Cardiology Exam Qs 2004-1999 Flashcards Preview

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Flashcards in Cardiology Exam Qs 2004-1999 Deck (72):
1

Idiopathic ascending aortic dilatation would be most likely to accompany which of the following syndromes/associations?


A. Down syndrome.
B. Noonan syndrome.
C. Turner syndrome.
D. VACTERL association.
E. Velocardiofacial syndrome. 

C. Turner syndrome. Coarct of aorta, bicuspid aortic valve, partial anomalous venous drainage, hypertension, aortic dilatation.

Downs - AVSD
Noonans - Pulmonary stenosis 50%, ASD
VACTERL assoc - VSD, ASD and TOF.
Vertebral, Anal, Cardiac, Tracheal Esophageal, Renal, Limb defects
VCF - TOF
 

2

A rhythm strip from an electrocardiogram (ECG) is shown above. Which of the following is the most likely cause for the abnormality displayed?


A. Hyperkalaemia.
B. Hyperthermia.
C. Hyperthyroidism.
D. Hypocalcaemia.
E. Uraemia. 

D. Hypocalcaemia.

Prolonged QT, occurs in hypocalcaemia.
Hyperkalemia - flattened P, wide QRS, tall T
Hyperthermia - tachycardia
Uraemia - causes hyperkalemia

3

An ambulance attends a 13-year-old girl who collapses at school and is unconscious for a brief period. Witnesses to the event said that she had some brief jerking of her limbs when on the ground. By the time she is brought to the emergency room she is back to normal. On further questioning, there have been three previous events.
An association with which of the following is most likely to raise concerns that the diagnosis is not simple syncope?
A. Exercise.
B. Mild trauma.
C. Prolonged standing.
D. Venipuncture.
E.Vomiting. 

A. Exercise.

4

The diagram shown represents the normal cardiac cycle. At which of the points marked on the diagram would the left ventricular volume be greatest?
A. A.
B. B.
C. C.
D. D.
E. E. 

A. A. Atrium is contracting into relaxed ventricle.

5

 A 10-year-old boy presents with a history of recurrent palpitations, precipitated by strenuous exercise, over the last 12 months. On the day of presentation he had the sudden onset of a rapid heart rate while playing cricket at school. On arrival at triage he has a pulse rate of 240/minute, a respiratory rate of 29/minute and a blood pressure of 80/50 mmHg. He is pale but alert and after a large vomit at triage his rapid heart rate spontaneously resolves. His 12-lead electrocardiogram (ECG) is shown opposite.

An echocardiogram is most likely to show which of the following?

A. Dilated cardiomyopathy.
B. Ebstein anomaly.
C. l-Transposition of the great arteries (corrected transposition).
D. Mitral valve prolapse.
E. Normal cardiac anatomy. 

E. Normal cardiac anatomy

6

A seven-year-old girl presents with sudden onset at rest of sharp left-sided chest pain, with no associated palpitations. There is a family history of sudden death from cardiac causes. A 12-lead electrocardiogram (ECG) is shown opposite.

Which of the following best describes the ECG?

A. Normal sinus rhythm.
B. Normal sinus rhythm with atrial ectopics.
C. Second degree heart block - Mobitz type I.
D. Second degree heart block - Mobitz type II.
E. Sinus arrhythmia. 

E. Sinus arrhythmia. 

7

The ratio of systemic to pulmonary vascular resistances changes postnatally. What changes would be expected to systemic vascular resistance (SVR) and pulmonary vascular resistance (PVR) one day after normal delivery of a term infant in comparison to antenatal values? 

C. Increase SVR, Decrease PVR.
Removal of low resistance placental circulation leads to rise in SVR.

Mechanical expansion of lungs + increase in arterial pO2 leads to decrease in PVR (as both mechanisms cause pulmonary vasodilation).

8

The echocardiograms shown above were obtained from an otherwise healthy three-month-old infant presenting for assessment of a murmur. They show multiple intraventricular lesions.
Which of the following is the most likely diagnosis?
A. Neurofibromatosis type 1.
B. Noonan syndrome.
C. Proteus syndrome.
D. Sturge-Weber syndrome.
E. Tuberous sclerosis.

E. Tuberous sclerosis.
Echo shows rhabdomyomas. Occurs in 50% of children with TSC.

TSC also has hypomelanotic skin lesions, shagreen patches, periungal fibromas, "dripping candle" lesions in brain ventricles.

Hallmark is involvement of CNS.
Most common CNS manifestations - epilepsy, cognitive impairment, autism spectrum disorders, infantile spasms (rx with vigabactin), hypsarrhythmic EEG.
From Nelsons.

West Syndrome- Triad of Infantile spasms, hypsarrhythmic EEG, and developmental regression. Only need two of these for diagnosis.

9

A 24-year-old pregnant woman reports at her first antenatal visit that her sister gave birth to a child with congenital heart block (CHB). Which one of the following tests would be most useful in assessing the risk of CHB in this pregnancy?

A. Antibodies to DNA.
B. Antibodies to extractable nuclear antigens (ENA).
C. Anticardiolipin antibodies.
D. Fluorescent antinuclear antibody (ANA) test.
E. Lupus anticoagulant.

B. Antibodies to extractable nuclear antigens (ENA).
Neonatal Lupus is responsible for 60-90% of congenital heart block. Caused by maternal antibodies (anti-Ro/SSA and/or anti-La/SSB = extractable nuclear antigens ENA) that cross the placenta.


PATHOPHYSIOLOGY
In most cases, congenital complete heart block is characterized pathologically by fibrous tissue that either replaces the AV node and its surrounding tissue or by an interruption between the atrial myocardium and the atrioventricular node (AV node); other lesions that can occur include congenital absence of the AV node. The net effect is that the block is usually at the level of the AV node. The heart is otherwise normal pathologically in children with neonatal lupus and certain other disorders.

Among infants with CHD due to neonatal lupus, anti-Ro/SSA and/or anti-La/SSB antibodies bind to fetal cardiac tissue, leading to autoimmune injury of the atrioventricular (AV) node and its surrounding tissue. Both Ro/SSA and La/SSB antigens are abundant in fetal heart tissue between 18 and 24 weeks. Apoptosis induces translocation of Ro/SSA and La/SSB to the surface of fetal cardiomyocytes; anti-Ro and anti-La antibodies then bind to the surface of the fetal cardiocytes and induce the release of tumor necrosis factor by macrophages, which then results in fibrosis.

In addition to inducing tissue damage, anti-Ro/SSA and/or anti-La/SSB antibodies inhibit calcium channel activation or the cardiac L- and T- type calcium channels themselves; L-type channels are crucial to action potential propagation and conduction in the AV node.
Up to Date
 

10

An infant is noted on day 2 of life to be cyanosed. The arterial oxygen saturation is 88% in room air. The remainder of the examination is normal, including normal cardiac and respiratory examinations.
Which of the following is the most likely diagnosis?

A. Alveolar capillary dysplasia.
B. Extralobar sequestration.
C. Tetralogy of Fallot.
D. Totally anomalous pulmonary venous return.
E. Transposition of the great arteries.

D. Totally anomalous pulmonary venous return.
Alveolar capillary dysplasia - very rare. Assoc with unresponsive Pulmonary hypertension within hours of delivery.
Extralobar sequestration (bronchopulmonary sequestration) is a rare congenital malformation of the lower respiratory tract. It consists of a nonfunctioning mass of lung tissue that lacks normal communication with the tracheobronchial tree, and that receives its arterial blood supply from the systemic circulation. Presents with respiratory distress.
TOF - Ejection systolic murmur
TAPVR - ESM, diastolic rumble, tachypnoea
TGA - severe cyanosis unresponsive to O2 unless L-R communication (causes murmur) 

11

An asymptomatic four-year-old boy is referred to you for assessment. He had previously undergone an echocardiogram, which was reported to show an isolated, restrictive, perimembranous ventricular septal defect (VSD) and a left to right shunt across the VSD with a pressure gradient of 90 mmHg. His blood pressure was 110/60 mmHg. His electrocardiogram (ECG) is shown below.

 

Which of the following conclusions is most justifiable based upon the available data?
A. The ECG is consistent with the echocardiogram and the VSD is large.
B. The ECG is consistent with the echocardiogram and the VSD is small.
C. The ECG is not consistent with the echocardiogram and there may be unrecognised left heart obstruction.
D. The ECG is not consistent with the echocardiogram and there may be
unrecognised right heart obstruction.
E. The ECG should be repeated as the limb leads are crossed.

D. The ECG is not consistent with the echocardiogram and there may be
unrecognised right heart obstruction.
ECG shows RVH

12

A nine-year-old boy presents to the emergency department with chest pain. He had cardiac surgery in infancy, but the parents are vague about the diagnosis. His electrocardiogram (ECG) is shown opposite.
The most likely cardiac lesion was:
A. atrioventricular canal defect.
B. hypoplastic right ventricle.
C. perimembranous ventricular septal defect.
D. tetralogy of Fallot.
E. truncus arteriosus.

A. AV canal defect

13

The electrocardiogram (ECG) shown above was obtained from a three-year-old girl with a systolic murmur.
Which one of the following is the most likely diagnosis?


A. Perimembranous ventricular septal defect.
B. Persistent ductus arteriosus.
C. Primum atrial septal defect.
D. Pulmonary valve stenosis.
E. Subaortic stenosis. 

C. Primum atrial septal defect.

M 'RSR' pattern in V1 characteristic of primum ASD. 

14

In the diagrammatic representation of the cardiac cycle shown above, the aortic trace is represented by ‘Ao’ and the left ventricular trace by ‘V’. Which one of the periods represented at the bottom of the diagram best corresponds to the period of isovolumetric contraction?
A. Period A.
B. Period B.
C. Period C.
D. Period D.
E. Period E. 

B. Period B.

No volume changes in B as ventricular contraction but no blood flow into aorta at that stage.

15

A 10-year-old boy presents with a history of recurrent palpitations, precipitated by strenuous exercise, over the last 12 months. On the day of presentation he had the sudden onset of a rapid heart rate while playing cricket at school. On arrival at triage he has a pulse rate of 240/minute, a respiratory rate of 29/minute and a blood pressure of 80/50 mmHg. He is pale but alert and after a large vomit at triage his rapid heart rate spontaneously resolves. His 12-lead electrocardiogram (ECG) is shown above.

An echocardiogram is most likely to show which of the following?

A. Dilated cardiomyopathy.
B. Ebstein anomaly.
C. l-Transposition of the great arteries (corrected transposition).
D. Mitral valve prolapse.
E. Normal cardiac anatomy. 

E. Normal cardiac anatomy.

16

In a patient with a ventricular septal defect (VSD), which of the following clinical signs best correlates with a pulmonary to systemic blood flow ratio (Qp/Qs) of greater than 2:1?
A. Apical mid-diastolic murmur.
B. Loud ejection systolic murmur.
C. Loud pulmonary component of the second heart sound.
D. Prominent systolic thrill.
E. Reversed splitting of the second heart sound.

A. Apical mid-diastolic murmur.

Diastolic murmurs in infancy usually = L to R shunting.
Diastolic rumble due to increased flow from shunt across mitral valve.
25% shunt = 1.33 fold increase in LV output
50% shunt = 2 fold increase in LV output
75% shunt = 4 fold increase in LV output
80% shunt = 5 fold increase in LV output

17

The electrocardiogram (ECG) of an asymptomatic four-year-old girl who presented with a systolic murmur is illustrated above. She was acyanotic. Precordial examination revealed a single loud second heart sound and a soft systolic ejection murmur at the left sternal edge.
Which one of the following is the most likely diagnosis?
A. Eisenmenger syndrome.
B. l-Transposition of the great arteries (corrected transposition).
C. Primary pulmonary hypertension.
D. Pulmonary stenosis (valvular).
E. Tetralogy of Fallot (acyanotic). 

B. l-Transposition of the great arteries (corrected transposition).
All other answers would cause RVH which is not evident on ECG.

18

A two-year-old girl is known to have doubly committed (supracristal, conal) ventricular septal defect. Over the six months prior to review her mother reports a significant deterioration in exercise tolerance.
The most likely aetiology of the progressive fatigue is:
A. aortic regurgitation.
B. bacterial endocarditis.
C. hypocalcaemia
D. increasing left to right shunt.
E. mitral regurgitation.

A. aortic regurgitation.
Pathophysiology: The unique location of the supracristal ventricular septal defect with its close proximity to the aortic root accounts for the common development of aortic insufficiency with this defect. Left-to-right shunting of blood through the defect is believed to progressively pull aortic valve tissue (especially the right coronary cusp) through a Venturi effect.
Exercise intolerance and dyspnea suggest progressive aortic insufficiency.
Surgical closure recommended in most cases.

19

A newborn infant is noted to have a rapid pulse but is not compromised. The electrocardiogram (ECG) shown above demonstrates long periods of tachycardia with intermittent sinus beats.
Which one of the following is the most appropriate course of action?
A. Direct current (DC) cardioversion.
B. Facial ice water.
C. Intravenous adenosine.
D. Intravenous sotalol.
E. Intravenous verapamil.

D. Intravenous sotalol.

DC current if compromised or in shock.

Facial ice water and IV adenosine are for SVT.

IV verapamil is unsafe and uncommon. Last resort Rx in SVT and given PO.

20

A 2.5 kg, one-week-old infant with Down syndrome is known to have a complete atrioventricular canal. He is feeding well. He has a respiratory rate of 40/minute, no evidence of respiratory distress and an arterial oxygen saturation of 91% in room air.
Which one of the following is the most appropriate course of action?
A. Administer oxygen.
B. Commence diuretics.
C. Observation.
D. Refer for pulmonary artery band.
E. Refer for surgical correction. 

C. Observation.

Medical management for heart failure first. Includes diuretics. Surgical correction less than 6 months.

21

An 18-month-old boy presents with a history of two episodes of having suddenly collapsed to the ground while running. He was found to be pallid on both occasions, with no spontaneous movements and no apparent respirations. On both occasions his parents gave him cardio-pulmonary resuscitation. Normal respiration was restored after a few minutes, and he was tired for some hours after each event. After the second occasion, he was admitted to hospital for observation and investigation. He has a normal developmental history and has never had any serious illnesses recognised. There is no family history of epilepsy.
This clinical presentation would be most consistent with:
A. atonic seizures.
B. breath holding episodes.
C. cardiac arrhythmia.
D. exercise-induced anaphylaxis.
E. hypoglycaemia.

C. cardiac arrhythmia.

22

A 13-year-old boy presents to the emergency department with sudden-onset left-sided chest pain and vomiting. He has a history of a double outlet right ventricle, which was repaired in infancy with an intracardiac baffle.

On examination his pulses are barely palpable, his heart rate is 190/minute, respiratory rate is 30/minute and systolic blood pressure is 70 mmHg. He is alert but agitated and poorly perfused peripherally with a capillary refill time of 4 seconds. A 12-lead electrocardiogram (ECG) is shown above.
Which of the following is the most appropriate acute management?
A. Elective cardioversion after prior endotracheal intubation.
B. Intravenous amiodarone.
C. Intravenous lignocaine.
D. Rapid intravenous bolus of adenosine.
E. Urgent cardioversion with prior sedation.

E. Urgent cardioversion with prior sedation.

Unstable VT

23

A term newborn infant is noted to be episodically cyanotic. On day 3, he has a normal physical examination with a resting arterial oxygen saturation of 96% in room air. Periodically his saturation is noted to fall to 80%.
Which one of the following is the most likely diagnosis?
A. Apnoea.
B. Seizures.
C. Tetralogy of Fallot.
D. Totally anomalous pulmonary venous return.
E. Transposition of the great arteries.

A. Apnoea.

24

A child is known to have pulmonary atresia. The oxygen saturations obtained at cardiac catheterisation are displayed on the diagram shown below.

What is the ratio of pulmonary to systemic blood flow (Qp:Qs ratio) based on the data supplied?
A. 0.2:1
B. 1:1
C. 4:1
D. 5:1
E. Incalculable based on the data supplied.

C. 4:1.
QP:QS = (Sat Ao - Sat MV)/(Sat LA - Sat PA)
MV = mixed venous
Minimal PA supply from RV due to atresia therefore most blood supply from PDA so Sat PA is 90%.
QP:QS = 90-66/96-90 = 24/6= 4/1
A QP:QS of 1:1 is normal heart. A QP:QS of <1:1 is a right to left shunt.

25

The rhythm strip shown above was obtained from an otherwise well five-year-old who had been noted to have an irregular pulse. Which one of the following is the most likely diagnosis?

A. Blocked atrial ectopics.
B. Complete heart block.
C. Mobitz type I block (Wenkebach).
D. Mobitz type II block.
E. Sinus arrhythmia.

C: Mobitz type I block

ECG shows PR intervals increasing until P is not followed by QRS.

First degree heart block = increased PR interval with NO DROPPED QRS

Second degree (Mobitz) Type I (Wenkebach) = increasing PR until QRS dropped

Second degree (Mobitz) Type II = constant increased PR interval until QRS dropped.

Third degree = no relationship between the P and QRS.

Mobitz Type II and third degree = admission due to danger of progression to VT/VF. Need immediate pacemaker.

26

Following tetralogy of Fallot repair a child is noted to have a widely split second heart sound.
The most likely aetiology for this is:

A. anomalous pulmonary venous return.
B. aortic stenosis.
C. left superior vena cava draining to the coronary sinus.
D. residual patent foramen ovale.
E. right bundle branch block.

E. RBBB
A complication of TOF repair is RBBB. This can cause widely split second heart sound.
Widely split second heart sound detected by presence of splitting during expiration, wider during inspiration.

27

The electrocardiogram (ECG) shown above was obtained from a three-year-old girl with a systolic murmur. Which one of the following is the most likely diagnosis?

A. Perimembranous ventricular septal defect.
B. Persistent ductus arteriosus.
C. Primum atrial septal defect.
D. Pulmonary valve stenosis.
E. Subaortic stenosis.

Primun ASD.
RSR (M) in V1 is characteristic of RBBB pattern.
Primum ASD causes RBBB pattern.
Perimembranous VSD causes LVH or Biventricular hypertrophy
PDA causes continuous machinery murmur both systolic and diastolic. No classical changes in ECG.
Pulmonary valve stenosis = RVH
Subaortic = LVH

28

Which one of the following cardiac conditions is most commonly associated with neurofibromatosis?

A. Aortic root dilatation. 
B. Cardiac rhabdomyomata. 
C. Coarctation of the aorta. 
D. Pulmonary stenosis. 
E. Supravalvular aortic stenosis. 

D. Pulmonary stenosis. Type I Neurofibromatosis

 

A. Aortic root dilatation. Marfans

B. Cardiac rhabdomyomata. Tuberous Sclerosis Complex

C. Coarctation of the aorta. Turners Syndrome

D. Pulmonary stenosis. Type I Neurofibromatosis

E. Supravalvular aortic stenosis. Williams Syndrome

 

29

A two-month-old boy presents with tachypnoea and poor feeding. He is afebrile. His respiratory rate is 65/minute with mild intercostal recession, his heart rate is 180/minute and his liver is palpable 4 cm below the right costal margin. No other abnormalities are identified on clinical examination. His electrocardiogram (ECG) is shown below.


Which one of the following is the most likely diagnosis?
A. Anomalous left coronary artery.
B. Dilated cardiomyopathy.
C. Hypertrophic cardiomyopathy.
D. Myocarditis.
E. Supraventricular tachycardia.

 

E. Supraventricular tachycardia.
Narrow QRS.

  • Anomalous left coronary artery - unable to find classic finding.
  • HOCM -Asymmetrical septal hypertrophy produces deep, narrow (“dagger-like”) Q waves in the lateral (V5-6, I, aVL) and inferior (II, III, aVF) leads.
  • Dilated cardiomyopathy - no typical changes but may include left atrial enlargement, LVH
  • Myocarditis - diffuse T wave inversions; saddle-shaped ST-segment elevations

30

A foetus is found to have an isolated echogenic focus in the left ventricle on a routine 18-week
ultrasound. Which one of the following is the most likely explanation for this finding?

A. A variant of normal.
B. Down syndrome.
C. Neurofibromatosis.
D. Papillary muscle myxoma.
Tuberous sclerosis (rhabdomyoma).

A. A variant of normal.

31

An asymptomatic infant undergoing a routine discharge check on day two is noted to have a continuous murmur. His arterial oxygen saturation is 88% in room air.

Which one of the following is the most likely diagnosis?

A. Coronary artery fistula.
B. Persistent ductus arteriosus.
C. Pulmonary atresia and ventricular septal defect.
D. Ruptured sinus of Valsalva. Catastrophic
E. Transposition of the great arteries.
 

C. Pulmonary atresia and ventricular septal defect.

  • Coronary artery fistula- Continuous murmur.
  • PDA - Continuous machinery murmur. Can be cyanotic beyond level of PDA
  • Pulmonary atresia and ventricular septal defect. Survival depends on ductus remaining open. Cyanotic shortly after birth.
  • TGA-Catastophic. Symptomatic, most common cyanotic heart disease. Cyanosis begins within hours of birth as PDA closes.

32

A four-month-old infant known to have tetralogy of Fallot presents to the emergency department with cyanotic episodes associated with crying.
Which one of the following is the most useful indicator of whether these episodes are representative of hypercyanotic (tetralogy) spells?

A. Baseline arterial saturation.
B. Echocardiographic gradient across the right ventricular outflow tract at rest.
C. Echocardiographic gradient across the right ventricular outflow tract during a cyanotic episode.
D. Intensity of the murmur during a cyanotic episode.
E. Oxygen responsiveness during a cyanotic episode.

D. Intensity of the murmur during a cyanotic episode.

During Tet spell, pulmonary resistance increases and peripheral vascular resistance decreases so VSD becomes right to left shunt causing cyanosis. VSD usually left to right so sound of murmur decreases with decreased flow across VSD. Tet spells occurs with crying, stress and waking from sleep.

33

A six-month-old boy with Down syndrome was diagnosed at birth with a complete atrioventricular
canal defect. He is asymptomatic, growing appropriately and has normal oxygen saturation in room air. On examination he has a right ventricular heave and a single second heart sound of increased intensity. His electrocardiogram (ECG) is shown below.

Which one of the following is the most appropriate course of action?

A. Instigate captopril therapy.
B. Instigate digoxin therapy.
C. Instigate diuretic therapy.
D. Refer for surgical correction.
E. Review in three months.

D. Refer for surgical correction.

Medical management initially to stabilise heart failure prior to surgery. Pts usually present at approx 6 weeks with heart failure. Rx includes diuretics, ionotropes (digoxin), ACE-I.

Elective surgical repair is recommended prior to six months of age in order to avoid the development of pulmonary vascular disease (PVD). Earlier repair should be considered in infants with heart failure who are unresponsive to medical management, symptomatic infants with significant AV valve regurgitation, or those with aortic coarctation. However, surgeons generally consider that optimal valve construction is more easily performed in patients who are older than six weeks [4].

In patients with Down syndrome, heart failure symptoms may not develop, probably due to persistent elevation of pulmonary vascular resistance from birth. Nevertheless, elective repair is recommended prior to six months of age regardless of whether symptoms are present or not because of concerns of developing PVD. Long-term outcome is similar in patients with Down syndrome repaired during infancy compared with patients without Down syndrome

34

Four subtypes of long QT (LQT) syndrome have been fully characterised: LQT1, LQT2, LQT3 and LQT5.
What is the abnormality in the cardiac action potential which results in QT prolongation in LQT1, LQT2 and LQT5?
A. Prolonged calcium efflux.
B. Prolonged calcium influx.
C. Prolonged potassium efflux.
D. Prolonged sodium efflux.
E. Prolonged sodium influx.


 

Prolonged potassium efflux.
LQT3 and LQT10 are Na+ efflux

35

Following tetralogy of Fallot repair a child is noted to have a widely split second heart sound.
The most likely aetiology for this is:
A. anomalous pulmonary venous return.
B. aortic stenosis.
C. left superior vena cava draining to the coronary sinus.
D. residual patent foramen ovale.
E. right bundle branch block.

 

E. RBBB

A complication of TOF repair is RBBB. This can cause widely split second heart sound.
Widely split second heart sound detected by presence of splitting during expiration, wider during inspiration.

36

A child is known to have pulmonary atresia. The oxygen saturations obtained at cardiac catheterisation are displayed on the diagram shown below.

What is the ratio of pulmonary to systemic blood flow (Qp:Qs ratio) based upon the data supplied?
A. 0.2:1
B. 1:1
C. 4:1
D. 5:1
E. Incalculable based upon the data supplied.

C. 4:1.

QP:QS = (Sat Ao - Sat MV)/(Sat LA - Sat PA)
MV = mixed venous

Minimal PA supply from RV due to atresia therefore most blood supply from PDA so Sat PA is 90%.

QP:QS = 90-66/96-90 = 24/6= 4/1

A QP:QS of 1:1 is normal heart. A QP:QS of <1:1 is a right to left shunt.

37

The following data were obtained at cardiac catheterisation from a patient with a ventricular septal defect.

Site                                  Oxygen Saturation
Superior vena cava      70%
Inferior vena cava         70%
Right atrium                   70%
Left pulmonary artery   85%
Left atrium                       95%
Aorta                                95%
Oxygen consumption   150 mL/minute
Oxygen content             100 mL/L

The cardiac output is:
A. 4 L/minute.
B. 6 L/minute.
C. 8 L/minute.
D. 10 L/minute.
E. incalculable.

B. 6 L/minute.

Cardiac Output = Oxygen consumption/Oxygen content arterial blood (aorta) - oxygen content venous blood (SVC &IVC)

CO=150/95-70 = 150/25 =6

38

A two-month-old girl had a murmur identified at birth. To your assessment now, she is pink and thriving. She shows no signs of respiratory distress or other signs of heart failure. The S2 is single and there is a long systolic murmur heard loudest at the left sternal edge. Her chest X-ray is normal and her ECG is shown above.
Which one of the following is the most likely diagnosis?
A. Aortic stenosis.
B. Atrial septal defect.
C. Physiological peripheral pulmonary stenosis.
D. Small ventricular septal defect.
E. Tetralogy of Fallot

 

E. Tetralogy of Fallot.

ECG shows RVH.
Aortic stenosis =LVH, ASD = no changes, physiol perip. pulm. stenosis shouldn’t cause ECG changes, small VSD wouldn’t cause RVH.

39

An eight-year-old girl presents with a six-month history of central chest pain. She describes the pain as squeezing in nature. The pain occurs most days and can occur both at rest and with exercise. She is a competitive swimmer and has not noted the pain whilst racing. As an infant she was diagnosed on clinical grounds with a small muscular ventricular septal defect but is now clinically normal.
Echocardiography is most likely to show which one of the following?
A. Aberrant origin of the left anterior descending coronary artery from the right coronary artery.
B. Anomalous left coronary artery.
C. L-transposition of the great arteries.
D. Normal heart.
E. Single coronary artery with a branch crossing between the great arteries

 

D. Normal heart. Pain is not occurring during racing - unlikely to be anatomical pathology

40

A child weighing 24 kg with a known, small, perimembranous ventricular septal defect is to undergo routine isolated dental ‘cleaning and scaling’.
Assuming the child is not allergic to penicillin, which one of the following is recommended in relation to bacterial endocarditis prophylaxis?

A. Amoxycillin 1.2 g, per oral, administered one hour before the procedure only.

B. Amoxycillin 600 mg, intravenously, administered one hour before the procedure and repeated six hours after the procedure.

C. Amoxycillin 600 mg, q8h, per oral, for 48 hours prior to the procedure.

D. Antibiotic prophylaxis is not recommended for this procedure.

E. Phenoxymethylpenicillin 1.2 g, per oral, administered one hour before the procedure. 

A. Amoxycillin 1.2 g, per oral, administered one hour before the procedure only.

But guideline is now IV


Antibiotic Guidelines Endocarditis Prophylaxis
Infective Endocarditis in Children with Heart "Defects"
Patients "At Risk":


Low Risk:-


  • *All children with Structural Congenital Heart Defects (except isolated ASD secundum?)
  • *All children with OPERATED heart defects (except repaired ASD secundum and DA)
  • *Children with acquired Valve Disease
  • *Children with Endocardial Pacemakers  

High Risk:-


  • Unrepaired cyanotic heart defects
  • Previous Endocarditis
  • Prosthetic valves, Conduits, 'Shunts'

Patients not at risk:

  • Children with murmurs if confirmed "Innocent"
  • Children with non-structural problems (eg. Arrhythmia)
  • Children with history of Kawasaki's disease

*Subject to advice from patient's cardiologist





Endocarditis Prophylaxis


Dental Procedures and procedures on mouth, skin or musculoskeletal structures where a bacteraemia may occur.

  • Low Risk: Antibiotic cover is no longer recommended as a routine in low risk patients.


  • High risk: (eg Prosthetic Valve/Previous endocarditis/Cyanotic heart defect)

Amp/Amoxycillin 50 mg/kg IV pre-op (at induction - max 2 gm) Single dose only


If Penicillin sensitive:
Vancomycin - 20 mg/kg IV infused over 1 hr pre-op (max 1 gm)

Note: Antibiotic cover for genito-urinary or gastro-intestinal procedures is not recommended for prophylaxis of endocarditis.

41

A three-day-old baby, not obviously dysmorphic and weighing 3 kg, presents with mild respiratory distress and a heart murmur. Subsequent investigation demonstrates truncus arteriosus and low serum calcium.
Which one of the following is the most likely diagnosis?
A. Congenital rubella syndrome.
B. Foetal alcohol syndrome.
C. Foetal hydantoin syndrome.
D. Velocardiofacial syndrome.
E. Williams syndrome. 

D. Velocardiofacial syndrome.


Congenital Rubella Syndrome -
In neonates — Manifestations of congenital rubella infection (CRI) during the neonatal period may include:

  • Intrauterine growth retardation
  • Meningoencephalitis, large anterior fontanelle
  • Hearing loss
  • Cloudy cornea, cataract , infantile glaucoma, retinopathy
  • Interstitial pneumonia
  • Cardiac defects
  • Hepatosplenomegaly, jaundice, hepatitis, diarrhea
  • Radiolucent bone lesions (in the long bones)
  • Petechiae and purpura ("blueberry muffin lesions")
  • Adenopathy
  • Hemolytic anemia, thrombocytopenia

Foetal Alcohol Syndrome - Growth retardation and CNS abnormalities
 

Foetal Hydantoin Syndrome - (Phenytoin) - Orofacial clefts, cardiac malformations, and genitourinary defects are the major anomalies described with phenytoin
In utero exposure to phenytoin has been associated with the development of neuroblastoma in several case reports.
 

VCF Syndrome (AKA DiGeorge) - The classic triad of features of DGS on presentation is conotruncal cardiac anomalies, hypoplastic thymus, and hypocalcemia. Most common cardiac defects include

  • Interrupted aortic arch
  • Truncus arteriosus
  • Tetralogy of Fallot
  • Atrial or ventricular septal defects (ASD, VSD)
  • Vascular rings

 

Williams Syndrome -

  • “Elfin” facies
  • Supravalvular aortic stenosis or other vascular anomalies
  • Hypertension
  • Cognitive profile consisting of impaired cognition and development accompanied by a friendly, social personality
  • Short stature
  • Endocrine abnormalities including hypercalcemia, diabetes mellitus, and subclinical hypothyroidism
  • Genitourinary abnormalities include congenital anomalies of the kidney and urinary tract, nephrolithiasis due to hypercalciuria, and dysfunctional voiding
  • Auditory, dental, gastrointestinal, genitourinary, musculoskeletal, neurologic, ophthalmologic, and dermatologic abnormalities

42

 

A four-week-old infant presents with tachypnoea and failure to thrive. On examination the infant has a respiratory rate of 60/minute with subcostal recession. The pulses are full and the liver is palpable 4 cm below the right costal margin. The praecordium is active. On auscultation there is an audible ejection click and a non-specific systolic ejection murmur is noted at both upper sternal edges. The infant’s oxygen saturation in room air is 93%.
Which one of the following is the most likely diagnosis?
A. Persistent ductus arteriosus.
B. Pulmonary valve stenosis.
C. Truncus arteriosus.
D. Ventricular septal defect.
E. Ventricular septal defect and severe pulmonary valve stenosis.

C. Truncus arteriosus.

Pulmonary Valve Stenosis

  • A systolic ejection murmur with maximal intensity at the left upper sternal border. In patients with milder stenosis, the murmur will vary with respiration. The murmur radiates to the back.
  • A pulmonary ejection sound (or click).
  • Splitting of the second heart sound results from prolonged ejection and delay in the pulmonic component. The degree of splitting increases with more severe stenosis, and may become fixed in the most severe forms due to a fixed right ventricular output or development of conduction delay.
  • Cyanosis occurs only in the most severe cases, and is indicative of right to left shunting at the atrial level through a septal defect or patent foramen ovale.

Truncus Arteriosis

  • Most patients with TA present within the first weeks of life with cyanosis, respiratory distress due to pulmonary congestion and heart failure, and/or a heart murmur.
  • Cyanosis – Over the first days of life, mild or moderate cyanosis can be present and may be the only initial sign of TA. In some cases, cyanosis may not be discernible by physical examination but may be detected by pulse oximetry screening during the birth hospitalization.
  • Pulmonary congestion and heart failure – During the next several days to weeks as pulmonary arterial resistance falls, symptoms of pulmonary congestion and heart failure develop with unrestricted abnormally high pulmonary blood flow. Clinical features include a history of poor feeding and lethargy, signs of respiratory distress (tachypnea, costal-sternal retractions, grunting, and nasal flaring), tachycardia, hyperdynamic precordium, and hepatomegaly.


Cardiovascular findings – In unrepaired TA patients, the second heart sound is loud and single, but this may be hard to appreciate in a tachycardiac neonate. A prominent ejection click is frequently heard at the apex or left sternal border. There is usually a systolic ejection murmur at the left sternal border. Peripheral pulses are accentuated and often bounding. The pulse pressure usually is increased because of runoff into the pulmonary vascular bed during diastole.

In patients with tricuspid regurgitation due to an incompetent truncal valve, a diastolic high-pitched murmur may be heard along the left sternal border.

43

A one-month-old boy presents severely shocked. His ECG is shown below.

The most appropriate treatment is:
A. ice water immersion.
B. intravenous adenosine.
C. intravenous verapamil.
D. one-sided carotid body massage.
E. synchronised direct current (DC) shock.

E. synchronised direct current (DC) shock.

Pt is shocked therefore shock appropriate. QRS complexes present so synchronised shock appropriate.

44

An otherwise well infant is found to have a continuous murmur at the time of delivery. The infant’s oxygen saturation in room air is 99%.
The most likely diagnosis is:
A. coronary artery fistula.
B. patent foramen ovale.
C. persistent ductus arteriosus.
D. pulmonary atresia.
tetralogy of Fallot and absent pulmonary valve syndrome.

 

A. coronary artery fistula.

PDA -In the newborn, aortic systolic pressure is greater than pulmonary systolic pressure, but this gradient may not be present during diastole. As a result, the murmur may be confined to systole.

45

Question 3
The mean pulmonary artery (capillary) wedge pressure most closely approximates which one of the following pressures?
A. Left atrial mean.
B. Pulmonary artery diastolic.
C. Pulmonary vein wedge mean.
D. Right atrial mean.
Right ventricular end-diastolic.

A. Left atrial mean.

46

A male infant has pulmonary atresia/ventricular septal defect. After undergoing a systemic to pulmonary artery shunt his arterial saturation is 90%. Assuming a mixed venous saturation of 70% and a pulmonary venous saturation of 95%, the pulmonary to systemic flow ratio (Qp:Qs) is:
A. incalculable without a known haemoglobin.
B. incalculable without a known oxygen consumption.
C. 0.2:1.
D. 1:1.25.
E. 4:1

 

E. 4:1

QP:QS = Sat AO- Sat MV/Sat LA-Sat PA = 90-70/95-90=20/5=4
Systemic to pulmonary shunt so Sat aorta and pulm. artery both 90%

47

Which one of the following is an endothelium-derived vasodilator?
A. Angiotensin II.
B. Endothelin 1.
C. Prostacyclin.
D. Prostaglandin H2.
E. Thromboxane A2.

 

C. Prostacyclin

Prostacyclin (PGI2) chiefly prevents formation of the platelet plug involved in primary hemostasis (a part of blood clot formation). It does this by inhibiting platelet activation. It is also an effective vasodilator.

48

A one-month-old infant presents with failure to thrive. There is a three-week history of progressive tachypnoea, sweating and feeding difficulties. The respiratory rate is 60/minute with moderate recession. The liver is palpable 5 cm below the costal margin. The praecordium is active and a grade 3/6 systolic ejection murmur and a grade 3/6 apical mid-diastolic rumble are audible. An echocardiogram demonstrates a large, non-restrictive perimembranous ventricular septal defect.

Which one of the following most accurately reflects the anticipated pulmonary blood flow (PBF), pulmonary vascular resistance (PVR) and pulmonary artery systolic pressure (PSP)? 

C.  high   normal  systemic
Left to right shunt indicated by mid-diastolic rumble (increased flow across mitral valve). PBF high because of increased volume. PVR normal to allow for increased flow. Pulmonary artery systemic pressure is systemic because PA is receiving blood from left ventricle (ie. VSD has made two ventricles become one) so pressure is same as aortic pressure -systemic

49

In the neonatal syndrome of "persistent foetal circulation" (persistent pulmonary hypertension of the newborn), which one group of physiological ratios is usually seen?

Note:
PAP = pulmonary arterial pressure
SAP = systemic arterial pressure
QP = pulmonary blood flow
QS = systemic blood flow
RP = pulmonary arteriolar resistance
RS = systemic arteriolar resistance

A.  1    <1    >1

Pulmonary resistance is higher than systemic resistance in Pulmonary hypertension (RP/RS), consequently pulmonary blood flow is decreased.

50

The following data were obtained at cardiac catheterisation from a patient with a ventricular septal defect.

Site     Oxygen Saturation
Superior vena cava   70%
Inferior vena cava   70%
Right atrium    70%
Left pulmonary artery   85%
Left atrium    95%
Aorta     95%

Oxygen consumption   150 mL/minute
Oxygen content   100 mL/L

The cardiac output is:
A. 4 L/minute.
B. 6 L/minute.
C. 8 L/minute.
D. 10 L/minute.
E. incalculable.

B. 6 L/minute.

CO = O2 consumption/ arterial saturation - venous saturation

150/95% (aorta) - 70% (RV) = 150/25 =6L

51

A 10-year-old boy presents with a six-month history of progressive dyspnoea and palpitations. On examination his respiratory rate is 35/minute with no overt distress. The liver is palpable 3 cm below the right costal margin. Occasional crackles are audible in both bases. His chest X-ray demonstrated moderate cardiomegaly. His electrocardiogram is shown below.
Which one of the following is the most likely diagnosis?
A. Anomalous origin of the left coronary artery from the pulmonary artery.
B. Idiopathic dilated cardiomyopathy.
C. Incessant atrial tachycardia.
D. Kawasaki disease with associated ischaemia.
E. Viral myocarditis.

C. Incessant atrial tachycardia.
Continuous atrial tachycardia can cause right heart failure after 3 days. Clinical signs of right heart failure. ECG shows left atrial hypertrophy and tachycardia.
D and E would show ST changes.

52

Which anti-arrhythmic drug is inappropriate in the treatment of the arrhythmia with which it is paired?
A. Atrial flutter: sotalol.
B. Atrial tachycardia in Wolff-Parkinson-White syndrome: flecainide.
C. Prolonged QT syndromes: propranolol.
D. Sinus node dysfunction: digoxin.
E. Ventricular tachycardia in cardiomyopathy: amiodarone. 

D. Sinus node dysfunction: digoxin

Digoxin acts on SA nodes. Stimulants are only appropriate measure in SA node dysfunction. Digoxin prolongs PR interval. Good for AT or atrial flutter.

53

Which one of the following cardiac lesions would be an unexpected finding in a baby with a 22q11 deletion?
A. Infradiaphragmatic totally anomalous pulmonary venous return.
B. Interrupted aortic arch.
C. Pulmonary atresia, ventricular septal defect and major aorto-pulmonary collaterals.
D. Tetralogy of Fallot.
E. Truncus arteriosus.

A. Infradiaphragmatic totally anomalous pulmonary venous return.

Cardiac anomalies — Conotruncal cardiac defects occur in approximately 80 percent of DGS patients and when present, are typically the initial abnormalities noted. The term conotruncal refers to the distal portion of the fetal heart (trunco-aortic sac) at an early stage in development. The aortic and pulmonary roots subsequently develop from this area, and defects in these structures are referred to as conotruncal defects.
The most common cardiac defects account for two-thirds of the cardiac anomalies seen in patients with DGS and include the following:

  • Interrupted aortic arch
  • Truncus arteriosus
  • Tetralogy of Fallot
  • Atrial or ventricular septal defects (ASD, VSD)
  • Vascular rings

54

A 15-year-old boy presents following a syncopal episode outside the headmaster’s office prior to being reprimanded. The above rhythm strip (A) was obtained by the ambulance officers upon their arrival and the attached 12 lead electrocardiogram (B) was obtained the following day.

Which one of the following is the most likely diagnosis?

A. Arrhythmogenic right ventricular dysplasia.
B. Congenitally corrected transposition of the great arteries.
C. Hyperparathyroidism.
D. Hypoparathyroidism.
E. Long QT syndrome.

E. Long QT syndrome.

First ECG shows U waves in 3rd row.
U waves seen in hypokalemia, hypercalcaemia, congenital prolonged QT.

The long QT syndrome (LQTS) is a disorder of myocardial repolarization characterized by a prolonged QT interval on the electrocardiogram (ECG) and an increased risk of sudden cardiac death. This syndrome is associated with an increased risk of a characteristic life-threatening cardiac arrhythmia, known as torsades de pointes or "twisting of the points". The primary symptoms in patients with LQTS include palpitations, syncope, seizures, and cardiac arrest.

55

A two-month-old girl had a murmur identified at birth. To your assessment now, she is pink and thriving. She shows no signs of respiratory distress or other signs of heart failure. The S2 is single and there is a long systolic murmur heard loudest at the left sternal edge. Her chest X-ray is normal and her ECG is shown above.
Which one of the following is the most likely diagnosis?
A. Aortic stenosis.
B. Atrial septal defect.
C. Physiological peripheral pulmonary stenosis.
D. Small ventricular septal defect.
E. Tetralogy of Fallot.

 

E. Tetralogy of Fallot.
ECG shows RVH. AS will have LVH. ASD will have fixed splitting of second heart sound. Small VSD would not cause RVH. TOF if small can be asymptomatic, and will have RVH. Physiological peripheral pulmonary stenosis is occurs because pulmonary arteries are so much larger than pulmonary vessels causing turbulent flow. This decreases as the pulmonary vessels increase in size as baby grows. Doesn’t cause ECG changes

56

In Kawasaki disease, which one of the following laboratory findings would be the least likely?
A. Aseptic meningitis.
B. Elevated serum transaminases.
C. Sterile pyuria.
D. Thrombocytopenia.
E. Toxic granulation of neutrophils. 

D. Thrombocytopenia.
KD is associated with thrombocytosis.

57

The child whose photograph and electrocardiogram are shown (see below), has a loud systolic ejection murmur at the upper left sternal edge, radiating to the back and axillae.

Which one of the following is the most likely diagnosis?

A. Congenitally corrected transposition of the great arteries.
B. Primum atrial septal defect (ASD).
C. Pulmonary artery branch stenoses and supra-aortic stenosis.
D. Pulmonary valve stenosis.
E. Secundum ASD. 

D. Pulmonary valve stenosis.
ECG shows RVH. Correlates with systolic ejection murmur.

58

The child pictured below is noted to have a grade 4/6 systolic murmur.
Which one of the following is the most likely diagnosis?
A. Atrioventricular septal (A-V canal) defect.
B. Hypertrophic cardiomyopathy.
C. Pulmonary valve stenosis.
D. Supravalvular aortic stenosis.
E. Ventricular septal defect.

 

D. Supravalvular aortic stenosis.

Williams syndrome - most common cardiac lesion is supravalvular aortic stenosis.

Facial features: small upturned nose, long philtrum (upper lip length), wide mouth, full lips, small chin, and puffiness around the eyes.

59

A three-year-old girl has a large secundum atrial septal defect.
The magnitude of the left-to-right atrial shunt will be most influenced by the:
A. left atrial pressure.
B. left ventricular compliance.
C. pulmonary vascular resistance.
D. right ventricular compliance.
E. systemic vascular resistance.

 

D. right ventricular compliance.

60

Which one of the following groups of physiological parameters best describes vaso-vagal syncope?

Note:
BP = blood pressure
HR = heart rate
SVR = systemic vascular resistance

 

C. low  low   low
Hypotension characterised by bradycardia and peripheral vasodilation.

61

In a four-year-old child, marked dilatation of the pulmonary artery would be most likely to occur in which one of the following?

A. Atrial septal defect.
B. Bilateral pulmonary artery branch stenosis.
C. Idiopathic arterial calcification.
D. Mild pulmonary valve stenosis.
E. Severe pulmonary valve stenosis.

D. Mild pulmonary valve stenosis.

Whirls form distal to stenosis causing dilatation. ? mild rather than severe due to increased flow across the valve.

62

The mean pulmonary artery (capillary) wedge pressure most closely approximates which one of the following pressures?

A. Left atrial mean.
B. Pulmonary artery diastolic.
C. Pulmonary vein wedge mean.
D. Right atrial mean.
E. Right ventricular end-diastolic.

 

A. Left atrial mean.

63

In congestive cardiac failure, which one of the following factors is most important in the pathogenesis of peripheral oedema?
A. Hypoalbuminaemia.
B. Impaired lymphatic drainage via thoracic duct.
C. Increased antidiuretic hormone production.
D. Increased capillary permeability.
E. Sodium retention.

 

E. Sodium retention.

Congestive heart failure is one of the most important causes of peripheral edema seen in clinical practice. Edema in congestive heart failure is the result of the activation of a series of humoral and neurohumoral mechanisms that promote sodium and water reabsorption by the kidneys and expansion of the extracellular fluid. These mechanisms, in concert with abnormal Starling forces such as increased venous capillary pressure and decreased plasma oncotic pressure, promote fluid extravasation and edema formation. The management of edema in congestive heart failure is designed to improve cardiac function and to inhibit the hormonal and neurohumoral pathways that promote edema. The combination of diuretics and vasodilators or angiotensin converting enzyme inhibitors and, in some cases, cardiac inotropic agents is highly effective in achieving these goals and providing significant symptomatic improvement in patients with edema secondary to congestive heart failure.

64

A six-week-old infant weighing 3300 g is referred for evaluation of tachypnoea and failure to thrive. He was born at term weighing 3000 g. He is tachypnoeic, mildly cyanosed and has tachycardia with normal volume pulses. Ejection and mid diastolic murmurs are heard. His transcutaneous oxygen saturation is 87% and two-dimensional Doppler echocardiography indicates double inlet left ventricle (single ventricle) with unrestricted pulmonary and aortic outflows.
Which one of the following would be the most appropriate surgical management?

A. Bidirectional Glenn shunt (superior vena cava to right pulmonary artery anastomosis).
B. Modified Blalock shunt (systemic to pulmonary artery anastomosis).
C. Modified Fontan procedure (total cavopulmonary connection).
D. Pulmonary artery banding.
E. Ventricular septation.

D. Pulmonary artery banding

Congenital heart defects with left-to-right shunting and unrestricted pulmonary blood flow (PBF) due to a drop in pulmonary vascular resistance result in pulmonary overcirculation. In the acute setting, this leads to pulmonary edema and CHF in the neonate. Within the first year of life, this unrestricted flow and pressure can lead to medial hypertrophy of the pulmonary arterioles and fixed pulmonary hypertension. Pulmonary artery banding creates a narrowing, or stenosing, of the main pulmonary artery (MPA) that decreases blood flow to the branch pulmonary arteries and reduces PBF and pulmonary artery pressure. In patients with cardiac defects that produce left-to-right shunting, this restriction of PBF reduces the shunt volume and consequently improves both systemic pressure and cardiac output. A reduction of PBF also decreases the total blood volume returning to the LV (or the systemic ventricle) and often improves ventricular function
 

65

A three-day-old term neonate weighing 3100 g has clinical features of Down syndrome. She has no respiratory distress and no apnoea. The pulses are normal, no murmur is heard and the second heart sound is loud. Transcutaneous oxygen saturations fall intermittently to the high 80s. Two-dimensional Doppler echocardiography shows complete atrioventricular septal defect.
Which one of the following would be the most appropriate?

A. Captopril.
B. Fluid restriction.
C. Frusemide.
D. Oxygen.
E. No intervention at this stage. 

E. No intervention at this stage.

Diuretic therapy reduces preload by promoting natriuresis, thereby relieving volume overload and pulmonary congestion. Diuretic agents include loop (eg, furosemide) and thiazide diuretics.
Ionotropic agents (eg,digoxin) improve myocardial contractility.

Angiotensin blockade reduces afterload, thereby decreasing left-to-right shunting and mitral regurgitation. In children, angiotensin converting enzyme (ACE) inhibitors are the preferred agents as there are no published data on the use of angiotensin receptor blockers in pediatric heart failure.
Promoting adequate nutrition, especially in patients with poor feeding and failure to thrive. In these patients, caloric supplementation is often needed and may require the use of nasogastric tube feeding or, rarely, gastric tube insertion before surgical repair.

66

A 14-year-old girl from interstate is seen in the Emergency Department with a fever. She has right thoracotomy and median sternotomy scars and her mother reports that she had heart operations at three and seven years of age. The chest X-ray obtained is shown below.

Her cardiac condition was most likely to have been:
A. persistent truncus arteriosus.
B. pulmonary atresia with intact ventricular septum.
C. tetralogy of Fallot.
D. transposition, ventricular septal defect and pulmonary stenosis.
E. tricuspid atresia 

D. transposition, ventricular septal defect and pulmonary stenosis.

The Aorta arises from the right ventricle and receives "blue" blood, whilst the Pulmonary Artery arises from the left ventricle (Transposition). The presence of pulmonary stenosis often protects the pulmonary circulation from the effects of high pressure (pulmonary hypertension), while the VSD allows adequate flow of blood from the left side of the heart into the main circulation to
maintain good oxygen levels. Many affected children can survive for many months or even years (sometimes into adult life) without surgery. If the pulmonary stenosis is severe then flow of blood in the lungs may be inadequate and early surgery (e.g. a 'shunt' operation) may be needed.

Conversely if the pulmonary stenosis is mild then pulmonary hypertension may be present and surgery may be needed to prevent this causing damage to the lungs.

Corrective surgery often involves a Rastelli operation. This results in blood being channelled through the VSD from the left ventricle to the Aorta. A patch in the right ventricle prevents blood passing from the RV to the Aorta. A conduit (which is an artificial tube containing a graft valve) is sewn in to connect the right ventricle to the Pulmonary Artery. A similar operation is called the 'REV' procedure.

67

A 10-year-old girl is referred for evaluation of an intermittent fast heart beat occurring when watching television and after retiring. Her physical examination, chest X-ray, 12 lead ECG and echocardiograph are normal. The two illustrated tracings are recorded from a simultaneous three channel Holter monitor system.

You would recommend treatment with which one of the following?
A. Amiodarone.
B. Flecainide.
C. Propranolol.
D. Sotalol.
E. No drugs.

E. No drugs

68

A four-month-old girl is referred with tachypnoea, failure to thrive, a murmur and hepatic enlargement. This still frame from her echocardiogram, shown below, is an apical four chamber projection displayed in the anatomical position.

It indicates that the most likely cause of her heart failure is:
A. complete atrioventricular septal defect.
B. inlet muscular ventricular septal defect.
C. multiple ventricular septal defects.
D. primum atrial septal defect.
E. secundum atrial septal defect

A. complete atrioventricular septal defect.

69

After an apparently normal discharge examination on day three, a five-day-old infant is brought to the Emergency Department because of severe shortness of breath and feeding difficulties. On examination she is markedly tachypnoeic, cyanosed and mottled. It is not possible to feel any pulses and both blood pressure and pulse oximetry are not recordable. An ECG monitor shows her heart rate to be 180/minute. She has a prominent parasternal heave and a liver edge 6 cm below the costal margin. On auscultation she has a gallop rhythm but no obvious murmurs. Her chest X-ray is shown below.

The most likely diagnosis is:
A. aortic valve atresia.
B. atrioventricular septal defect.
C. tetralogy of Fallot.
D. transposition with ventricular septal defect.
E. truncus arteriosus.

 

A. aortic valve atresia.
No blood flow from LV to Aorta, consequently no BP and no pulses. All systemic blood from PDA so hypoxic picture. Baby is in heart failure causing gallop rhythm.

70

The following information was obtained at cardiac catheterisation in a six-month-old infant with a loud systolic murmur at the lower left sternal edge:


Which one of the following additional auscultatory murmurs is most likely to be heard?
A. A continuous murmur at the upper left sternal edge.
B. A mid diastolic murmur at the apex.
C. A mid diastolic murmur at the lower left sternal edge.
D. An early diastolic murmur at the upper left sternal edge.
E. An ejection systolic murmur at the upper left sternal edge.

 

B. A mid diastolic murmur at the apex.

Saturations show a VSD. VSD associated with flow murmur across mitral valve which is mid diastolic rumble. Mitral valve murmur heard best in apex.

71

Which anti-arrhythmic drug is inappropriate in the treatment of the arrhythmia with which it is paired?

A. Atrial flutter: sotalol.
B. Atrial tachycardia in Wolff-Parkinson-White syndrome:  flecainide.
C. Prolonged QT syndromes: propranolol.
D. Sinus node dysfunction: digoxin.
E. Ventricular tachycardia in cardiomyopathy: amiodarone.

D. Sinus node dysfunction: digoxin.

Digoxin action is on SA node therefore will not be effective if defect at SA node.

72

A three-month-old infant is recognised as having an early systolic murmur, grade 3/6 in intensity at the lower left sternal edge and towards the apex. It starts abruptly with the first heart sound and disappears before the second. Examination is otherwise normal.

The most likely diagnosis is:
A. atrial septal defect.
B. tetralogy of Fallot.
C. peripheral pulmonary artery stenosis.
D. pulmonary valve stenosis.
E. small muscular ventricular septal defect.

 

E. small muscular ventricular septal defect.
Clinically well child with brief early systolic murmur.