Download - cadiology 1.pdf
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 4 of 10 / Overall score: 60%
A 7 day old baby presents with cyanosis. This is pathophysiologically due to abnormal mixing in the following conditions:
True / False
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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nmlkj nmlkji Tetralogy of Fallot. Correct
nmlkji nmlkj Transposition of the great arteries. Correct
nmlkji nmlkj Total anomalous pulmonary venous drainage. Correct
nmlkji nmlkj Univentricular heart. Correct
nmlkji nmlkj Aortic coarctation. Incorrect answer selected
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 3 of 10 / Overall score: 53%
The following may be found in atrioventricular septal defects:
True / False
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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nmlkji nmlkj RSR1 pattern in V1. Correct
nmlkj nmlkji Mild cyanosis in the early stages. Correct
nmlkji nmlkj A pan-systolic murmur at the apex. Correct
nmlkj nmlkji Wide but variable splitting of the second heart sound. Correct
nmlkj nmlkji An ejection murmur at the upper left sternal border. Incorrect answer selected
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 2 of 10 / Overall score: 40%
Regarding cardiogenic shock:
True / False
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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nmlkj nmlkji Increased firing of neurones in the carotid body stimulates the vagus nerve.
Correct
nmlkji nmlkj Renal cortical necrosis is a recognised complication. Correct
nmlkji nmlkj Constriction of the renal afferent arterioles stimulates renin production. Correct
nmlkji nmlkj Angiotensin II production is increased in the liver. Incorrect answer selected
nmlkji nmlkj ANP production is increased. Incorrect answer selected
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 2 of 10 / Overall score: 40%
The following are recognised treatments for Fallot's spell:
True / False
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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nmlkj nmlkji Noradrenaline infusion Incorrect answer selected
nmlkji nmlkj knee-chest position. Correct
nmlkji nmlkj Morphine 0.1mg/kg. Correct
nmlkj nmlkji Prostaglandin E2 50mg/kg/min. Correct
nmlkji nmlkj Propranolol 0.1mg/kg IV. Correct
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 1 of 10 / Overall score: 20%
In the fetal circulation
True / False
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.
Copyright 2002 Colin Melville
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nmlkj nmlkji Approximately equal volumes of blood are pumped by the left and right ventricles.
Incorrect answer selected
nmlkj nmlkji The saturation of blood returning from the placenta is about 80%. Incorrect answer selected
nmlkji nmlkj Approximately 25% of cardiac output traverses the fetal lung. Incorrect answer selected
nmlkji nmlkj The ductus venosus is probe-patent in 75% of cases. Incorrect answer selected
nmlkji nmlkj Pulmonary vascular resistance is suprasystemic. Correct
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 19 of 40 / Overall score: 64%
The following lesions can be diagnosed antenatally on a routine 4 chamber view at 18 weeks gestation:
True / False
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.
Copyright 2002 Colin Melville
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nmlkji nmlkj Tetralogy of Fallot. Incorrect answer selected
nmlkji nmlkj Hypoplastic left heart syndrome. Correct
nmlkj nmlkji VSD. Correct
nmlkj nmlkji Pulmonary stenosis. Correct
nmlkji nmlkj Transposition of the great arteries. Incorrect answer selected
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 18 of 40 / Overall score: 64%
An infant is noted at postnatal examination to have a slow pulse rate. ECG confirms congenital complete heart block. The following statements are true:
True / False
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.
Copyright 2002 Colin Melville
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nmlkji nmlkj Echocardiography is usually normal. Correct
nmlkji nmlkj It is usually associated with a presence of anti-Ro antibodies in the mother. Correct
nmlkj nmlkji Heart failure is uncommon. Incorrect answer selected
nmlkji nmlkj Mother's thyroid function should be checked. Incorrect answer selected
nmlkji nmlkj The underlying defect may be due to prolonged QT Syndrome. Incorrect answer selected
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 17 of 40 / Overall score: 66%
The following are features of aortic coarctation in infancy:
True / False
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.
Copyright 2002 Colin Melville
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nmlkj nmlkji Rib-notching. Correct
nmlkji nmlkj Inverted T wave in V6. Correct
nmlkji nmlkj Ejection systolic murmur between the shoulder blades. Incorrect answer selected
nmlkji nmlkj Radio-femoral delay. Correct
nmlkj nmlkji Plateau pulses. Correct
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Work smart: MRCPCH Part 1 B
Question: 16 of 40 / Overall score: 65%
The following are recognised features of cardiac failure in the infant:
True / False
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.
Copyright 2002 Colin Melville
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nmlkj nmlkji Jaundice. Correct
nmlkj nmlkji Splenomegaly. Incorrect answer selected
nmlkj nmlkji Clinically detectable elevation jugular venous pulse. Correct
nmlkji nmlkj Pallor. Correct
nmlkji nmlkj Grunting. Correct
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 15 of 40 / Overall score: 64%
Interventional catheterisation can be used to repair the following defects:
True / False
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.
Copyright 2002 Colin Melville
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nmlkji nmlkj Ostium primum ASD. Incorrect answer selected
nmlkji nmlkj Ostium secundum ASD. Correct
nmlkj nmlkji Muscular VSD. Correct
nmlkji nmlkj PDA. Correct
nmlkji nmlkj Dysplastic pulmonary valve. Incorrect answer selected
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 14 of 40 / Overall score: 64%
Myocarditis is a recognised association of:
True / False
A, B, C, D Hurler's is associated with a cardiomyopathy.
Copyright 2002 Colin Melville
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nmlkji nmlkj Salmonella infection. Correct
nmlkj nmlkji Mycoplasma infection. Incorrect answer selected
nmlkj nmlkji Lyme Disease. Incorrect answer selected
nmlkji nmlkj Measles. Correct
nmlkji nmlkj Hurler's Syndrome. Incorrect answer selected
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 13 of 40 / Overall score: 66%
Recognised features of a large uncomplicated patent ductus arteriosus include:
True / False
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.
Copyright 2002 Colin Melville
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nmlkj nmlkji Narrow pulse pressure. Correct
nmlkj nmlkji Pulmonary plethora. Incorrect answer selected
nmlkj nmlkji Decreased noise of murmur on squatting. Correct
nmlkj nmlkji Left ventricular hypertrophy on ECG. Incorrect answer selected
nmlkji nmlkj Soft pulmonary second heart sound. Incorrect answer selected
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 9 of 10 / Overall score: 87%
Do the following suggest pulmonary rather than aortic
valve stenosis?
True / False
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.
Copyright 2002 Colin Melville
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nmlkji nmlkj A deep S wave in V2 Incorrect answer selected
nmlkji nmlkj An ejection click best heard in the second and third left intercostal spaces Correct
nmlkj nmlkji An ejection murmur radiating to the neck Correct
nmlkj nmlkji An upright T wave in V1 Incorrect answer selected
nmlkji nmlkj Prominence in the left upper mediastinum on chest x ray Incorrect answer selected
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 12 of 40 / Overall score: 68%
The following are indications for immediate transfer to a tertiary cardiology centre in a neonate:
True / False
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.
Copyright 2002 Colin Melville
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nmlkji nmlkj Suspected large VSD. Incorrect answer selected
nmlkj nmlkji Suspected ASD. Correct
nmlkj nmlkji A diagnosis of Down's Syndrome. Correct
nmlkji nmlkj A diagnosis of Kawasaki Disease. Incorrect answer selected
nmlkji nmlkj Suspected aortic coarctation. Correct
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 11 of 40 / Overall score: 69%
Broad complex tachycardias:
True / False
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.
Copyright 2002 Colin Melville
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nmlkji nmlkj May be associated with shock. Correct
nmlkji nmlkj May be associated with pulselessness Correct
nmlkji nmlkj In the shocked patient are usually treated with synchronous DC shock. Correct
nmlkj nmlkji Are uncommon in childhood. Incorrect answer selected
nmlkj nmlkji May be due to supraventricular tachycardia. Incorrect answer selected
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Work smart: MRCPCH Part 1 B
Question: 10 of 40 / Overall score: 70%
Regarding infective endocarditis:
True / False
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.
Copyright 2002 Colin Melville
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nmlkj nmlkji The commonest causative organism is Group A Streptococcus. Correct
nmlkji nmlkj Lesions are most frequent in areas of high velocity blood flow. Correct
nmlkji nmlkj Amoxicillin is first choice for prophylaxis in procedures done under local anaesthetic.
Correct
nmlkj nmlkji Presentation in infancy is extremely rare. Incorrect answer selected
nmlkj nmlkji Splenomegaly is a subtle early sign. Correct
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Work smart: MRCPCH Part 1 B
Question: 9 of 40 / Overall score: 69%
The following findings suggest that a murmur is innocent:
True / False
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.
Copyright 2002 Colin Melville
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nmlkj nmlkji It is grade 3/6 Correct
nmlkji nmlkj It has a vibratory quality. Correct
nmlkj nmlkji It is loudest at the apex. Correct
nmlkj nmlkji It is only heard when the patient is febrile. Incorrect answer selected
nmlkji nmlkj It changes in intensity with posture. Correct
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Work smart: MRCPCH Part 1 B
Question: 8 of 40 / Overall score: 68%
The following are recognised causes of cyanosis in the newborn:
True / False
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.
Copyright 2002 Colin Melville
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nmlkji nmlkj Persistent fetal circulation. Correct
nmlkj nmlkji Severe anaemia. Correct
nmlkj nmlkji Congenital pneumonia. Incorrect answer selected
nmlkj nmlkji Transient tachypnoea of the newborn. Incorrect answer selected
nmlkji nmlkj Hypoplastic left heart syndrome. Correct
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Work smart: MRCPCH Part 1 B
Question: 7 of 40 / Overall score: 69%
Nitric oxide (endothelial relaxation factor):
True / False
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.
Copyright 2002 Colin Melville
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nmlkj nmlkji Unlike prostacyclin, promotes platelet aggregation. Correct
nmlkji nmlkj Can be enzymatically synthesised from glyceryl trinitrate. Correct
nmlkj nmlkji Has a half life of 1-2 minutes in vivo. Correct
nmlkji nmlkj Can be synthesised by macrophages. Correct
nmlkji nmlkj Is synthesised from L-arginine. Correct
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Work smart: MRCPCH Part 1 B
Question: 6 of 40 / Overall score: 63%
Paradoxical (reverse) splitting of the second heart sound is typical of:
True / False
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.
Copyright 2002 Colin Melville
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nmlkj nmlkji Ostium primum ASD. Correct
nmlkj nmlkji Pulmonary stenosis. Correct
nmlkji nmlkj Aortic stenosis. Correct
nmlkji nmlkj Left bundle branch block. Correct
nmlkj nmlkji Ostium secundum ASD. Correct
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Work smart: MRCPCH Part 1 B
Question: 5 of 40 / Overall score: 56%
Diastolic dysfunction of the left ventricle may be a dominant feature of:
True / False
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.
Copyright 2002 Colin Melville
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nmlkji nmlkj Hypertrophic obstructive cardiomyopathy. Correct
nmlkji nmlkj Endocardial fibroelastosis. Correct
nmlkj nmlkji Doxorubicin toxicity. Incorrect answer selected
nmlkji nmlkj Anomalous left coronary artery. Correct
nmlkj nmlkji Myocardial infarction after Kawasaki Disease. Incorrect answer selected
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 4 of 40 / Overall score: 55%
Regarding supraventricular tachycardias:
True / False
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.
Copyright 2002 Colin Melville
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nmlkj nmlkji Rates of 120-180 beats per minute are typical in adolescence. Correct
nmlkj nmlkji They are usually associated with structural cardiac defects. Correct
nmlkj nmlkji Lown-Ganong-Levine Syndrome is the most commonest cause. Correct
nmlkji nmlkj J waves may be seen. Incorrect answer selected
nmlkj nmlkji DC shock is most commonly required. Correct
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 3 of 40 / Overall score: 47%
Regarding systemic hypertension in childhood:
True / False
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.
Copyright 2002 Colin Melville
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nmlkj nmlkji Sodium nitroprusside is useful for the long-term treatment of severe cases. Correct
nmlkj nmlkji Headache is the usual presenting feature. Correct
nmlkj nmlkji It is defined as systolic blood pressure above the 99th centile for age. Correct
nmlkji nmlkj Abnormalities are frequently seen on DMSA scan. Correct
nmlkji nmlkj Aortic coarctation is the commonest secondary cause. Incorrect answer selected
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 9 of 10 / Overall score: 72%
A 3 month old girl presents with apnoea. She had been well
that morning, but had become unsettled, crying
inconsolably and gradually more mottled. Mother was
bringing her to A and E when she stopped breating. She
responded to physical stimulation. She was born at
40+3/40 weighing 3.6kg and there were no neonatal
problems.
On examination she has a temperature of 36.3°C
(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)
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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nmlkj Acute life-threatening event
nmlkji Cardiac dysrhythmias Correct
nmlkj Seizures
nmlkj Sudden infant death syndrome
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 8 of 10 / Overall score: 68%
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?
(Please select 1 option)
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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nmlkj Blood flow in the lower body
nmlkj Blood flow in the upper body
nmlkj Blood pressure in the upper limbs
nmlkj Vascular resistance in the lower limbs This is the correct answer
nmlkji Vascular resistance in the upper limbs Incorrect answer selected
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Work smart: MRCPCH Part 1 B
Question: 7 of 10 / Overall score: 78%
Theme: Genetics - cardiac abnormalities in genetic disorders
Match each of the following cardiovascular abnormalities to the single most likely associated genetic disorder.
Dilation of the aorta with aneurysms.
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.
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.
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.
A Angelman's syndrome
B Beckwith-Wiedemann syndrome
C Congenital Rubella syndrome
D Down syndrome
E Foetal alcohol syndrome
F Glycogen storage disease
G Marfan's syndrome
H Noonan's syndrome
I Turner's syndrome
J Williams syndrome
66Angelman's syndrome
66Williams syndrome
66Noonan's syndrome
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Work smart: MRCPCH Part 1 B
Question: 6 of 10 / Overall score: 80%
Theme: Congenital cardiac defects
Match each of the following cardiovascular abnormalities to the single most likely associated disorder.
Endocardial cushion defect.
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.
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.
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.
A Angelman's syndrome
B Beckwith-Wiedemann syndrome
C Congenital Rubella syndrome
D Down syndrome
E Foetal alcohol syndrome
F Glycogen storage disease
G Marfan's syndrome
H Noonan's syndrome
I Turner's syndrome
J Williams syndrome
66Angelman's syndrome
66Turner's syndrome
66Down syndrome
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Work smart: MRCPCH Part 1 B
Question: 5 of 10 / Overall score: 89%
Theme: Emergency medicine
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.
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.
Correct
A Adenosine
B Adrenaline
C Atropine
D DC shock
E Dobutamine
F Diving reflex
G Endotracheal intubation
H Intraosseous line
I IV Morphine
J Naloxone
66Endotracheal intubation
66DC shock
66Intraosseous line
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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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Work smart: MRCPCH Part 1 B
Question: 10 of 10 / Overall score: 81%
Theme: Emergency treatments
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.
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.
Incorrect - The correct answer is Adrenaline
A Adenosine
B Adrenaline
C Atropine
D DC shock
E Dobutamine
F Diving reflex
G Endotracheal intubation
H Intraosseous line
I IV morphine
J Naloxone
66Endotracheal intubation
66DC shock
66Atropine
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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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Work smart: MRCPCH Part 1 B
Question: 9 of 10 / Overall score: 74%
A 3 month old girl presents with liver enlargement of 4 cm.
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
to date. No family or social history of note.
On examination:
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.
What is the most likely diagnosis?
(Please select 1 option)
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 atrio-
ventricular 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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Temperature 36.5C
Respiratory rate 45/min (mild recession),
Pulse 140/min
nmlkj Aortic coarctation
nmlkj Atrial septal defect, primum This is the correct answer
nmlkj Atrial septal defect, secundum
nmlkj Hypoplastic left heart syndrome
nmlkji Ventricular septal defect Incorrect answer selected
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 8 of 10 / Overall score: 83%
An 8-year-old girl presents with a 2 week history of
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
deteriorating over the past term. Full term normal delivery
with no neonatal complications. Immunisations up to date.
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.
What is the most likely diagnosis?
(Please select 1 option)
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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nmlkj Cerebral abscess
nmlkji Medulloblastoma Correct
nmlkj Migraine
nmlkj Sinusitis
nmlkj Tension headache
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Work smart: MRCPCH Part 1 B
Question: 8 of 10 / Overall score: 92%
A 4-month-old boy presents with heart murmur,
breathlessness and failure to thrive.
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
neonatal problems. Immunisations are up to date. There is
no family or social history of note.
On examination the temperature is 36.9°C, respiratory rate
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.
What is the most likely diagnosis?
(Please select 1 option)
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
nmlkj Atrial septal defect, primum
nmlkj Atrial septal defect, secundum
nmlkj Mitral regurgitation
nmlkj Tricuspid atresia
nmlkji Ventricular septal defect (VSD) Correct
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endocarditis.
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 6 of 10 / Overall score: 78%
A 14-year-old boy falls awkwardly while swinging from a
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
delivery, no neonatal problems. Immunisations up to date.
No family or social history of note.
On examination the temperature is 36.5°C. He has been
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?
(Please select 1 option)
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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nmlkj Anaemic
nmlkj Cardiogenic
nmlkji Distributive Correct
nmlkj Hypovolaemic
nmlkj Septic
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Work smart: MRCPCH Part 1 B
Question: 5 of 10 / Overall score: 88%
A 2-week-old girl presents with a history of poor feeding,
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.
On examination temperature is 35.4°C (tympanic), RR
60/min, HR 160/min on monitor (all pulses impalpable).
Cold mottled peripheries, capillary refill time six seconds.
Oxygen saturations 45% in air. Gallop rhythm, 4 cm
hepatomegaly, no spleen. No bruising or rash.
What is the most likely diagnosis?
(Please select 1 option)
The history is of shock, hepatomegaly and hypoxia in a 2-
week-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.
nmlkj Congenital adrenal hyperplasia
nmlkj Escherichia coli septicaemia
nmlkj Galactosaemia
nmlkj Group B streptococcal infection
nmlkji Hypoplastic left heart syndrome Correct
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Urgent transfer to a cardiac centre should then follow so a
specific diagnosis can be made.
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Work smart: MRCPCH Part 1 B
Question: 3 of 10 / Overall score: 78%
Theme: Syncope.
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.
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 Romano-
Ward 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.
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.
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.
A Arrhythmia
B Breath-holding, blue
C Breath-holding, white
D Hypertrophic cardiomyopathy
E Hypoglycaemia
F Hypotention, vasovagal
G Hypotension, orthostatic
H Seizure
I Tumour, brain
66Arrhythmia
66Seizure
66Tumour, brain
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 1 of 10 / Overall score: 100%
An 16-year-old female is found to be hypertensive at 26
weeks of her first pregnancy.
What is the most appropriate management?
(Please select 1 option)
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.
Rate this question
Blood pressure 144/92 mmHg
Dipstick urine +++ proteinuria
Serum creatinine 80 µmol/L
24 hour urine collection 1.2 g of protein/d
nmlkj Atenolol
nmlkji Methyldopa Correct
nmlkj Nifedipine
nmlkj Ramipril
nmlkj Re-measure blood pressure in one week
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Work smart: MRCPCH Part 1 B
Question: 3 of 10 / Overall score: 80%
A 17-year-old boy whose brother had hypertrophic
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?
(Please select 1 option)
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.
Rate this question
nmlkj A gradient of 10 mmHg across the left ventricular outflow tract
nmlkji Significant thickening of the interventricular septum Correct
nmlkj An enlarged left atrium
nmlkj Systolic anterior motion of the mitral valve
nmlkj The presence of mitral regurgitation
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 9 of 10 / Overall score: 55%
The following are true of cardiac transplantation:
True / False
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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nmlkji nmlkj the donor heart is provided to the recipient with the best HLA match Incorrect answer selected
nmlkji nmlkj long term hypotension is a problem Incorrect answer selected
nmlkji nmlkj recipients suffer less angina but accelerated coronary artery disease This is the correct answer
nmlkj nmlkji the histology of atheroma in the transplanted heart is the same as that of typical atherosclerosis Correct
nmlkji nmlkj 75% 5 year survival Correct
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 8 of 10 / Overall score: 57%
Cerebral abscess in the absence of endocarditis is a recognised complication of:
True / False
This is paradoxical embolization seen in right to left shunts. It
is not associated with left to right shunts except if
Eisenmenger's supervenes.
Rate this question
nmlkji nmlkj tetralogy of Fallot Correct
nmlkj nmlkji persistent ductus arteriosus Correct
nmlkji nmlkj tricuspid artresia Correct
nmlkji nmlkj transposition of the great arteries Correct
nmlkj nmlkji atrial septal defect of the ostium primum type Correct
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 7 of 10 / Overall score: 50%
A short PR interval on the ECG is associated with:
True / False
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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nmlkj nmlkji Duchenne muscular dystrophy Incorrect answer selected
nmlkj nmlkji dystrophia myotonica Correct
nmlkji nmlkj Friedrich's ataxia Correct
nmlkji nmlkj rheumatic carditis Incorrect answer selected
nmlkj nmlkji hypertrophic cardiomyopathy Incorrect answer selected
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 5 of 10 / Overall score: 56%
The following are recognised causes of pericardial disease:
True / False
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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nmlkji nmlkj Hepatitis B virus Correct
nmlkj nmlkji Mulibrey nanism Incorrect answer selected
nmlkji nmlkj rheumatic fever Correct
nmlkj nmlkji hypereosinophilic syndrome Incorrect answer selected
nmlkji nmlkj ankylosing spondylitis Incorrect answer selected
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Work smart: MRCPCH Part 1 B
Question: 2 of 10 / Overall score: 70%
Theme: Syndromes associated with congenital heart disease
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
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
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.
A Charcot-Marie-Tooth syndrome
B Down syndrome
C Fragile X syndrome
D Marfan's syndrome
E Noonan syndrome
F Sturge-Weber syndrome
G Tetrology of Fallot
H Tourette syndrome
I Turner's syndrome
J William's syndrome
66Sturge-Weber syndrome
66Turner's syndrome
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However they have normal intellectual development.
Dilatation of aortic root/aortic regurgitation
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)
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
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,
66Marfan's syndrome
66William's syndrome
66Noonan syndrome
© 2010 BMJ Publishing Group Ltd . All rights reserved . Terms and
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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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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 2 of 40 / Overall score: 30%
The following regarding Atrial Natriuretic Peptide are correct
True / False
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.
Rate this question
nmlkji nmlkj It is secreted in response to right atrial distension Correct
nmlkji nmlkj It inhibits aldosterone release Correct
nmlkji nmlkj It is a direct inotrope Incorrect answer selected
nmlkj nmlkji It is a direct vasodilator Incorrect answer selected
nmlkji nmlkj It is inactivated by endopeptidase Correct
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 1 of 40 / Overall score: 0%
Which one of the following is a recognised feature of abetalipoproteinaemia?
(Please select 1 option)
Acanthocytes are seen in abetalipoproteinaemia.
Retinitis pigmentosa is seen in abetalipoproteinaemia.
Neurodegenerative changes are seen such as ataxia but IQ is
normal.
Rate this question
nmlkji a high serum cholesterol Incorrect answer selected
nmlkj palmar xanthomas
nmlkj advanced atherosclerotic vascular disease
nmlkj abnormal red blood cell morphology This is the correct answer
nmlkj Severe mental retardation
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 10 of 10 / Overall score: 65%
A post-marketing surveillance study of a new heart failure
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
such a study?
(Please select 1 option)
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.
Rate this question
nmlkj Adverse events profile This is the correct answer
nmlkj Cost benefit analysis
nmlkji Cost effectiveness Incorrect answer selected
nmlkj Comparative therapeutic efficacy
nmlkj Drug potency
Finish
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Work smart: MRCPCH Part 1 B
Question: 6 of 10 / Overall score: 52%
Theme: Congenital heart disease
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.
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
A Ostium secundum atrial septal defect
B Ventricular septal defect
C Transposition of the great arteries
D Total anomalous pulmonary venous drainage
E Atrioventricular septal defect
F Patent ductus arteriosus
G Pulmonary valve stenosis
H Coarctation of the aorta
I Tetralogy of Fallot
J Hypoplastic left heart syndrome
66Ventricular septal defect
66Coarctation of the aorta
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and decreased pulmonary vascular markings.
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.
Rate this question
66Tetralogy of Fallot
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 1 of 10 / Overall score: 100%
A 16-year-old boy is admitted after a blackout at the
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
a minute or so and was oriented but nauseous.There were
no similar episodes in the past and he is totally unaware of
what happened.
Examination was normal and his ECG was normal. Which
one of the following is the most likely diagnosis?
(Please select 1 option)
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.
Rate this question
nmlkj Complex partial seizure
nmlkj Pseudoseizure
nmlkj Stokes-Adams attack
nmlkj Tonic-clonic seizure
nmlkji Vasovagal syncope Correct
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 4 of 10 / Overall score: 95%
A 16-year-old male is brought to emergency admissions
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
normal. What is the most appropriate management for this
patient?
(Please select 1 option)
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.
Rate this question
nmlkj Aspirin for 3 months
nmlkj Bisoprolol for 3 months
nmlkji Lifestyle advice Correct
nmlkj Sotalol for one month
nmlkj Warfarin for one month
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Work smart: MRCPCH Part 1 B
Question: 3 of 10 / Overall score: 93%
A 16-year-old male presents with acute severe asthma. On
examination his peripheral pulse volume fell during
inspiration.
Which one of the following is the most likely explanation for
this clinical sign?
(Please select 1 option)
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.
Rate this question
nmlkj The cardiac effect of high dose beta agonist bronchodilator drugs
nmlkj A falling heart rate on inspiration
nmlkj Myocardial depression due to hypoxia
nmlkj Peripheral vasodilatation
nmlkji Reduced left atrial filling pressure on inspiration Correct
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Work smart: MRCPCH Part 1 B
Question: 2 of 10 / Overall score: 90%
In disorders of cardiac conduction:
True / False
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.
nmlkji nmlkj right bundle branch block and left axis deviation indicate bifascicular block Correct
nmlkj nmlkji left anterior hemiblock causes right axis deviation Correct
nmlkji nmlkj left posterior hemiblock causes left axis deviation Incorrect answer selected
nmlkji nmlkj deep S waves are found in leads I and V6 in right bundle branch block Correct
nmlkji nmlkj right ventricular hypertrophy cannot be diagnosed in the presence of left bundle branch block Correct
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 4 of 10 / Overall score: 85%
A 3-month-old girl presents with apnoea. She had been
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
stimulation. She was born at 40+3/40 weighing 3.6kg and
there were no neonatal problems.
On examination she has a temperature of 36.3C
(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)
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.
Rate this question
nmlkj Acute life-threatening event
nmlkj Bronchiolitis
nmlkj Gastro-oesophageal reflux
nmlkj Seizure
nmlkji Supraventricular tachycardia Correct
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 10 of 10 / Overall score: 77%
A 4-year-old girl presents with heart murmur and
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
complications. Immunisations up to date. There is no
FH/SH of note.
On examination:
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.
What is the most likely diagnosis?
(Please select 1 option)
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.
Rate this question
Respiratory rate 25/min
pulse 130/min
BP 80/60 mmHg
nmlkj Aortic coarctation
nmlkj Atrioventricular canal defect
nmlkj Kawasaki disease
nmlkji Myocarditis Correct
nmlkj Ventricular septal defect
Finish
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Work smart: MRCPCH Part 1 B
Question: 6 of 10 / Overall score: 90%
A 3-year-old boy is referred with heart murmur. He has
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
FH/SH of note.
On examination he is well and apyrexial. There are no
dysmorphic features. Respiratory rate is 17/min and pulse
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.
What is the most likely diagnosis?
(Please select 1 option)
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.
Rate this question
nmlkj Aortic coarctation
nmlkj Atrial septal defect, primum
nmlkji Atrial septal defect, secundum Correct
nmlkj Innocent murmur
nmlkj Pulmonary stenosis
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Work smart: MRCPCH Part 1 B
Question: 7 of 10 / Overall score: 81%
A 3-year-old girl is referred with incidentally-discovered
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.
On examination she is apyrexial, well and well grown with
no dysmorphic features. Respiratory rate is 20/min and
pulse is 100/min. Heart sounds are normal. She has a 1-
2/6 murmur best heard under the right clavicle, which
changes with posture. The liver is impalpable.
What is the most likely diagnosis?
(Please select 1 option)
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.
Rate this question
nmlkj Aortic stenosis
nmlkj Atrial septal defect, secundum
nmlkj Atrioventricular canal defect
nmlkji Innocent murmur Correct
nmlkj Pulmonary stenosis
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Work smart Work hard Mock exams Revision advice
Work smart: MRCPCH Part 1 B
Question: 10 of 10 / Overall score: 59%
An 11-day-old baby presents with poor feeding and
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.
On examination she is responding to pain, mottled and had
a tympanic temperature of 34.6°C. Her heart rate is
130/min with impalpable pulses and gallop rhythm. Her
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?
(Please select 1 option)
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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nmlkj Anaemic
nmlkji Cardiogenic Correct
nmlkj Distributive
nmlkj Hypovolaemic
nmlkj Septic
Finish
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Work smart: MRCPCH Part 1 B
Question: 5 of 10 / Overall score: 73%
Theme: Chest pain in children.
For each scenario choose the most likely diagnosis:
A 13-year-old girl presents with chest pain. On examination there is pain and tenderness of the 8th right costochondral cartilage.
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.
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.
Correct
The 14-year-old girl is hyperventilating. The finger
paraesthesiae amy represent reduced ionised calcium
associated with a respiratory alkalosis. The symptoms should
A Asthma
B Costochondritis
C Herpes zoster
D Hypertrophic cardiomyopathy
E Hyperventilation
F Mitral valve prolapse
G Musculoskeletal pain
H Pericarditis
I Psychogenic
66Pericarditis
66Musculoskeletal pain
66Hyperventilation
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resolve with re-breathing into a paper bag.
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Work smart: MRCPCH Part 1 B
Question: 4 of 10 / Overall score: 83%
Theme: Palpitations in children.
For each scenario choose the most likely diagnosis:
A 14-year-old girl complains of awareness of heart beat. She began menstruating at 12 years, and has heavy periods. She appears pale.
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.
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
A Anaemia
B Cardiomyopathy
C Hyperthyroidism
D Myocarditis
E Panic attacks
F Prolonged QT syndrome
G Sinus arrhythmia
H Sinus tachycardia
I Supraventricular tachycardia
66Anaemia
66Hyperthyroidism
66Supraventricular tachycardia
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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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Work smart: MRCPCH Part 1 B
Question: 2 of 10 / Overall score: 83%
Theme: Apnoea
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 high-pitched cry and is difficult to settle.
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.
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.
A Blood cultures
B CSF culture
C CT scan head
D Drug screen on mother
E EEG
F Nasopharyngeal aspirate for viruses
G Nasopharyngeal aspirate for pertussis
H pH studies
I Urine metabolic screen
66CSF culture
66Blood cultures
66Urine 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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