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(1)

Recognised toxic effects of Amiodarone include:

True / False

Amiodarone is a potent anti-arrhythmic useful in all forms of dysrhythmias. Caution is required in renal impairment and there is a risk of thyroid dysfunction with accumulation of iodine. It enhances the effects of Warfarin and increased Digoxin, Phenytoin and Cyclosporin levels. There is an increased risk of bradycardia and AV block and myocardial depression with betablockers and calcium channel blockers. Toxicity is increased if hypokalaemia occurs with diuretics. Reversible corneal microdeposits, optic neuritis, peripheral neuropathy and myopathy, bradycardia and conduction disturbances, phototoxicity, and rarely a persistent slate grey discolouration, hypo and hyperthyroidism, diffuse pulmonary alveolitis in pneumonitis and fibrosis, disturbed liver function tests, jaundice, hepatitis and cirrhosis are all reported.

n m l k j i nmlkj Hyperthyroidism Correct n m l k j

i nmlkj Peripheral neuropathy Correct n m l k j i nmlkj Hepatitis Correct n m l k

j nmlkji Hypothyroidism Incorrect answer selected

n m l k j i nmlkj Photosensitivity Correct

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Work Smart Session - MRCPCH Part 1 A / DCH

An 18 month old girl presents with stridor at 1 am. She has had a cold for 48 hours, with low-grade fever, but went to bed as usual at 7.30pm. She awoke 4 hours later crying and distressed, with a barking cough. What is the most likely diagnosis?

(Please select 1 option)

Classical history of viral croup. Most of these episodes are one-off and settle rapidly without treatment or with dexamethasone orally. n m l k j Allergic rhinitis n m l k j Asthma n m l k

j Croup This is the correct answer n

m l k j

i Gastroesophageal reflux Incorrect answer selected

n

m l k

j Sinusitis

Question: 48 of 50

Time taken: 55:56

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Work Smart Session - MRCPCH Part 1 A / DCH

Which one of the following is/are true of sudden infant death syndrome?

True / False

SIDS is the commonest cause of death in this age group. Post mortem fails to reveal a cause of death.

There is a twice normal risk of SIDS in siblings and low birth weight/premature infants.

n

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i nmlkj It is more common in low birth weight infants. Correct n

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i nmlkj It is more common in low socio-economic groups. Correct n

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j nmlkji It is the commonest cause of death in infants aged 1-2 months in this country. Incorrect answer selected

n

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j nmlkji Post mortem shows asphyxia in over 80% of cases. Correct n

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j nmlkji There is a tenfold increased risk of sudden infant death to siblings of children with SIDS. Correct

Question: 46 of 50

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Work Smart Session - MRCPCH Part 1 A / DCH

A 9-year-old girl presents with throat discomfort and cough, particularly during the daytime. She has been well over the winter, with symptoms worsening in the Spring. 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 well. She is constantly clearing her throat, and has a clear nasal discharge. She has no cervical lymphadenopathy. Her throat is minimally injected, and ear examination is normal.

Select themost likely diagnosis? (Please select 1 option)

The history is of nasal discharge and cough, worse since Spring. This points to allergens rather than infection as the underlying cause. A postnasal drip is the likely culprit for these symptoms, which may also disturb sleep because of cough. Treatment of the underlying problem with antihistamines and topical steroids is indicated.

i Asthma Incorrect answer selected

n m l k j Foreign body n m l k j Infectious mononucleosis n m l k j Paratonsillar abscess n m l k

j Postnasal drip This is the correct answer

Question: 40 of 50

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Work Smart Session - MRCPCH Part 1 A / DCH

In ventricular septal defect (VSD) which of the following statements is/are true?

Large defects may be associated with soft murmurs; right to left shunting causes cerebral abscesses.

Diastolic murmur occurs due to aortic incompetence (5%) or increased flow across the mitral valve and relative mitral stenosis.

Although pulmonary hypertension may occur in association with increased flow across the shunt, it may indicate decreased flow across the shunt and increased pulmonary vascular resistance in which case the murmur would be softer.

The risk of bacterial endocarditis is high in this lesion and even greater with haemodynamically trivial lesions.

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j Left-to-right shunting leads to increased risk of cerebral abscess

n

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j The smallest defects tend to produce the softest murmurs

n

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j The systolic murmur increases in intensity as pulmonary hypertension develops

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j There is no risk of bacterial endocarditis

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i There may be a diastolic murmur at the apex Correct

Question: 37 of 50

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Work Smart Session - MRCPCH Part 1 A / DCH

A small ventricular septal defect is associated with:

True / False

A small ventricular septal defect is classically associated with the holosystolic murmur at the left sternal edge. The risk of infective endocarditis is greater in haemodynamically insignificant defects. The second heart sound may be obscured by the murmur, but is normally split. Cyanosis occurs if there is Eisenmengers syndrome. A mid diastolic murmur at the apex is associated with a moderate sized shunt and increased flow through the left atrium. n m l k j

i nmlkj A pansystolic murmur heard best at the lower left sternal edge Correct n

m l k j

i nmlkj A high risk of infective endocarditis Correct n

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j nmlkji Fixed splitting of the second sound Correct n m l k j nmlkji Cyanosis Correct n m l k j

i nmlkj A mid-diastolic murmur at the apex Incorrect answer selected

Question: 33 of 50

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Work Smart Session - MRCPCH Part 1 A / DCH

In the management of congenital heart disease:

True / False

Prophylactic antibiotics are only required prior to dental procedures, surgery and other invasive procedures. If spontaneous closure of a patent ductus arteriosus does not occur, closure is recommended between six months to one year. This can be done surgically, or, more often these days, through the trans-catheter route. Atrial septal defect may spontaneously close usually if less than 8 mm in size.

Oedema and ascites and raised jugular venous pressure are uncommon signs of heart failure in infancy. Squatting is a typical occurrence in an older child with tetralogy of Fallot.

Further reading:

Congenital Heart Defects MedlinePlus

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j nmlkji Patients with ventricular septal defects require daily prophylactic ampicillin to prevent bacterial endocarditis Correct n m l k j

i nmlkj Ductus arteriosus that does not close by six months old will require surgical ligation Correct n

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j nmlkji All atrial septal defects have to be closed surgically Correct n

m l k j

i nmlkj Peripheral oedema is an uncommon finding in infants with heart failure Correct n

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j nmlkji Squatting is a typical description in patients with transposition of the great vessels Correct

Question: 31 of 50

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Work Smart Session - MRCPCH Part 1 A / DCH

Cyanosis is a typical feature of which of the following conditions:

TAPVC is associated with cyanosis in the newborn. Total anomalous pulmonary venous connection (TAPVC) consists of an abnormality of blood flow in which all 4 pulmonary veins drain into systemic veins or the right atrium with or without pulmonary venous obstruction. Systemic and pulmonary venous blood mix in the right atrium. PDA, ASD and VSD are left to right shunts. Tricuspid atresia is typically associated with cyanosis rather than Mitral.

n

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j Patent ductus arteriosus. n

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j Ventricular septal defect. n

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i Total anomalous pulmonary venous drainage. Correct n

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j Atrial septal defect n

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j Mitral atresia.

Question: 26 of 50

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Work Smart Session - MRCPCH Part 1 A / DCH

An opening snap is found in:

True / False

d-10% of cases secondary to increasing left atrial pressure. Soft late snap - actually a 'tumour plop' that mimics the opening snap of Mitral Stenosis. (Wassermil M, Warkentin DL, Ravin A: Myxoma of the left atrium: Phonocardiographic study of three cases. Circulation 1962;25:50–56 )

Heart Sound reference n

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i nmlkj mitral stenosis due to rheumatic heart disease Correct n

m

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i nmlkj congenital mitral stenosis Correct n

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i nmlkj mitral incompetence associated with a rigid posterior valve leaflet but a normal anterior leaflet Correct n

m

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i nmlkj left atrial myxoma Correct n

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j nmlkji severe aortic incompetence Correct

Question: 22 of 50

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Work Smart Session - MRCPCH Part 1 A / DCH

Which ONE of the following statements is true about the Austin Flint murmur?

The Austin Flint murmur is a low frequency mid/late diastolic murmur which may show pre-systolic accentuation and is virtually indistinguishable from that of mitral stenosis. It is due to partial closure of the anterior leaflet of the mitral valve by the regurgitant jet. There is no correlation between presence of murmur and severity of AR, or aetiology. The first heart sound is normal but in severe cases, it may be absent.

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j It is associated with a loud first heart sound. n

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j It is an early sign of aortic regurgitation n

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j It can be distinguished from the murmur of mitral stenosis by absence of presystolic accentuation n

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i It is due to partial closure of the anterior leaflet of the mitral valve Correct n

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j It does not occur in aortic incompetence secondary to an aortitis

Question: 19 of 50

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Work Smart Session - MRCPCH Part 1 A / DCH

A left-to-right shunt is a feature in which of the following congenital heart defects:

True / False

The commonest examples of a left-to-right shunt are an atrial septal defect (ASD), ventricular septal defect (VSD) and patent ductus arteriosus (PDA). Children with this defect are usually not cyanosed (providing there is no left ventricular failure or reversal of the shunt).

Fallot’s tetralogy is the commonest form of a right-to-left shunt and the children are cyanosed. Eisenmenger’s syndrome occurs when there is reversal of the left-to-right shunt (to a right-to-left shunt), due to irreversible pulmonary vessel disease.

n

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j nmlkji Eisenmenger’s syndrome Correct n

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i nmlkj Patent ductus arteriosus Correct n

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i nmlkj Tetralogy of Fallot Incorrect answer selected n

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i nmlkj Atrial septal defect Correct n

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i nmlkj Ventricular septal defect Correct

Question: 17 of 50

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Work Smart Session - MRCPCH Part 1 A / DCH

In children with a ventricular septal defect:

True / False

VSDs are the commonest congenital heart defect with the vast majority being small often spontaneously closing and of no haemodynamic significance. Small residual VSDs may be picked up due to the loud murmur (Maladie de Roger) and VSDs are associated with increased pulmonary blood flow. Larger VSDs would be associated with biventricular hypertrophy associated with volume overload.

n

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i nmlkj it is the commonest congenital heart lesion Correct n

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i nmlkj closes spontaneously in up to 50% of cases Correct n

m

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j nmlkji of Maladie de Roger type is the most severe form Correct n

m

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j nmlkji is associated with pulmonary oligaemia Correct n

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i nmlkj with a large lesion is associated with biventricular hypertrophy Correct

Question: 13 of 50

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Work Smart Session - MRCPCH Part 1 A / DCH

Which of the following are true regarding randomised controlled trials in single patients (n of 1 trials)?

True / False

A. In an n of 1 trial the treatment and placebo are given in random treatment periods in the same patient. B. The results are specific to the one drug and one patient studied and cannot usually be generalised. C. Or where the practitioner has doubts. They are also useful for dosing or working out if a symptom is a side effect or not.

D. Drugs with short effects are best as long wash-out periods need to be included for long-acting drugs. E. The studies are with single patients on or off a drug and do not normally require ethics committee approval. (DTB 1998;36(5):40) n m l k j

i nmlkj A. Need to be repeated in many patients to achieve significant results Incorrect answer selected n

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i nmlkj B. Can guide treatment in other patients Incorrect answer selected n

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i nmlkj C. Are useful where the patient doubts the effectiveness of a treatment Correct n

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i nmlkj D. Have an advantage in studying drugs with long half lives Incorrect answer selected n

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j nmlkji E. Do not normally require approval from ethics committees Incorrect answer selected

Question: 11 of 50

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Work Smart Session - MRCPCH Part 1 A / DCH

A loud first heart sound may be due to:

True / False

A loud first heart sound is due to abrupt closure of the mitral valve against a high left atrial pressure. MR occurs with papillary muscle rupture and thereby 1st heart sound is soft.A2 and P2 are loud in systemic HT and pulmonary hypertension respectively. n m l k j

i nmlkj a long preceding diastolic interval Incorrect answer selected n

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i nmlkj mitral stenosis Correct n

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j nmlkji rupture of a papillary muscle This is the correct answer n

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i nmlkj increased systemic arterial pressure Incorrect answer selected n

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j nmlkji increrased pulmonary arterial pressure Correct

Question: 9 of 50

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Work Smart Session - MRCPCH Part 1 A / DCH

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.

Correct

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.

Correct

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 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.

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

6 6 Coarctation of the aorta

6 6 Pulmonary valve stenosis

6 6 Tetralogy of Fallot

Question: 7 of 50

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Work Smart Session - MRCPCH Part 1 A / DCH

A newborn baby, one of twins born at term, is noted to be centrally cyanosed soon after birth. Which of the following is the most likely cause?

VSD and PDA are left to right shunts; coarctation of the aorta is not associated with cyanosis. Tricuspid atresia and transposition of the great arteries are both cyanotic congenital heart diseases and present in the immediate newborn period. Of the 2 transposition of the great arteries is more common and hence more likely to be the cause.

n

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j Ventricular septal defect n

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i Transposition of great arteries Correct n

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j Patent ductus arteriosus n

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j Coarction of the aorta n

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j Tricuspid atresia

Question: 4 of 50

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