deep S waves are found in leads I and V6 in

right bundle branch block
Correct
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right ventricular hypertrophy cannot be

diagnosed in the presence of left bundle branch
block
Correct
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The bundle of His consists of three fascicles: - the right

bundle branch, and the anterior and posterior fascicles of
the left bundle branch. Bifascicular block describes the
combination of right bundle branch and either left anterior
or left posterior hemiblock.M
Left anterior hemiblock causes left axis deviation, and left
posterior hemiblock causes right axis deviation. A-V
conduction is maintained by the remaining functional
fascicle. Impaired conduction in the latter may be manifest
by a prolonged P-R interval. Bifascicular block may progress
to trifascicular block and may be an indication for the
insertion of a permanent cardiac pacemaker.
Right bundle branch block (RBBB) in isolation, is a normal
ECG variant. The delayed right ventricular depolarization
results in a second positive (R???) wave in the right
ventricular leads and a second negative wave in the left
ventricular leads. Diagnostic features include a widened
QRS complex and a second positive wave in V1 . Other
features include deep slurred S waves in leads I, aVL, V4-6 ,
and S-T segment and T wave changes in leads V1-3 .
Left bundle branch block (LBBB) is always pathological. In
the presence of LBBB, the diagnosis of ventricular
hypertrophy, myocardial ischaemia/infarction, and
abnormalities of the p wave, QRS complex or S-T segment
cannot be made using conventional ECG criteria.
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20%
0%
0%
0%
80%
A 3-month-old girl presents with apnoea. She had been

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well that morning, but had become unsettled, crying

inconsolably and gradually more mottled.
Mother was bringing her to the emergency department
when she stopped breathing . She responded to physical
Help
stimulation. She was born at 40+3/40 weighing 3.6kg and

there were no neonatal problems.
Shop
(tympanic), RR 30/min and HR of 240/min. Her pulse in

thready. She has a 3 cm liver and gallop rhythm.
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Acute life-threatening event
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Bronchiolitis
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Gastro-oesophageal reflux
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Seizure
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Supraventricular tachycardia
Times answered
230
Average user rating

5
On examination she has a temperature of 36.3C
Correct
The history suggests apnoea precipitated by

tachyarrhythmia. This is most likely to be a supraventricular
tachycardia.
This can be confirmed by ECG monitoring, and is usually
successfully reverted by adenosine with digoxin
maintenance therapy.
An echocardiogram will exclude the rare possibility of an
underlying structural defect.
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2%
4%
3%
64%
26%
A 4-year-old girl presents with heart murmur and

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breathlessness. She was well until 7 days ago, when she

developed a nasty cold and fever. She has progressively
deteriorated. Full term normal delivery with no neonatal
Community
Times answered
208
complications. Immunisations up to date. There is no

FH/SH of note.
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On examination:
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Respiratory rate
25/min
pulse
130/min
BP
80/60 mmHg
She is apyrexial and on the 25% centile for height and

weight. She has slightly cool peripheries and a capillary refill
time of 2 seconds. She has a loud gallop rhythm and 4 cm
liver. Pulses are easily palpable in all 4 limbs. Chest X-ray
shows globular cardiac enlargement with pulmonary
plethora.
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Aortic coarctation
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Atrioventricular canal defect
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Kawasaki disease
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Myocarditis
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Correct
The history is of viral illness followed by heart failure. The likely

diagnosis is viral myocarditis or cardiomyopathy. An
echocardiogram shows dilated chambers with poor
contractility. Treatment is with diuretics and ACE inhibitors.
Spontaneous recovery is variable, and transplant may be
required.
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A 3-year-old boy is referred with heart murmur. He has

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otherwise been well, with no blueness and normal exercise

tolerance. Full term normal delivery with no neonatal
complications. Immunisations up to date. There is no
Community
FH/SH of note.
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On examination he is well and apyrexial. There are no

dysmorphic features. Respiratory rate is 17/min and pulse
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100/min. He has a widely split S2 which does not vary with

breathing, and 2/6 ejection systolic murmur at the upper
left sternal edge. Liver is impalpable.
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Aortic coarctation
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Innocent murmur
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Pulmonary stenosis
Correct
The history of asymptomatic heart murmur, with fixed

splitting of the second heart sound and pulmonary flow
murmur all point to atrial septal defect (ASD) as the
diagnosis here.
This may be treatable using 'umbrella' closure via a cardiac
catheter. Occasionally surgical closure is required if very
large or associated with other heart defects.
Antibiotic prophylaxis is essential to reduce the risk of
endocarditis, although the risk is lower than for 'high-flow'
lesions.
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Times answered
222
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A 3-year-old girl is referred with incidentally-discovered

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heart murmur. She has had no blueness nor breathless.

Full term normal delivery with no neonatal complications.
Immunisations up to date. There is no FH/SH of note.
Times answered
223
Community
On examination she is apyrexial, well and well grown with

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no dysmorphic features. Respiratory rate is 20/min and

pulse is 100/min. Heart sounds are normal. She has a 1-
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2/6 murmur best heard under the right clavicle, which

changes with posture. The liver is impalpable.
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Aortic stenosis
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Atrioventricular canal defect
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Innocent murmur
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Pulmonary stenosis
Correct
The history is of asymptomatic heart murmur which is soft and

varies with posture. This is most likely an Innocent Murmur, in
this case a venous hum caused by the rumble of blood through
the great veins of the neck. It requires explanation and
reassurance.
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An 11-day-old baby presents with poor feeding and

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breathlessness. She had been born at 37/40 weighing 2.7

kg by elective caesarian section. She has never fed well,
and had deteriorated markedly on the day of admission.
Times answered
260
Community
On examination she is responding to pain, mottled and had

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a tympanic temperature of 34.6C. Her heart rate is

130/min with impalpable pulses and gallop rhythm. Her
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respiratory rate is 40/min with marked recession. She has a

4 cm liver. Her saturations and blood pressure are
unrecordable, but she has obvious central cyanosis.
What is the most likely mechanism of shock?
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Anaemic
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Cardiogenic
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Distributive
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Hypovolaemic
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Septic
Correct
The picture suggests duct-dependent congenital heart

disease, with progressive worsening of symptoms as the
duct closed. Typical conditions include
aortic coarctation
critical aortic stenosis
truncus arteriosus
hypoplastic left heart syndrome.
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Times answered
279
Theme: Chest pain in children.

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Asthma
Costochondritis
Herpes zoster
Hypertrophic cardiomyopathy
Hyperventilation
Mitral valve prolapse
Musculoskeletal pain
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H Pericarditis
I
Psychogenic

A 13-year-old girl presents with chest pain. On examination there
is pain and tenderness of the 8th right costochondral cartilage.
Pericarditis
Incorrect - The correct answer is Costochondritis
The 13-year-old girl has localised chest pain, most likely due to
costochondritis. A history of preceding viral infection or trauma
should be sought.
A 12-year-old boy presents with chest pain. He has previously

fainted during a football game. On examination he has a 2/6
ejection murmur at the left sternal edge.
Musculoskeletal pain
Incorrect - The correct answer is Hypertrophic cardiomyopathy
The 12-year-old boy has a history of collapse on exercise

together with a heart murmur would suggest a possible
diagnosis of HOCM. Mitral valve prolapse is less likely as the
murmur is described as ejection systolic in nature. A family
history of sudden death may also be present.
A 14-year-old girl presents with chest pain and dizziness. She

looks breathless, anxious, and complains of tingling in her fingers.
Hyperventilation
Correct
The 14-year-old girl is hyperventilating. The finger

paraesthesiae amy represent reduced ionised calcium
associated with a respiratory alkalosis. The symptoms should
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resolve with re-breathing into a paper bag.
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Theme: Palpitations in children.

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Anaemia
Cardiomyopathy
Hyperthyroidism
Myocarditis
Panic attacks
Prolonged QT syndrome
Sinus arrhythmia
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H Sinus tachycardia
I

A 14-year-old girl complains of awareness of heart beat. She
began menstruating at 12 years, and has heavy periods. She
appears pale.
Anaemia
Correct
The 14-year-old girl is anaemic due to blood loss, resulting in

palpitations
A 10-year-old girl complains of awareness of heart beat. She has

sweaty palms and a heart rate of 100/min, with a 2/6 ejection
systolic murmur in the pulmonary area.
Hyperthyroidism
Correct
The 10-year-old girl has a resting tachycardia and sweating,

suggesting hyperthyroidism. Careful examination should be
made for goitre, bruit and eye signs. The murmur is caused by
increased flow across the pulmonary valve due to
hyperdynamic circulation.
A 9-year-old boy presents with awareness of heart beat and chest

discomfort. Episodes last about 20-30 minutes. He drinks 4 cups of
coffee per day.
Correct
The 9-year-old boy is probably having episodes of

tachyarrhythmia, probably SVT. This may be precipitated by
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caffeine (coffee, tea, coke) or pseudoephedrine (cold cures).

Caffeine itself may give sinus tachycardia. Asking the patient
to tap out the rate of heartbeat can be helpful.
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Theme: Apnoea
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Blood cultures
CSF culture
CT scan head
Drug screen on mother
EEG
Nasopharyngeal aspirate for viruses
Nasopharyngeal aspirate for pertussis
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4
H pH studies
I
Urine metabolic screen
For each scenario choose the most specific diagnostic

investigation:
A 5 day old girl presents to casualty following a brief apnoeic

episode. On examination she is apyrexial, sweating, has a highpitched cry and is difficult to settle.
CSF culture
Incorrect - The correct answer is Drug screen on mother
The 5day old presents with apnoea in the absence of fever or

structural problem. The other symptoms point towards drug
withdrawal, so a drug screen will be diagnostic.
A 4 month old girl presents with a 12 hour history of poor feeding

and fever. She is rushed to hospital after she stops breathing. On
examination she has an Oxygen saturation of 94%, and responds
to pain. Serum glucose is 3.4 mmol/l.
Blood cultures
Correct
The 4 month old girl presents with a brief history of fever

followed by apnoea. This suggests an infective cause. The
decreased level of consciousness mean that LP should not be
done yet. Blood cultures are likely to be the most helpful test.
A 5 month old boy has a 2 day history of coryza and poor feeding.
On the day of admission he has a brief period of apnoea and is
rushed to hospital. On examination he has 2 cm hepatomegaly
and a blood glucose of 0.8 mmol/l.
Urine metabolic screen
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Correct
The 5 month old presents with apnoea associated with mild

infection and hypoglycaemia. This points to a metabolic cause,
and a metabolic screen should be most helpful.
Apnoea can be caused by 3 main groups of conditions:

infections, obstructions, or toxins/drugs.
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Work smart: MRCPCH Part 1 B

Question: 4 of 10 / Overall score: 60%

A 7 day old baby presents with cyanosis. This is

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Transposition of the great arteries.

Total anomalous pulmonary venous

B, C, D 'Abnormal mixing' refers to those conditions where

Work smart: MRCPCH Part 1 B

Question: 3 of 10 / Overall score: 53%

The following may be found in atrioventricular septal defects:

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RSR1 pattern in V1.

Mild cyanosis in the early stages.

A pan-systolic murmur at the apex.

Wide but variable splitting of the second heart

An ejection murmur at the upper left sternal

An RSR pattern is seen in primum ASD. An apical pan-systolic

Work smart: MRCPCH Part 1 B

Question: 2 of 10 / Overall score: 40%

Regarding cardiogenic shock:

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Increased firing of neurones in the carotid

Renal cortical necrosis is a recognised

Constriction of the renal afferent arterioles

Angiotensin II production is increased in the

ANP production is increased.

B, C The vagus slows the heart, and angiotensin II is

Work smart: MRCPCH Part 1 B

The following are recognised treatments for Fallot's spell:

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Propranolol 0.1mg/kg IV.

The Fallot's spell is caused by a sudden increase in right

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Work smart: MRCPCH Part 1 B

In the fetal circulation

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Approximately equal volumes of blood are

The saturation of blood returning from the

Approximately 25% of cardiac output traverses

The ductus venosus is probe-patent in 75% of

Pulmonary vascular resistance is

In the fetal circulation, the vast majority of blood bypasses

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Work smart: MRCPCH Part 1 B

The following lesions can be diagnosed antenatally on a routine 4

Hypoplastic left heart syndrome.

Transposition of the great arteries.

The routine 4 chamber view is likely to pick up only severe

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Work smart: MRCPCH Part 1 B

An infant is noted at postnatal examination to have a slow pulse

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Echocardiography is usually normal.

It is usually associated with a presence of antiRo antibodies in the mother.

Heart failure is uncommon.

Mother's thyroid function should be checked.

The underlying defect may be due to prolonged

The commonest cause is maternal SLE, which may be

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Work smart: MRCPCH Part 1 B