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1) In the diagnosis of rheumatic fever, which of the following may be helpful? Available marks are shown in brackets 1) 2) 3) 4) 5) A generalized macular-papular rash. ASO titre of less than 1:200. Polyarthritis. Staphylococcus aureus grown on throat culture. Splinter haemorrhages. [0] [0] [100] [0] [0]

Comments: A Jones criteria require two major or one major and two minor, and evidence of recent streptococcal infection for the diagnosis of rheumatic fever. MAJOR: 1) Pancarditis. 2) Polyarthritis 3) Erythema marginatum 4) Chorea 5) Subcutaneous nodules - The rash is macular. MINOR: 1) Fever 2) Polyarthralgia 3) History of RF 4) Raised ESR/CRP 5) Prolonged PR interval on ECG. 2) Cyanosis in the newborn may be caused by which of the following: Available marks are shown in brackets 1) 2) 3) 4) 5) Transposition of the great arteries. VSD Hyperbilirubinaemia. Coarctation of the aorta. Eisenmenger syndrome [100] [0] [0] [0] [0]

Comments: VSD is a left to right shunt, coarctation is a narrowing of the aortic arch and hyperbilirubinaemia is not associated with cyanosis. Eisenmengers syndrome though a cause of cyanosis develops much later following reversal of a left to right shunt. 3) A 1-year-old infant is known to have heart disease and is noted to be cyanosed. Which of the following is the most likely diagnosis? Available marks are shown in brackets [0] 1) Atrial septal defect 2) Coarctation of the aorta [0] 3) Patent Ductus Arteriosus [0] 4) Tricuspid atresia [100] 5) Ventricular septal defect [0] Comments: The patient is cyanosed and of the diseases described only tricuspid atresia is associated with cyanosis. Other causes are Fallots tetralogy, single ventricle and Transposition of the great vessels. Eisenmengers syndrome develops with the switch to right to left flow associated with deteriorating VSD etc.


4) An uncomplicated ventricular septal defect in a 5-year-old boy may be associated with which one of the following? Available marks are shown in brackets 1) 2) 3) 4) 5) A collapsing pulse. Wide and fixed splitting of the second heart sound. Clubbing of the fingers. A pansystolic murmur of grade 4/6 in intensity. Splenomegaly. [0] [0] [0] [100] [0]

Comments: A collapsing pulse may indicate associated aortic incompetence. The S2 splits normally in an uncomplicated VSD and P2 is normal. Clubbing is a feature of cyanotic congenital heart disease. A holo-systolic murmur of variable intensity is characteristic. Splenomegaly is not associated. 5) 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 Available marks are shown in brackets 1) 2) 3) 4) 5) Ventricular septal defect Transposition of great arteries Patent ductus arteriosus Coarction of the aorta Tricuspid atresia [0] [100] [0] [0] [0]

Comments: 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. 6) Plethoric lung fields are a common radiological feature of: Available marks are shown in brackets 1) 2) 3) 4) 5) A large ventricular septal defect Fallots tetralogy Coarctation of the aorta Epsteins anomaly Eisenmengers complex [100] [0] [0] [0] [0]

Comments: Coarctation is associated with normal pulmonary blood flow and other than a large VSD the rest are associated with reduced pulmonary blood flow.


7) A 14-year-old boy presents with hypertension. Which of the following statements concerning hypertension in the young is true? Available marks are shown in brackets 1) 2) 3) 4) 5) Sodium nitroprusside is useful for the long-term treatment of severe cases. Headache is the usual presenting feature. It is defined as systolic blood pressure above the 99th centile for age. Abnormalities are frequently seen on DMSA scan. Aortic coarctation is the commonest secondary cause. [0] [0] [0] [100] [0]

Comments: 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. 8) Which one of the following is a recognized feature of abeta-lipoproteinaemia? Available marks are shown in brackets 1) 2) 3) 4) 5) A high serum cholesterol Palmar xanthomas Advanced atherosclerotic vascular disease Abnormal red blood cell morphology Severe mental retardation [0] [0] [0] [100] [0]

Comments: Acanthocytes are seen in abetalipoproteinaemia.

Retinitis pigmentosa is seen in abeta-lipoproteinaemia. Neurodegenerative changes are seen such as ataxia but IQ is normal. ABETALIPOPROTEINAEMIA Rare recessive disorder inability to produce LDL chylomicron formation is defective Characteristic features to age 2

Steatorrhoea, failure to thrive Low serum lipids, acanthocytosis

Later childhood

Ataxia, intention tremor, nystagmus Athetosis, muscle weakness, dec. reflexes IQ normal but emotionally labile

Some develop retinitis pigmentosa


9) A baby is diagnosed with a ventricular septal defect. Which one of the following is true concerning a VSD? Available marks are shown in brackets 1) 2) 3) 4) 5) Is likely to cause heart failure in the first week of life. Is associated with plethoric lung fields on chest x-ray in a 10-week-old infant. Requires surgical correction in all but a small minority of cases. Requires surgical correction if central cyanosis occurs. Causes fixed splitting of the second heart sound. [0] [100] [0] [0] [0]

Comments: VSD is unlikely to present till after the first month of life and associated with pulmonary plethora. The majority of cases will resolve spontaneously. Central cyanosis indicates shunt reversal and pulmonary hypertension, which implies poor prognosis and response to operative repair of the VSD. The second heart sound is normally spilt. 10) In ventricular septal defect (VSD) Available marks are shown in brackets 1) 2) 3) 4) 5) The smallest defects tend to produce the softest murmurs Left-to-right shunting leads to increased risk of cerebral abscess There may be a diastolic murmur at the apex The systolic murmur increases in intensity as pulmonary hypertension develops There is no risk of bacterial endocarditis [0] [0] [100] [0] [0]

Comments: 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 mtral 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. 11) Which of the following concerning congenital heart disease is correct? Available marks are shown in brackets 1) 2) 3) 4) 5) ASD is the commonest malformation at birth Congenital complete heart block is usually associated with Anti-Ro antibodies in the mother Ebstein's anomaly is associated with maternal exposure to lithium carbonate Hypo plastic left heart syndrome is characterized by a large, dilated left ventricle Osteogenesis imperfecta is associated with aortic stenosis [0] [0] [100] [0] [0]

Comments: A VSD is the commonest at 30%, ASD is 10%. Aortic regurgitation may be a feature of Osteogenesis imperfecta. Ebstein's anomaly is associated with maternal LiCO3 use if exposed in the first trimester. In the vast majority of cases of neonates with complete heart block the cause is unknown but in the minority it may be caused by autoimmune disease, particularly Anti-Ro antibodies, in the mother. LV Hypolasia occurs when the left sided chambers fail to develop and blood enters the systemic circulation from the right ventricle via the pulmonary artery and a patent ductus arteriosus.


12) In an infant, which of the following make Cardiac failure unlikely? Available marks are shown in brackets 1) 2) 3) 4) 5) A respiratory rate of more than 30 per minute A heart rate of more than 180 per minute An enlarged liver Ascites Excessive perspiration [0] [0] [0] [100] [0]

Comments: Ascites is rare in cardiac failure in infancy. Other features such as bibasal crackles, raised JVP, third heart sound, pulsus alternans are common clinical features. 13) 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 36.3C (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? Available marks are shown in brackets 1) 2) 3) 4) Acute life-threatening event Cardiac dysrhythmias Seizures Sudden infant death syndrome [0] [100] [0] [0]

Comments: 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. 14) Which one of the following would lead you to consider a murmur to be innocent in a four and a half year old child? Available marks are shown in brackets 1) 2) 3) 4) 5) Pansystolic timing. Association with a thrill. Marked variation of loudness with change of posture. Radiation towards the left axilla. Association with fixed splitting of the second heart sound. [0] [0] [100] [0] [0]

Comments: Innocent murmurs are of short duration and vary with posture. A thrill implies significant turbulence and hence valvular disease. Radiation to the axilla implies mitral incompetence and fixed splitting implies an ASD.


15) Cyanosis is a typical feature of which of the following conditions: Available marks are shown in brackets 1) 2) 3) 4) 5) Patent ductus arteriosus. Ventricular septal defect. Total anomalous pulmonary venous drainage. Atrial septal defect Mitral atresia. [0] [0] [100] [0] [0]

Comments: PDA, ASD and VSD are left to right shunts. Tricuspid atresia is typically associated with cyanosis rather than Mitral. 16) A four year old child is found to have the classical murmur of a patent ductus. He is underweight for age but otherwise well. Which of the following would you recommend for this patient? Available marks are shown in brackets 1) 2) 3) 4) 5) Recommend early operative closure? Review the child constantly, expecting spontaneous closure within the next five years? Recommend prophylactic penicillin until operation is performed? Delay operation until the child has reached its expected weight for age? Explain to the parents that this is of little significance and can be ignored? [100] [0] [0] [0] [0]

Comments: Early operative closure is recommended, in any patient in whom the defect has persisted beyond six months of age. Prophylactic antibiotics are recommended for dental and other invasive procedures. 16) A 9 year old boy presents with fever and joint pains. Initially the pain affected his right wrist, but now affects his left wrist and right ankle. He had tonsillitis 4 weeks previously treated with oral penicillin. Full term normal delivery with no neonatal complications. Immunisations up to date. There is no family or social history of note. On examination temperature is 38.7C, respiratory rate 15/min, heart rate 95/min. 2/6 ejection systolic murmur at the left sternal edge. His left wrist and right ankle are exquisitely tender, such that even the bedclothes cause pain. His ESR is 95 mm/hr and CRP 129 mg/l. ECG shows a PR interval of 0.15s. His ASOT is 800 IU/l. What is the most likely diagnosis? Available marks are shown in brackets 1) 2) 3) 4) 5) Acute rheumatic fever Bacteraemia/sepsis Henoch-Schonlein disease Septic arthritis Urinary tract infection [100] [0] [0] [0] [0]

Comments: The history of sore throat followed by fever, migratory polyarthritis and heart murmur suggest Acute Rheumatic Fever. This is diagnosed using the Duckett-Jones criteria. Evidence of Streptococcal infection PLUS Major criteria: arthritis, carditis, nodules, erythema marginatum and chorea. Minor criteria: fever, arthralgia, raised acute phase reactants, prolonged PR interval.


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

Available marks are shown in brackets 1 ) It is associated with a loud first heart sound. 2 ) It is an early sign of aortic regurgitation It can be distinguished from the murmur of mitral stenosis by absence of 3) presystolic accentuation 4 ) It is due to partial closure of the anterior leaflet of the mitral valve 5 ) It does not occur in aortic incompetence secondary to an aortitis

[0] [0] [0] [100] [0]

Comments: Austin Flint murmur is a low frequency mid/late diastolic murmur which may show presystolic accentuation which is virtually indistinguishable from that of mitral stenosis. 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. 18) A 2 week old girl presents with a history of poor feeding, worse over the last 6 hours. 38+3/40, 2.95kg, no problems at birth. First pregnancy of healthy Caucasian mother. No FH/SH of note. No drugs nor immunisations. On examination temperature is 35.4C (tympanic), RR 60/min, HR 160/min on monitor (all pulses impalpable). Cold mottled peripheries, capillary refill time 6 seconds. O2 saturations 45% in air. Gallop rhythm, 4 cm liver, no spleen. No bruising or rash. What is the most likely diagnosis? Available marks are shown in brackets 1) Congenital adrenal hyperplasia 2) Congenital heart disease 3) Inborn errors of metabolism 4) Sepsis

[0] [100] [0] [0]

Comments: 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 coarcation/ 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. Urgent transfer to a cardiac centre should then follow so a specific diagnosis can be made.


19) Select which of the following is correct in relation to congenital heart disease. Available marks are shown in brackets 1 ) The murmur of a ventricular septal defect is likely to be loud in the first day of life. In Downs syndrome with an endocardial cushion defect irreversible pulmonary 2) hypertension occurs earlier than in children with normal chromosomes. Atrial septal defects, in contrast with ventricular septal defects, never close 3) spontaneously. Transposition of the great vessels is the most common congenital cyanotic heart 4) disease. Failure to thrive is often found associated with Fallots tetralogy at about 3 months 5) of age.

[0] [100] [0] [0] [0]

Comments: The murmur of VSD is intensified after one month of life. ASDs often close spontaneously and the chance of spontaneous closure is related to size ie 5-8mm 80% closure, whereas if the defect is> 8mm then there is little chance of closure. Tetralogy of Fallot is the most common cyanotic congenital heart disease. Fallots tetralogy more commonly presents beyond 3 months of age. 20) Concerning complete atrioventricular septal defects which of the following statements is true?
Available marks are shown in brackets 1) 2) 3) 4) 5) are seen frequently in patients with trisomy 21 frequently have aortic valve insufficiency have a normal mitral valve structure include a coronary sinus atrial septal defect include a perimembranous ventricular septal defect [100] [0] [0] [0] [0]

Comments: Partial AV canal defect or ostium primum ASD Complete AV septal defect Large deficiency in the atrial and ventricular septa. Commonly AV valve orifice and the commonest defect associated with Down's


A) Ostium secundum Is the most common defect and presents in adult life. SIGNS: Right ventricular heave, pulmonary ESM (increased flow), fixed and widened splitting of the second heart sound, mid-diastolic rumbling murmur (increased flow across tricuspid valve).
CXR: large RA and RV, pulmonary plethora and large pulmonary arteries. ECHO: paradoxical septal motion TREATMENT: Surgery age 3-6yrs if significant left to right shunt (> 1.5:1)


B) OSTIUM PRIMUM DEFECT: (common in Down's syndrome) have associated PSM's of MR, TR, and/or VSD. This type is also associated with RBBB and left axis deviation 1HB. DOWN'S SYNDROME trisomy 21 (95% nondisjunction, 2% 21-14 translocation) features at birth most constant o flat facial profile, abundant neck skin o dysplastic ears+pelvis , muscle hypotonia others o round head, protruding tongue o peripheral silver iris spots (Brushfield's) o blunt inner eye angle, short broad hands o single palmar crease, incurving 5th digit o widely spaced 1st + 2nd toes, high arched palate associated problems o duodenal atresia, imperforate anus o Hypo/hyperthyroidism? o Cardiac 40%. VSD, PDA, primum ASD o IQ 25-50, small stature o strabismus 33% o cryptorchidism 27% o Leukaemia 1% (ALL)

21) 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?
Available marks are shown in brackets 1) 2) 3) 4) 5) Blood flow in the lower body Blood flow in the upper body Blood pressure in the upper limbs Vascular resistance in the lower limbs Vascular resistance in the upper limbs [0] [0] [0] [100] [0]

Comments: 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.


22) A 4 month old boy is brought in dead to hospital. He had had a cold for 3 days, with crusty nose and mild fever. He went to bed at 7 pm as usual. Mother checked him at 11pm before going to bed. In the morning she found him stiff and cold. He was brought to A and E by ambulance, but resuscitation was unsuccessful. Mother is single 19 years and smokes 20/d. He was born at 39/40 weighing 3.25 kg, and there were no neonatal problems. He had been growing along the 50th centile for height and weight. What is the most likely diagnosis? Available marks are shown in brackets 1 ) Acute life-threatening event 2 ) Cardiac dysrhythmias 3 ) Seizures 4 ) Sudden infant death syndrome

[0] [0] [0] [100]

Comments: This is a typical history of SIDS. The official definition is: "The sudden death of an infant under 1 year of age that remains unexplained after a thorough case investigation, including performance of a complete autopsy, examination of the death scene, and a review of the clinical history." Risk factors include: maternal smoking, prematurity, over-wrapping, intercurrent infection, prone sleeping position, low social class

23) An 18 year old man had repeated episodes of breathlessness and palpitations, lasting about 20 minutes and resolving gradually. There were no abnormal physical signs. What is the most likely cause of these features? Available marks are shown in brackets 1) Drug abuse 2) Panic disorder 3) Paroxysmal supraventricular tachycardia 4) Personality disorder 5) Thyrotoxicosis

[0] [100] [0] [0] [0]

Comments: Drug abuse is unlikely since ther symptoms are quite short lived. We sould expect other symptoms such as GI disturbance, headaches or hypertension to accompany a variety of drug abuse causes. Paroxysmal SVT would start and stop suddenly, not gradually. Personality disorder and thyrotoxicosis would both be expected to lead to symptoms of longer duration with other associated symptoms. This leaves 'panic disorder' as the most likely diagnosis.



24) A 15 year old female presents following a sore throat with chest pain, fever, and a skin rash. Examination reveals a diastolic murmur. Her ASO titre is elevated. Which of the following is a major criterion for the diagnosis of Rheumatic fever? Available marks are shown in brackets 1) Fever 2) Raised ESR 3) Polyarthritis 4) Migratory erythema 5) Prolonged PR interval

[0] [0] [100] [0] [0]

Comments: Polyarthritis together with erythema marginatum, Sydenham's chorea, carditis and subcutaneous nodules constitute the major criteria associayed with Rheumatic fever. Minor criteria include raised ESR, Arthralgia, pyrexia and a prolonged PR interval. Migratory erythema Q is associated with a glucagonoma.

7-year-old girl with Downs syndrome has central cyanosis. Examination of her chest shows only a soft mid-systolic murmur at the apex and in her abdomen there is a well healed scar in the epigastrium dating from the newborn period. Which of the following statements would be correct: Available marks are shown in brackets 1 ) Her haematocrit is likely to be 30% 2 ) She has Fallots tetralogy 3 ) Arterial Blood gases show CO2 retention The newborn period is likely to have been complicated by Hirschsprungs 4) disease 5 ) Dental extraction should be covered by antibiotic prophylaxis Comments: Most probably she will have secondary polycythaemia and a raised haematocrit. She most likely has Eisenmongers syndrome secondary to a reversed VSD shunt. She has cyanotic heart disease and the blood gases will show hypoxia rather than CO2 retention . Hirschprungs disease (congenital aganglionic megacolon) occurs more frequently in patients with Downs syndrome but it is still a relatively uncommon development. Duodenal atresia may have occurred.She is a high risk for the development of SBE and therefore she should receive antibiotic prophylaxis. [0] [0] [0] [0] [100]