Cardiology

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Cardiology
In uncomplicated patent ductus arteriosus:
A CXR shows evidence of pulmonary plethora (True)
B raised audibility of murmur when standing (False)
C there is evidence of LVH on ECG (True)
D decreased pulse volume (False)
E cyanosis is present (False)
Comments:
The ductus arteriosus is a normal vascular channel during intrauterine life. It is a large vessel with a
muscular wall which courses between the pulmonary artery and the aorta. The ductus arteriosus normally
closes within the first 48 hours of life. If it remains patent longer than this it is unlikely to close
spontaneously. The exception is in premature babies where closure should occur within 3 months. A
persistently patent ductus is a common congenital heart lesion, occurring either singly or in combination
with other defects.
It is more common in: girls than in boys, congenital rubella syndrome, premature babies
Features include increased cardiac output, increased pulmonary flow (plethora on CXR) LVH due to
increased output, increased pulse volume. Cyanosis is not a feature.
At birth the following circulatory changes occur
A A rise in right atrial pressure (False)
B Flap closure of the foramen ovale (True)
C Anatomical closure of the ductus arteriosus (False)
D Functional closure of the ductus venosus (True)
E A 20-fold increase in lung blood flow (False)
Comments:
At the first breath, air fills the lungs and pulmonary vascular resistance falls. Blood flow to the lungs
increases from 10 to 50% of cardiac output, and the increased pulmonary venous return raises left atrial
pressure. There is functional closure of the ductus arteriosus and venosus. Ligation of the umbilicus
increases systemic resistance.
Copyright 2002 Dr Colin Melville
Ostium secundum atrial septal defect:
A often leads to atrial fibrillation during the second decade (False)
B is associated with left axis deviation on the ECG (False)
C is associated with mitral valve prolapse (True)
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D accounts for the majority of cases of ASD (True)
E is generally thought to require surgical correction if the pulmonary blood flow is
greater than twice the systemic
(True)
Comments:
a-Not that young. b-Long PR and RBBB. RAD with secundum, LAD with primum defect.
The following associations are recognised:
A Down's Syndrome and SD (True)
B Maternal tyrosinaemia and congenital heart disease (False)
C Fragile X Syndrome and VSD (False)
D Noonan's Syndrome ad cardiomyopathy (True)
E Maternal phenylketonuria and congenital heart disease. (True)
Comments:
Down's Syndrome is associated particularly with AV canal defects, VSDs and ASDs. Fragile X Syndrome
is associated with mitral valve prolapse and aortic route dilatation. Noonan's Syndrome is associated with
pulmonary stenosis (dysplastic pulmonary valve), ASD and cardiomyopathy. Maternal phenylketonuria is
associated with VSD, ASD, PDA, and coarctation of the aorta.
Copyright 2002 Dr Colin Melville
The following cutaneous stigmata may be associated with congenital heart
disease:
A Steinberg's sign. (True)
B Hypotelorism (True)
C Absent radius. (True)
D Distal triradius. (True)
E Low hair-line (True)
Comments:
A, B, C, D, E Steinberg's sign is where the thumb, when held in the clenched hand protrudes beyond the
palm. This is characteristic of Marfan's Syndrome, and suggest arachnodactyly. Hypotelorism is found in
Patau's Syndrome, which is associated with a number of cardiac defects including VSD, ASD, PDA,
coarctation and bicuspid aortical pulmonary valves. TAR Syndrome (thrombocytopenia and absent
radius) is associated with ASD and Tetralogy of Fallot. A distal triradius is found in Down's Syndrome
which is associated with atrioventricular septal defects, and a low hairline in Turner's Syndrome which is
associated with coarctation.
Copyright 2002 Colin Melville
In the fetal circulation
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A Approximately each volumes of blood are pumped by the left and right
ventricles.
(True)
B The saturation of blood returning from the placenta is about 80%. (True)
C Approximately 25% of cardiac output traverses the fetal lung. (False)
D The ductus venosus is probe-patent in 75% of cases. (False)
E Pulmonary vascular resistance is suprasystemic. (True)
Comments:
A, B, E 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
Nitric oxide (endothelial relaxation factor):
A Unlike prostacyclin, promotes platelet aggregation. (False)
B Can be enzymatically synthesised from glyceryl trinitrate. (True)
C Has a half life of 1-2 minutes in vivo. (False)
D Can be synthesised by macrophages. (True)
E Is synthesised from L-arginine. (True)
Comments:
B, D, E 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
In mitral stenosis caused by rheumatic heart disease, prophylaxis against
endocarditis is unnecessary in:
A cystoscopy (False)
B dental scaling (False)
C caesarean section (True)
D cardiac catherization (True)
E transesophageal echocardiogram (True)
Comments:
The American College of Cardiology guidelines recommend antibiotic prophylaxis for dental work
including scaling, cystoscopy, endoscopy, bronchoscopy and biliary tract surgery. It is not recommended
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for Caesarian section, ToE (except high risk cases) and Cardiac catheterisation.
Which of the following are true of Wolf-Parkinson-White syndrome?
A there is an abberant conduction between atria and ventricles (True)
B broad complex tachycardia is more frequent than narrow-complex tacchycardia (True)
C verapamil is the treatment of choice for an associated SVT (False)
D Amiodarone increases the refractory period in the accessory path. (True)
E Atrial fibrillation is a well recognised rhythm disturbance. (True)
Comments:
a-Bundle of Kent. b-Although orthodromic tachycardia is the commonest it frequently has phasic aberrant
conduction making it broad. Verapmil may exacerbate the rhythm disturbance associated with WPW and
AF may result in VF.
Myocarditis is a recognised association of:
A Salmonella infection. (True)
B Mycoplasma infection. (True)
C Lyme Disease. (True)
D Measles. (True)
E Hurler's Syndrome. (False)
Comments:
A, B, C, D Hurler's is associated with a cardiomyopathy.
Copyright 2002 Colin Melville
The following are features of aortic coarctation in infancy:
A Rib-notching. (False)
B Inverted T wave in V6. (True)
C Ejection systolic murmur between the shoulder blades. (True)
D Radio-femoral delay. (True)
E Plateau pulses. (False)
Comments:
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.
Copyright 2002 Colin Melville
Pulsus paradoxus is found with
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A a severe asthmatic attack (True)
B severe left ventricular failure (False)
C myocardial disease (True)
D constrictive pericarditis (True)
E cardiac amyloidosis (True)
Comments:
Pulsus paradoxus is the exaggerated fall of pressure associated with inspiration due to disease process
like asthma, constrictive pericarditis, tamponade and left ventricular disease (cardiomyopathy). It is not a
feature of left ventricular failure.

Giant "a" waves in the jugular vein can occur in:
A Constrictive pericarditis. (True)
B Pulmonary hypertension. (True)
C Aortic regurgitation. (False)
D Tricuspid stenosis. (True)
E Thyrotoxicosis. (False)
Comments:
A, B, D "a" waves are the positive deflection in the jugular venous pulse following right atrial contraction.
This becomes giant in situations where the atrium is hypertrophied or contracts against resistance, such
as constrictive pericarditis, pulmonary hypertension, and tricuspid stenosis or atresia.
Copyright 2002 Colin Melville
Immediately after a large haemorrhage:
A pulse pressure is increased (False)
B thirst occurs (True)
C anaerobic glycolysis increases (True)
D coronary vasoconstriction occurs (False)
E carotid chemoreceptors are inhibited (False)
Comments:
There is a precipitant fall in pulse pressure with narrowing. Thirst is a consequence of ADH secretion and
stimulation of the thirst receptors. Anaerobic glycolysis occurs due to reduced perfusion with increased
propensity to lactic acidosis. Coronary vasodilatation would be expected and chemoreceptors would of
course be stimulated.
The following are true of cardiac transplantation:
A the donor heart is provided to the recipient with the best HLA match (False)
B long term hypotension is a problem (False)
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C recipients suffer less angina but accelerated coronary artery disease (True)
D the histology of atheroma in the transplanted heart is the same as that of typical
atherosclerosis
(False)
E 75% 5 year survival (True)
Comments:
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.
The following findings suggest that a murmur is innocent:
A It is grade 3/6 (False)
B It has a vibratory quality. (True)
C It is loudest at the apex. (False)
D It is only heard when the patient is febrile. (True)
E It changes in intensity with posture. (True)
Comments:
B, D, E 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
The following are recognised causes of cyanosis in the newborn:
A Persistent fetal circulation. (True)
B Severe anaemia. (False)
C Congenital pneumonia. (True)
D Transient tachypnoea of the newborn. (True)
E Hypoplastic left heart syndrome. (True)
Comments:
A, C, D, E 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
The following are associated with sudden death in childhood:
A Aortic stenosis (True)
B Primary pulmonary hypertension (True)
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C Atrioventricular septal defect (False)
D Fallot's tetralogy (True)
E Cardiomyopathy (True)
Comments:
Sudden death may be caused by tricyclic antidepressants, haemosiderosis, SIDS, metabolic disorders
such as MCAD, child abuse, trauma, hyperthermia, asthma, meningitis/septicaemia, bacterial
endocarditis, pertussis, cholera, RSV, gastro-oesophageal reflux, Reye Syndrome, unrecognised
diaphragmatic hernia, upper respiratory obstruction, pulmonary thromboembolism, prolonged QT
Syndrome, aortic stenosis, mitral valve prolapse, aspiration, anomalous right coronary artery or left
coronary artery, Tetralogy of Fallot, pulmonary atresia intact septum, tricuspid atresia, transposition of the
great arteries, arrhythmia, coronary artery disease in Hurler's Syndrome, calcinosis of the coronary
arteries, viral myocarditis, primary cardiomyopathy, hypertrophic cardiomyopathy, Marfan's Syndrome,
achondroplasia secondary to cervical cord compression, and volatile substance abuse.
Copyright 2002 Dr Colin Melville
A short PR interval on the ECG is associated with
A hypertrophic obstructive cardiomyopathy (True)
B dystrophia myotonica (False)
C Lown-Ganong-Levine syndrome (True)
D rheumatic carditis (False)
E Duchenne muscular dystrophy (True)
Comments:
a - In some cases a short PR interval may be seen the mechanism is not undertood. These cases are
often associated with QRS changes suggestive of pre-excitation.
b+d = long PR interval
A short PR interval on the ECG is associated with:
A Duchenne muscular dystrophy (True)
B dystrophia myotonica (False)
C Friedrich's ataxia (True)
D rheumatic carditis (False)
E hypertrophic cardiomyopathy (True)
Comments:
a-It may be associated with accelerated AV conduction. 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.
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The following are recognised treatments for Fallot's spell:
A Noradrenaline infusion (True)
B Heel-chest position. (True)
C Morphine 0.1mg/kg. (True)
D Prostaglandin E2 50mg/kg/min. (False)
E Propranolol 0.1mg/kg IV. (True)
Comments:
A, B, C, E 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 heel-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
A 6 year old boy presents with fever. The following might contribute to a
diagnosis of rheumatic fever:
A The finding of target lesions on the hands. (False)
B The finding of tender nodules in the fingertips. (False)
C A prolonged PR interval on ECG. (True)
D A CRP of 10. (False)
E Positive Romberg's sign. (False)
Comments:
The modified Jones Criteria include: Finding of preceeding streptococcal infection (recent scarlett fever,
raised ASOT or other streptococcal antibodies, positive throat swab for Group A Strep). Plus:
a) MAJOR CRITERIA:
o Carditis
o Polyarthritis
o Chorea
o Subcutaneous nodules
o Erythema marginatum.
b) MINOR CRITERIA:
o Fever
o Arthralgia
o Previous history of rheumatic fever
o Elevated acute phase reactions
o Prolonged PR interval.
Erythema marginatum involves red circular lesions which gradually enlarge with central clearing.
Sydenham's chorea consists of choreoathetoid movements with increased clumsiness, e.g. deteriorating
handwriting. This is often associated with emotional lability. Target lesions suggest erythema multiforme.
A CRP of 10 is not elevated much beyond the normal range. Erythema marginatum initially manifests as
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non-specific pink macules seen over the trunk, with later blanching in the middle of the lesions and
sometimes fusing of the borders resulting in a serpiginous (serpent-like) looking lesion. The rash is
worsened with heat, but is characteristically evanescent. It does not itch, and can be mistaken for the
rash of Lyme disease. Sub-cutaneous nodules are pea-sized, firm and non-tender. There is no
associated inflammation and they are characteristically seen on the extensor surfaces of joints such as
knees and elbows and also over the spine.
Copyright 2002 Dr Colin Melville
Diastolic dysfunction of the left ventricle may be a dominant feature of:
A Hypertrophic obstructive cardiomyopathy. (True)
B Endocardial fibroelastosis. (True)
C Doxorubicin toxicity. (True)
D Anomalous left coronary artery. (True)
E Myocardial infarction after Kawasaki Disease. (True)
Comments:
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
The following are recognised causes of an infant cyanosed from day 2:
A Eisenmenger's Syndrome. (False)
B Ebstein's anomaly. (True)
C Pulmonary atresia. (True)
D Transposition of the great arteries. (True)
E Total anomalous pulmonary venous drainage. (True)
Comments:
B, C, D, E Eisenmenger's Syndrome is a late complication of left to right shunt, where pulmonary
hypertension leads to shunt reversal. Ebstein's anomaly is due to a distally placed tricuspid valve with
small ventricle and very large right atrium. This results in tricuspid regurgitation with right to left shunting
at atrial level producing cyanosis. Usually patients are well into adult life, but newborn presentation is
described. TAPVD refers to a situation where pulmonary veins drain via an aberrant confluence into the
right side of the circulation. The aberrant vessel may become obstructed causing pulmonary plethora, but
a normal cardio-thoracic ratio. Alternatively, venous return to the right side may be reduced such that the
child becomes cyanosed.
Copyright 2002 Colin Melville
A 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:
A Her haematocrit is likely to be 30% (False)
B She has Fallots tetralogy (False)
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C Investigation of her blood gases shows carbon dioxide retention (False)
D The newborn period is likely to have been complicated by Hirschsprungs
disease
(False)
E Dental extraction should be covered by antibiotic prophylaxis (True)
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. She is a high risk for the development of SBE and therefore she should receive antibiotic
prophylaxis.
A 7 day old baby presents with cyanosis. This is pathophysiologically due to
abnormal mixing in the following conditions:
A Tetralogy of Fallot. (False)
B Transposition of the great arteries. (True)
C Total anomalous pulmonary venous drainage. (True)
D Univentricular heart. (True)
E Aortic coarctation. (False)
Comments:
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
Giant 'a' waves in the JVP occur in:
A pulmonary hypertension (True)
B aortic regurgitation (False)
C thyrotoxicosis (False)
D constrictive pericarditis (False)
E tricuspid stenosis (True)
Comments:
a - Giant 'a' waves occur when there is a poorly compliant right ventricle (or tricuspid stenosis) increasing
the impedence against which the right atrium has to eject blood. d - In constrictive pericarditis the JVP is
high with an abrupt fall in systole (x descent) and may rise with inspiration (Kussmaul's sign).
In a 2 year old child with persistent chest symptoms, the following should be
considered:
A Maternal smoking. (True)
B ASD. (True)
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C VSD. (True)
D PDA. (True)
E Aortic coarctation. (False)
Comments:
A, B, C, D In the neonatal period, aortic coarctation presents with shock. Later, an inter-scapular murmur
or absent femoral pulses are commonest. In the older child, hypertension is likely. In a child with
persistent chestiness, attendance at nursery, asthma, cystic fibrosis, and minor immune deficiency
should also be considered.
Copyright 2002 Colin Melville
In a child with patent ductus arteriosus, the following may be important
aetiologically
A Maternal SLE (False)
B Maternal alcoholism (False)
C Maternal Warfarin therapy (True)
D Maternal CMV infection (False)
E Maternal rubella infection (True)
Comments:
SLE in the mother is associated with Ro antibody and congenital heart block. The mother may be
presymptomatic. Fetal alcoholism is associated with ASD, VSD and Fallot's. CMV us not associated with
cardiac defects, but rubella is associated with PDA and peripheral pulmonary stenosis. Congenital CMV
causes IUGR, hepatitis, splenomegaly, jaundice, microcephaly, intra-cranial calcification and deafness.
Copyright 2002 Dr Colin Melville
Recognised toxic effects of Amiodarone include:
A Hyperthyroidism (True)
B Peripheral neuropathy (True)
C Hepatitis (True)
D Hypothyroidism (True)
E Photosensitivity (True)
Comments:
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,
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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.
Copyright 2002 Dr Colin Melville
In patients with congenital heart disease:
A Approximately 10-15% will have more than one cardiac abnormality. (True)
B The majority of lesions can be diagnosed antenatally using ultrasound. (True)
C The majority of infants will require cardiac catheterisation. (False)
D Complex defects usually require staged surgical procedures. (False)
E Approximately 25-30% will also have a non-cardiac abnormality. (False)
Comments:
B, E The majority of patients with CHD can be diagnosed antenatally by specially trained operators.
Postnatal echocardiography will diagnose most lesions, and catheterisation is less necessary. Most
defects can be corrected in the neonatal period as a single stage procedure. About 10-15% have an
associated non-cardiac abnormality.
Copyright 2002 Colin Melville
The following are features of mitral stenosis:
A right axis deviation on the ECG (True)
B increased pulmonary artery capillary wedge pressure (True)
C left parasternal heave (True)
D palpable 1st heart sound (True)
E palpable 2nd heart sound (True)
Comments:
This lesion is almost always (99%) due to rheumatic heart disease although a clear history is obtained in
only 50% of cases. 25% of patients with rheumatic heart disease have pure mitral stenosis, two-thirds are
female. In parallel with the incidence of rheumatic fever, mitral stenosis is more common and generally
more severe in developing countries. It causes elevated right sided pressures with a parasternal heave
reflecting RVH. The tapping apex beat signifies a palpable 1st heart sound. With pulmonary
hypertension, a palpable second sound may be felt.
The following may be found in atrioventricular septal defects:
A RSR1 pattern in V1. (False)
B Mild cyanosis in the early stages. (False)
C A pan-systolic murmur at the apex. (True)
D Wide but variable splitting of the second heart sound. (False)
E An ejection murmur at the upper left sternal border. (True)
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Comments:
C, E An RSR pattern is caused by right bundle branch block, and is seen in secundum 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.
Question supplied by Colin Melville Consultant Paediatrician
Cerebral abscess in the absence of endocarditis is a recognised complication
of:
A tetralogy of Fallot (True)
B persistant ductus arteriosus (False)
C tricuspid artresia (True)
D transposition of the great arteries (True)
E atrial septal defect of the ostium primum type (False)
Comments:
This is paradoxical embolization seen in right to left shunts. Associated with ASD of patent foramen ovale
and ostium secundum types.
The following lesions can be diagnosed antenatally on a routine 4 chamber
view at 18 weeks gestation:
A Tetralogy of Fallot. (False)
B Hypoplastic left heart syndrome. (True)
C VSD. (False)
D Pulmonary stenosis. (False)
E Transposition of the great arteries. (False)
Comments:
B 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
In the diagnosis of rheumatic fever, the following may be helpful:
A Polyarthritis. (True)
B ASO titre of less than 1:200. (False)
C Staphylococcus aureus grown on throat culture. (False)
D A generalised macular-papular rash. (False)
E Splinter haemorrhages. (False)
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: - Pancarditis. - Polyarthritis - Erythema
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marginatum - Chorea - Subcutaneous nodules - The rash is macular. MINOR: - Fever - Polyarthralgia -
History of RF - Raised ESR/CRP - Prolonged PR interval on ECG.
Copyright 2002 Colin Melville
Causes of cyanotic heart disease with oligaemia include
A Tricuspid atresia (True)
B Pulmonary atresia with ventricular septal defect and multiple aorto-pulmonary
collateral arteries
(False)
C Fallot's tetralogy (True)
D Transposition of the great arteries (False)
E Pulmonary stenosis (True)
Comments:
Cyanotic heart disease can be divided into 2 groups: that with increased, or that with decreased
pulmonary blood flow. The former include conditions such as transposition of the great arteries while the
latter include tricuspid atresia and Fallot's Tetralogy.
Copyright 2002 Dr Colin Melville
The following are recognised causes of secondary hypertension:
A Henoch Schonlein Purpura (True)
B Nephroblastoma (True)
C Neuroblastoma (True)
D Addison's Disease (False)
E Hypothyroidism (False)
Comments:
Recognised secondary causes include:
1. RENAL:
o Congenital: Dysplastic or polycystic kidneys.
o Acquired: Glomerulonephritis, chronic pyelonephritis, reflux nephropathy, HUS, HSP,
Wilm's tumour.
2. VASCULAR:
o Coarctation.
o Renal artery stenosis or embolisation, renal vein thrombosis.
3. ESSENTIAL..
4. ENDOCRINE:
o Pheochromocytoma.
o Neuroblastoma.
o Cushing's Disease.
o Conn's Syndrome.
o Diabetes mellitus.
o Adrenogenital syndrome.
o Hyperthyroidism.
5. NEUROLOGICAL:
o Neurofibromatosis.
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o Raised intracranial pressure.
o Encephalitis.
6. MISCELLANEOUS:
o lead poisoning.
o Obesity.
o Porphyria.
o Drugs, e.g. steroids.
Copyright 2002 Dr Colin Melville
Which of the following procedures are appropriate?
A Rastelli procedure for Taussig-Bing heart (double-outlet right ventricle with sub-
pulmonary VSD).
(True)
B Modified Blalock-Taussig shunt for critical aortic stenosis. (False)
C Balloon valvotomy for critical pulmonary stenosis. (True)
D Stage 1 Norwood procedure for hypoplastic left heart syndrome. (True)
E Fontan procedure for tricuspid atresia. (True)
Comments:
A Rastelli operation involves using a pulmonary of aortic homograft conduit to relieve pulmonary
obstruction in double outlet right ventricle with pulmonary stenosis (Taussig-Bing heart).
A Blalock-Taussig shunt is used to increase pulmonary blood flow in duct dependent cyanotic conditions,
e.g. pulmonary atresia.
A Stage 1 Norwood Procedure for hypoplastic left heart syndrome involves atrial septectomy and
transection and ligation of the distal main pulmonary artery. The paroximal pulmonary artery is then
connected to the hypoplastic aortic arch, while the coarcted segment of the aorta is repaired. An aorto-
pulmonary shunt is created to connect the aorta to the main pulmonary artery to provide pulmonary blood
flow. A Fontan procedure is used to direct blood flow from the systemic veins to the pulmonary artery
directly, bypassing the hypoplastic ventricle.
Copyright 2002 Dr Colin Melville
Recognised features of abetalipoproteinaemia include:
A a high serum cholesterol (False)
B palmar xanthomas (False)
C advanced atherosclerotic vascular disease (False)
D abnormal red blood cell morphology (True)
E severe mental retardation (False)
Comments:
Abetalipoproteinaemia causes neurodegenerative nervous system changes - ataxia with retinitis
pigmentosa. IQ is usually normal. Other clinical manifestations of steatorrhoea and malabsorption which
may lead to osteopenia and skeletal fractures. Radiologically, there is thickening of the mucosal folds of
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the duodenum and jejunum. Inheritance is autosomal recessive.
The following are compatible with a secundum ASD:
A Mid-diastolic rumble at the left sternal edge. (True)
B QRS axis of -30 degrees. (False)
C RSR pattern in V1. (True)
D Wide splitting of the second heart sound. (True)
E Pulmonic ejection click (False)
Comments:
A, C, D Secundum ASD usually presents with a murmur due to increased blood flow through the
pulmonary valve. If the shunt is large, then a mid-diastolic rumble may be heard at the apex due to
increased mitral valve flow, but a tricuspid rumble does not occur. A superior axis deviation suggests a
primum ASD, but an RSR pattern (M pattern) in V1 is characteristic. Wide fixed splitting of the second
heart sound occurs because of equal filling of the left and right atria during all phases of the respiratory
cycle. A pulmonic ejection click is characteristic of pulmonary valve stenosis.
Question Supplied by Colin Melville Consultant Paediatrician
The following features suggest Tetralogy of Fallot rather than transposition of
the great arteries:
A Presentation at 2 months of age with a murmur. (True)
B Absence of clinical cyanosis (True)
C Increased pulmonary vascular markings on chest x-ray. (False)
D Single second heart sound. (False)
E Absence of S wave in V1. (True)
Comments:
A, B, E On chest x-ray, a 'boot-shaped' heart with pulmonary artery bay and uptilted apex suggests
Fallot's, while an 'egg on side' appearance and pulmonary plethora suggests TGA. In Fallot's lung blood
flow is decreased, and the absence of an S wave in V1 suggests right ventricular hypertrophy. The
second heart sound is single in both conditions. Transposition usually presents in the first few days of life,
and the patient is invariably clinically cyanosed.
Copyright 2002 Colin Melville
The following are features of Fallot's tetralogy:
A prominent systolic murmur of ventricular septal defect (False)
B pulmonary plethora (False)
C squatting (True)
Cardiology
17
D paradoxical embolus (True)
E presentation in late teens (False)
Comments:
a-The systolic murmur comes from the pulmonary infundibular stenosis. b-There is usually pulmonary
oligaemia as there is a right to left shunt. c-Squatting relieves the dyspnoea. d-Because of the right to left
shunt at ventricular level. e-Presentation is invariably in childhood. Read here for more on Fallot's
Regarding cardiogenic shock:
A Increased firing of neurones in the carotid body stimulates the vagus nerve. (False)
B Renal cortical necrosis is a recognised complication. (True)
C Constriction of the renal afferent arterioles stimulates renin production. (True)
D Angiotensin II production is increased in the liver. (False)
E ANP production is increased. (False)
Comments:
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
Broad complex tachycardias:
A May be associated with shock. (True)
B May be associated with pulselessness (True)
C Are usually treated with asynchronous DC shock. (True)
D Are uncommon in childhood. (True)
E May be due to supraventricular tachycardia. (True)
Comments:
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 Lignocaine
can be tried in VT if no shock is present. Otherwise (and usually) asynchronous DC shock 0.5J/kg is
used.
Copyright 2002 Colin Melville
The following are recognised associations with pulmonary hypertension:
A An apgar of 3 at 5 minutes. (True)
B Meconium aspiration (True)
Cardiology
18
C Hyaline membrane disease (True)
D Hypoglycaemia (True)
E Oligohydraminos (True)
Comments:
Persistent fetal circulation occurs with all of these. It is usually treated using nitric oxide, a specific
pulmonary vasodilator.
Copyright 2002 Dr Colin Melville
The following drugs are contra-indicated for the treatment of atrial fibrillation
associated with the WPW syndrome:
A Digoxin (True)
B Adenosine (False)
C Amiodarone (False)
D Verapamil (True)
E Sotalol (False)
Comments:
In WPW digoxin or calcium channel blockers may increase anterograde conduction through the bypass
tract, so they should be avoided.
The following are found in VSD:
A Upright T wave in V1 in pulmonary hypertension. (True)
B Pulmonary plethora and a cardio-thoracic ratio of 60%. (True)
C Mild cyanosis in the first few days of life. (False)
D A loud second heart sound. (True)
E A parasternal thrill. (True)
Comments:
A, B, D, E An upright T wave in V1 suggests right ventricular hypertrophy. Pulmonary plethora and
increased CTR suggests right heart failure, usually due to left to right shunt. A loud second sound is
caused by increased pulmonary artery pressure from the shunt. Cyanosis is not a feature of VSD.
Copyright 2002 Colin Melville
Infective endocarditis rarely occurs with:
A mitral valve prolapse (False)
B patent ductus arteriosus (False)
Cardiology
19
C bicuspid aortic valve (False)
D atrial septal defect (True)
E tight mitral stenosis (True)
Comments:
a - Regurgitant mitral valve is most frequent site of endocarditis. Mitral valve prolapse has a high
prevalence (7.6% females, 2.5% males in Framingham study). b - However early surgery has virtualy
abolished patent ductus arteriosus. e - The rate is 1 - 5%. (Read more ...)
The following findings may occur with a cyanotic episode in an individual with
Fallot's tetralogy:
A bradycardia (False)
B pallor (False)
C increased systolic murmur (False)
D loss of consciousness (True)
E continuous murmur at the upper left sternal border (True)
Comments:
Usually polycythaemic and cyanosed. The Murmur may becoma softer during a cyanotic episode and
syncope can occur. The continuous murmur may still be audible. (Dr Jacob Easaw)
Rheumatic chorea
A does not occur in conjunction with rheumatic fever (False)
B is not associated with muscular weakness (False)
C may be unilateral (True)
D responds rapidly to ACTH (False)
E movements cease during sleep (True)
Comments:
Sydenham's Chorea/St Vitus dance is associated with Rheumatic fever and frequently seen in children.
Unilateral chorea may be a feature and muscular weakness is associated. ACTH is not used for
treatment and is treated with appropirate therapy of underlying condition.
An Early Diastolic Murmur at the left sternal edge is characteristic in:
A Mitral stenosis (False)
B Ankylosing spondylitis (True)
C Pulmonary stenosis (False)
D SLE (True)
E Eisenmenger's syndrome (True)
Comments:
Cardiology
20
A - There will be a rumbling low pitched mid diastolic murmur best heard in the mitral area. B - Due to
AR. Other causes of AR include Rheumatic heart disease, Congenital bicuspid aortic valves,
Hypertension, Dilated aortic root as in Marfan's, SBE, Syphilitic aortitis, Degenerative valve disease,
SLE, Prolapse of a aortic cusp due to a VSD, Rheumatoid arthritis, Trauma, Takayasu's Disease. C -
There will be a loud ejection murmur usually in the pulmonary area best heard on inspiration. E - Due to
PR (Graham Steell murmur)due to pulmonary hypertension.
In the interpretation of ECGs, the following are correct:
A Pulmonary hypertension id the P wave in lead II is greater than 2mm. (False)
B Inverted T waves in V5 and V6 are occasionally found in infants. (False)
C Mean QRS axis in the newborn lies between 60 and 160 degrees. (True)
D Slightly elevated ST segments in V3 may occur in normal teenagers. (True)
E An inverted T wave in lead I may indicate situs inversus. (True)
Comments:
C, D, E In pulmonary hypertension the P wave in lead II is greater than 2.5mm. Inverted T waves may be
found across to V3 in normal infants, and may remain so beyond the second decade. The mean QRS
axis in the newborn lies between 60 degrees and 160 degrees. Dextracardia with dextraversion may
result in inverted ECG appearances.
Question supplied by Colin Melville Consultant Paediatrician
The following drug side effects and interactions are recognised:
A Adenosine and sinus arrest. (True)
B Verapamil and asystole. (True)
C Amiodarone and hyperthyroidism. (True)
D Amiodarone and hypothyroidism. (True)
E Adenosine and bronchospasm. (True)
Comments:
A, B, C, D, E Verapamil is rarely used in childhood. In combination with betablockers, it is particularly
lethal.
Copyright 2002 Colin Melville
In cardiac tamponade, the following may be recognised:
A Pulsus alternans (False)
B Raised jugular venous pulse (True)
C Muffled heart sounds (True)
D Cannon waves (False)
E Diminished pulse volume (True)
Comments:
Cardiology
21
Cardiac tamponade is caused by fluid in the pericardial sac constricting the heart. This results in pulsus
paradoxis, raised jugular venous pulse, muffled heart sounds, and if severe, diminished pulse volume
and shock. Cannon waves occur because of atrial contraction against the closed tricuspid valve as in
atrial fibrillation, and pulsus alternans suggests a severely diseased ventricular muscle. Electrical
alternans, with variable QRS complex amplitude, may be present in pericarditis. (See example on
ecglibrary.com)
Copyright 2002 Dr Colin Melville
Cyanotic attack in Tetralogy of Fallot is characterised by
A Pallor (False)
B Bradycardia (True)
C Increase in severity of systolic murmur (False)
D Apnoea (False)
E Impaired consciousness (True)
Comments:
Paroxysmal hypercyanotic attacks (blue or 'tet' spells) are a problem in the first 2 years of life. The infant
hyperventilates and becomes restless with cyanosis, gasping and finally syncope. Spells occur most
often in the early morning or after vigorous crying. The pulmonary murmur disappears or quietens during
the episodes as RVOT flow decreases. They last from minutes to hours, but are rarely fatal.
Complications include severe hypoxia, metabolic acidosis, convulsions or hemiparesis.
Copyright 2002 Dr Colin Melville
The following are recognised complications of cyanotic congenital heart
disease:
A Psychomotor delay. (True)
B Cerebral thrombosis. (True)
C Infective endocarditis. (True)
D Arthritis. (True)
E Cerebral abscess. (True)
Comments:
A, B, C, D, E Complications of cyanotic congenital heart disease in Eisenmenger physiology include: -
Polycythemia - CNS abscess - Thromboembolic stroke - Low grade DIC with thrombocytopenia -
Haemoptysis - Gum disease - Gout - Arthritis - Clubbing - Failure to thrive - Psychomotor delay Janeway
lesions are painless, small, erythematous or haemorrhagic lesions on the soles of the palms or soles,
that occur in infective endocarditis.
Copyright 2002 Colin Melville
The calibre of arterioles is affected as follows:
Cardiology
22
A angiotensin II causes marked vasodilatation (False)
B epinephrine causes vasodilatation in skeletal muscles (True)
C decreased oxygen tension causes vasodilatation (True)
D locally released serotonin causes vasoconstriction (True)
E increased noradrenergic discharge causes vasodilatation (False)
Comments:
Adrenaline produces vasodilatation of arterioles within muscles but constriction within the skin.
Noradrenaline causes vasoconstriction. Decreased O2 produces vasodilatation but serotonin/5HT causes
vasoconstriction except for vasodilatation of muscle arterioles.
Regarding supraventricular tachycardias:
A Rates of 120080 beats per minute are typical in adolescence. (False)
B They are usually associated with structural cardiac defects. (False)
C Lown-Ganong-Levine Syndrome is the most commonest cause. (False)
D J waves may be seen. (False)
E DC shock is most commonly required. (False)
Comments:
All answers are false. SVT is usually due to re-entry within the AV node, 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
A 3 day old baby, discharged following a normal routine postnatal
examination, is rushed to hospital with a thready pulse and heart rate of
180/min, a 5 cm liver edge, and a capillary refill time of 6 seconds. The
following cardiac lesions should be considered:
A Transposition of the great arteries (False)
B Double outlet right ventricle (False)
C Pulmonary atresia (False)
D Hypoplastic left heart syndrome (True)
E Truncus arteriosus (True)
Comments:
Cardiology
23
Neonatal shock may be caused by infection, cardiac lesions or metabolic abnormalities. TGA, double
outlet right ventricle, and pulmonary atresia cause cyanosis. Hypoplastic left heart syndrome, truncus
arteriosus, coarctation, or critical aortic stenosis are the likely cardiac causes of this presentation.
Copyright 2002 Dr Colin Melville
A murmur is exaggerated during inspiration in:
A pulmonary regurgitation (True)
B aortic regurgitation (False)
C tricuspid regurgitation (True)
D patent ductus arteriosus (False)
E mitral stenosis (False)
Comments:
a - All right sided events increase on inspiration except a pulmonary click.
A 6 week old girl presents with respiratory distress. The following suggests
cardiac failure rather than bronchiolitis:
A Wheezing. (False)
B Poor weight gain for the past 3 weeks. (True)
C A respiratory rate of 60 breaths/min. (False)
D Cardio-thoracic ratio on AP x-ray of 60%. (True)
E A 4cm liver. (True)
Comments:
B, D, E Tachypnoea and wheezing may occur in both heart failure and bronchiolitis, though crepitations
are commoner in the latter. Bronchiolitis rarely lasts as long as 3 weeks. A normal AP cardio-thoracic
ratio is less than 55%. H Heart failure is rare in bronchiolitis, so an increased CTR and very large liver
suggest heart failure. Not uncommonly, a 2-3cm liver is found in bronchiolitis because of hyperinflation of
the lungs.
Copyright 2002 Colin Melville
Recognised features of a large uncomplicated patent ductus arteriosus
include:
A Narrow pulse pressure. (False)
B Pulmonary plethora. (True)
C Decreased noise of murmur on squatting. (False)
D Left ventricular hypertrophy on ECG. (True)
E Soft pulmonary second heart sound. (False)
Cardiology
24
Comments:
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
Regarding systemic hypertension in childhood:
A Sodium nitroprusside is useful for the long-term treatment of severe cases. (False)
B Headache is the usual presenting feature. (False)
C It is defined as systolic blood pressure above the 99th centile for age. (False)
D Abnormalities are frequently seen on DMSA scan. (True)
E Aortic coarctation is the commonest secondary cause. (False)
Comments:
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
Interventional catheterisation can be used to repair the following defects:
A Ostium primum ASD. (False)
B Ostium secundum ASD. (True)
C Muscular VSD. (False)
D PDA. (True)
E Dysplastic pulmonary valve. (False)
Comments:
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
The carotid body chemoreceptors are:
A stimulated by a decrease in pO2 of arterial blood (True)
B inhibited by a decrease in pH of arterial blood (False)
Cardiology
25
C stimulated in an hypotensive subject at rest (True)
D responsible for production of reflex peripheral vasoconstiction (True)
E have a blood flow of over 50ml/100g per minute (True)
Comments:
a - discriminating question
c+d-Hard.
e-Highest of any tissue.
carotid sinus is baroreceptor responding to hypotension & mediating vasoconstriction, while carotid body
is chemoreceptor. Respiratory arrest and circulatory shock dramatically increase chemoreceptor activity
leading to enhanced sympathetic outflow to the heart and vasculature via activation of the vasomotor
centre.
The following are indications for immediate transfer to a tertiary cardiology
centre in infancy:
A Suspected large VSD. (False)
B Suspected ASD. (False)
C A diagnosis of Down's Syndrome. (False)
D A diagnosis of Kawasaki Disease. (False)
E Suspected aortic coarctation. (True)
Comments:
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. Severe coarctation of the aorta. 4. Interruption of the aortic arch. The
other conditions mentioned require more routine cardiac evaluation.
Copyright 2002 Colin Melville
Myocardial infarction is a recognised feature of the following
A Kawasaki Disease (True)
B Viral myocarditis (True)
C Hypertrophic obstructive cardiomyopathy (True)
D Mitral stenosis (False)
E Anomolous left coronary artery (True)
Comments:
In mitral stenosis, large p-mitrale is first seen, with AF being a late sign. In hypertrophic obstructive
Cardiology
26
cardiomyopathy, the heart muscle outgrows the blood supply, causing angina or myocardial infarction.
Copyright 2002 Dr Colin Melville
The following are characteristic of tricuspid atresia:
A P wave of >2.5mm which is narrow. (True)
B Increased pulmonary blood flow. (False)
C Co-existing ASD. (True)
D Left axis deviation on the ECG. (True)
E Small right ventricle on echocardiography. (True)
Comments:
In tricuspid atresia there is a fibromuscular membrane in place of the tricuspid valve, a variably small
right ventricle, a VSD, and a large left ventricle and aorta. Since there is no outlet from the right atrium,
the entire systemic venous return enters the left heart via the foramen ovale or an associated ASD. Left
ventricular blood is then pumped into the right ventricle by the VSD into the pulmonary circulation. The
pulmonary blood flow is therefore decreased and a characteristic presentation is cyanosis with decreased
pulmonary blood flow. Since the right ventricle is small, there is usually left axis deviation on ECG.
Treatment is often with a modified Fontan procedure where the systemic venous return is diverted using
a right atrial baffle to the pulmonary arteries with subsequent closure of the ASD and VSD.
Copyright 2002 Dr Colin Melville
An opening snap is found in:
A mitral stenosis due to rheumatic heart disease (True)
B congenital mitral stenosis (True)
C mitral incompetence associated with a rigid posterior valve leaflet but a normal
anterior leaflet
(True)
D left atrial myxoma (True)
E severe aortic incompetence (False)
Comments:
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;25:5056 )
A loud first heart sound may be due to:
A a long preceding diastolic interval (False)
B mitral stenosis (True)
C rupture of a papillary muscle (False)
D increased systemic arterial pressure (False)
Cardiology
27
E increrased pulmonary arterial pressure (False)
Comments:
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.
Cyanosis occurs in:
A patent ductus arteriosus (False)
B Fallot's tetralogy (True)
C ostium primum Atrial Septal Defect (False)
D tricuspid atresia (True)
E Eisenmenger's complex (True)
Comments:
Cyanosis defined as greater than 5g% reduced haemoglobin typically occurs in association with right to
left shounts as is Fallot's, Transposition, hypoplastic left heart, tricuspid atresia and Eisenmenger's. PDA
and ostium primum are not associated with cyanosis unless the latter converts to eisenmenger's complex
with time.
The following can cause bradycardia:
A hypothermia (True)
B hypothyroidism (True)
C severe anaemia (False)
D subdural haematoma (True)
E shock (True)
Comments:
a-One of the main features - can be extreme bradycardia, wide QRS, long QT interval and prominent J
wave. b-Sinus tachycardia / AF with hyperthyroidism. c-Usually associated with sinus tachycardia
especially if severe. d-From raised intracranial pressure (Cushing's reflex - raised ICP leads to increased
blood pressure and decreased heart rate). e-This is arguable as it is usually associated with sinus
tachycardia however there may be a bradycardic response to shock.
Fixed splitting of the second heart sound occurs in the following conditions
A Ostium primum defect (True)
B Fallot's tetralogy (False)
C Chronic constrictive pericarditis (False)
D Aortic stenosis (False)
E Ostium secundum defect (True)
Cardiology
28
Comments:
Fixed splitting of the second heart sound occurs in atrial septal defects because of equal pressures in the
atria throughout the respiratory cycle.
Copyright 2002 Dr Colin Melville
Fixed splitting of the second heart sound is found in:
A Ostium secundum ASD (True)
B Perimembranous VSD (False)
C Ebstein's anomaly (False)
D Ostium primum ASD (True)
E Pulmonary valve stenosis (False)
Comments:
Fixed splitting of the second heart sound occurs with ASDs. VSD is associated with a loud second heart
sound, and Ebstein's anomaly with a widely split variable second heart sound. Pulmonary valve stenosis
is associated with a soft or absent pulmonary component to the second heart sound.
Copyright 2002 Dr Colin Melville
The following associations are recognised:
A Pulmonary plethora and pulmonary atresia with intact ventricular septum. (False)
B Prominent right heart border and ASD. (True)
C Pericardial calcification and endocardial fibroelastosis. (False)
D 'Cottage loaf' heart and total anomalous pulmonary venous drainage. (True)
E Selenium deficiency and cardiomyopathy. (True)
Comments:
B, D, E In pulmonary atresia with intact ventricular septum there is decreased pulmonary blood flow. The
prominent right heart border occurs in ASD becasue of right atrial hypertrophy. In endocardial
fibroelastosis the interior of the ventricles appear echogenic, but there is no pericardial calcification.
Pericardial calcification is found in constrictive pericarditis. A 'cottage loaf' heart is characteristic of
TAPVD, and selenium deficiency is the cause of Keshan Disease, a rare nutritional cardiomyopathy that
is frequently fatal in the Keshan Province of China.
Copyright 2002 Colin Melville
In the differential diagnosis of cyanosis in the newborn:
A A ground glass appearance confirms hyaline membrane disease. (False)
B An arterial PO2 above 13 kPa suggests non-cyanotic congenital heart disease. (False)
C A normal cardiothoracic ratio excludes total anomalous pulmonary venous (False)
Cardiology
29
drainage.
D Persistent hypoxia in a child of 34 weeks gestation suggests transposition of the
great arteries.
(False)
E A saturation of 91% in the right hand and 85% in the right leg suggests
significant shunting.
(True)
Comments:
A ground glass appearance is not specific for hyaline membrane disease: Group B Streptococcus can
also present in this way. In the nitrogen wash-out test, an arterial PO2 <13kPa in 100% oxygen suggests
cyanotic congenital heart disease. Other causes of cyanosis, such as pneumonia or hyaline membrane
disease, can normally be oxygenated beyond this level. A normal cardio-thoracic ration is characteristic
of total anomalous pulmonary venous drainage. This is becasue the obstruction is extra-cardiac. The
usual reason for hypoxia in a pre-term baby is hyaline membrane disease or infection, with cardiac
causes being rarer. A significant difference in oxygen saturation between the pre-ductal and post-ductal
circulations suggests that hypoxic blood is shunting from the pulmonary artery to the aorta.
Copyright 2002 Dr Colin Melville
The following are normal findings on the neonatal ECG
A A frontal axis of +50 (False)
B Larger R wave than S wave in V1 (True)
C An RS ratio in V6 of 2 (False)
D An inverted T wave in V4R (True)
E A small Q wave in V1 (False)
Comments:
In the neonate the ECG reflects the transitional circulation. Since in-utero the right ventricle is the
systemic ventricle, the right sided forces of greater than the left, gradually changing postnatally. The right
sided chest leads in the neonates, therefore show larger positive R waves than negative waves, and this
may remain for several months or years. The left sided leads (V5 and V6) also reflect the RV dominance,
and the RS ratio in these leads may be <1. The QRS axis usually lies between 110 and 180. The T
waves are inverted in V4R, V1, V2 and V3 during infancy, and this may remain until the middle of the
second decade and beyond. Q waves are always abnormal.
Copyright 2002 Dr Colin Melville
The following are characteristic of acute pericardial effusion:
A Giant "a" waves in the jugular venous pulse. (True)
B Pulsus paradoxus. (True)
C Right ventricular end-diastolic collapse on echocardiography. (True)
D Low blood pressure. (True)
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30
E Cold extremities. (True)
Comments:
A, B, C, D, E Acute pericardial effusion causes cardiac tamponade with all these signs. In addition the
heart sounds may be muffled and there may be signs of shock.
Copyright 2002 Colin Melville
Regarding the ductus arteriosus:
A It usually closes functionally by 6 hours post delivery in a term infant. (True)
B May close prematurely due to maternal aspirin in pregnancy. (True)
C Has a right to left shunt in utero. (True)
D Closure can be delayed by the use of prostaglandin E2. (True)
E Closure is encouraged by hypoxaemia. (False)
Comments:
A, B, C, D The ductus arteriosus functionally closes within the first few hours after birth. Aspirin
antenatally is associated with impaired platelet function and risk of haemorrhage, delayed onset and
increased duration of labour, and fetal ductus arteriosus closure in high doses. It may also be related to
persistent pulmonary hypertension and kernicterus in jaundiced neonates. Prostaglandin E2 is used in
the emergency management of duct dependent cardiac lesions prior to transfer to cardiac units.
Hypoxaemia tends to keep the duct open.
Copyright 2002 Colin Melville
An infant is noted at postnatal examination to have a slow pulse rate. ECG
confirms congenital complete heart block. The following statements are true:
A Echocardiography is usually normal. (True)
B It is usually associated with a presence of anti-Ro antibodies in the mother. (True)
C Heart failure is uncommon. (True)
D Mother's thyroid function should be checked. (False)
E The underlying defect may be due to prolonged QT Syndrome. (False)
Comments:
The commonest cause is maternal SLE, which may be asymptomatic. 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
The following suggest pulmonary rather than aortic valve stenosis:
A An ejection murmur radiating to the neck. (False)
B An ejection click best heard in the 2nd and 3rd left intercostal spaces. (True)
C An upright T wave in V1. (True)
D A deep S wave in V2. (False)
Cardiology
31
E Prominence in the left upper mediastinum. (False)
Comments:
B, C 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 LVH, while an upright T wave in V1 suggests 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
Cannon waves may be seen in the jugular veins in:
A constrictive pericarditis (False)
B first degree AV heart block (False)
C tricuspid stenosis (False)
D ventricular pacing (True)
E nodal tachycardia (True)
Comments:
Canon waves are seen with unsynchronised closure of the tricuspid valve and right atrial contraction.
Giant V waves are seen with constrictive pericarditis and tall a waves but not cannon a waves are seen
with Tricuspid stenosis.
Nitric oxide:
A is generated from glutamine (False)
B is produced by both inducible and constitutive forms of nitric oxide synthetase (True)
C raises systemic vascular resistance (False)
D is inactivated by oxygen free radicals (True)
E is increased by cyclic AMP activation. (False)
Comments:
Nitric oxide is produced from l-arginine by nitric oxide synthase and is produced by the vascular
endothelium in response to haemodynamic stress and produces smooth muscle relaxation and reduced
vascular resistance. Nitric oxide is a free radical and may be inactivated through interaction with other
oxygen free radicals e.g. oxidised LDL. It causes the production of cGMP as a second messenger.
The following conditions are inherited as an autosomal dominant trait:
A Friedrich's ataxia (False)
B Ehlers-Danlos syndrome (True)
C Noonan's syndrome (True)
D Romano-Ward syndrome (True)
E Marfan's syndrome (True)
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Comments:
a-Autosomal recessive and associated with cardiomyopathy (Modes of inheritance questions are very
common in the MRCP part 1 exam). b-Associated with aortic aneurysm. c-Chromosome 12. Associated
with pulmonary stenosis, short stature, webbing of the neck, undescended testes in boys, HOCM, ASD
(more ...) d-Associated with long QT (Jervell-Lange-Neilson syndrome is Autosomal Recessive). e-
Associated with mitral valve prolapse, aortic aneurysm and aortic regurgitation.
The following findings may occur with a cyanotic episode in an individual with
Fallot's tetralogy:
A bradycardia (False)
B pallor (False)
C increased systolic murmur (False)
D loss of consciousness (True)
E continuous murmur at the upper left sternal border (True)
Comments:
B- Usually polycythaemic and cyanosed. The Murmur may becoma softer during a cyanotic episode and
syncope can occur. The continuous murmur may still be audible. (Dr Jacob Easaw)
Which of the following features would be expected in a 17 year old male with
pre-excitation tachycardia of Wolff-Parkinson-White syndrome?
A Narrow complex tachycardia is usually regular (True)
B Amiodarone is contraindicated (False)
C Digoxin is contraindicated (True)
D Verapamil is useful in irregular broad-complex tachycardia (False)
E VF is always stopped by amiodarone (False)
Comments:
There is typically a narrow complex tacchycarida with a characterisitic short PR and delta wave present
between episodes. Amiodarone can be used as a treatment but the most appropriate therapy is
destruction of the accessory pathway with frequency ablation. Digoxin is not useful as a single drug since
it shortens refractory period in the accessory pathway and speeds up ventricular response during AF and
like verapamil may precipitate VF.
Hypertrophic obstructive cardiomyopathy is associated with:
A a double apical impulse (True)
B decreased left ventricular ejection fraction (False)
C left ventricular diastolic dysfunction (True)
D increased left ventricular outflow obstruction (True)
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E mitral regurgitation (True)
Comments:
Hypertrophic cardiomyopathy is defined as the unexplained, asymmetical or concentric hypertrophy of
the undilated left ventricle. There is also hypertrophy of the right ventricle. It may be inherited as an
autosomal dominant condition, but at least half of cases may be the result of sporadic mutation. Double
apical impulse may be felt, with diastolic dysfunction being typical. Outflow obstruction develops over
time associated with increasing hypertrophy and associated mitral regurgitation is common.
The following are recognised determinants of systemic blood pressure in
childhood:
A Systolic blood pressure increases with age. (True)
B Systemic hypertension is commoner in girls. (False)
C Diastolic blood pressure correlates with height. (True)
D Hypertension is defined as systolic blood pressure greater than the 99th
percentile for age.
(False)
E In children under the age of 5, the majority will have secondary hypertensions. (True)
Comments:
Blood pressure is the product is the product of peripheral vascular resistance and cardiac output.
Accurate measurement is dependent on the quality of the equipment and the skill of the observer.
Anxiety may raise the initial level. Careful attention is required to cuff size. The bladder of the pressure
cuff should nearly encircle the upper arm, but the ends should not overlap. The cuff should cover at least
two thirds of the length of the arm. Systolic blood pressure is indicated by the first carotid sound, and in
pre-adolescence the diastolic by the fourth sound. Systemic blood pressure gradually increases with age
and correlates with weight and height. Hypertension is defined as a blood pressure consistently above
the 95th centile for age. The younger the child the more likely there is to be a secondary cause for the
raised blood pressure. In the majority of these cases, this will be due to renal disease (e.g. renal artery
stenosis, reflux nephropathy). Coarctation of the aorta and neuroendocrine causes should also be
considered.
Copyright 2002 Dr Colin Melville
The following are recognised features of cardiac failure in the infant:
A Jaundice. (False)
B Splenomegaly. (True)
C Raised jugular venous pulse. (False)
D Pallor. (True)
E Grunting. (True)
Comments:
B, D, E 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
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34
assess reliably, and jaundice does not occur.
Copyright 2002 Colin Melville
Paradoxical (reverse) splitting of the second heart sound is typical of:
A Ostium primum ASD. (False)
B Pulmonary stenosis. (False)
C Aortic stenosis. (True)
D Left bundle branch block. (True)
E Ostium secundum ASD. (False)
Comments:
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
The following are characteristic of supraventricular tachycardia in a 6 month
old child:
A A cardiac rate of 190/min (True)
B Sudden collapse (False)
C Hepatomegaly (True)
D A cardiac rate of 200/min that increases with crying (True)
E Irritability (True)
Comments:
SVT is usually caused by a re-entry within the AV node. The attacks are characteristically abrupt in onset
and cessation, and may be precipitate by acute infection. They usually occur when the patient is at rest.
In infants, the heart rate is usually above 200, but in older children usually exceeds 180/min. The only
complaint may be awareness of a rapid heart rate, and this is usually well tolerated. If the rate is
exceptionally rapid or the attack is prolonged then precordial discomfort in congestive heart failure may
supervene. In the infant whose attack lasts 6-24 hours with an extreme heart rate, the child may become
acutely ill with a poor colour, irritability, tachypnoea, and hepatomegaly. There may be fever and
leukocytosis. In the fetus, there may be hydrops fetalis. SVT is usually associated with an anatomically
normal heart, or may be associated with a bypass tract in one of the pre-excitation syndromes (Wolff-
Parkinson-White, Lown-Ganong-Levine). Occasionally, it occurs in association with Ebstein's anomaly, or
corrected transposition of the great arteries. It may be precipitated by exposure to
sympathomiemeticamines (e.g. pseudoephedrine in cold cures).
Copyright 2002 Dr Colin Melville
The following are found in ostium secundum ASD:
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A Cardio-thoracic ratio of 60% with pulmonary bay. (False)
B Superior axis deviation on ECG. (False)
C Grade 1/6 diastolic murmur at the lower left sternal edge. (True)
D Cardiac arrhythmias in later life. (True)
E A fixed widely split second heart sound. (True)
Comments:
A pulmonary bay suggests small pulmonary arteries, as in Fallot's. Superior axis deviation may be found
in primum ASD. A diastolic tricuspid rumble is due to increased blood flow, and suggest a large shunt.
Fixed splitting is due to equal left and right atrial pressure throughout the respiratory cycle.
Copyright 2002 Dr Colin Melville
Cardiac output is altered by the following factors:
A reduction during sleep (True)
B cardiac output is altered by moderate changes in environmental temperature (False)
C standing from a lying position decreases the cardiac output (False)
D histamine increases the cardiac output (True)
E eating decreases the cardiac output (False)
Comments:
The fundamental equation is BP=COxTPR. Sleeping is associated with reduced metabolic requirements,
decreased HR, BP and hence reduced CO. Output is affected by changes in the temp of the individual
but not by small changes in environmental temp. The orthostatic response is associated with an increase
in CO. Histamine is associated with vasodilatation and hence increased CO. Post-prandially CO is
increased by 30%
Regarding infective endocarditis:
A The commonest causative organism is Group A Streptococcus. (False)
B Lesions are most frequent in areas of high velocity blood flow. (True)
C Amoxycillin is first choice for prophylaxis in procedures done under local
anaesthetic.
(True)
D Presentation in infancy is extremely rare. (True)
E Splenomegaly is a subtle early sign. (False)
Comments:
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
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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