Cardiology Exam Qs 2004-1999 Flashcards
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
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
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.
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.
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
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.
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).
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.
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
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)
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
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
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.
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.
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.
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
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.
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.
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.
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.
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.
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
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.
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.
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.
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.
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
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
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
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
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.
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.
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.
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
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.
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.
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.
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
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.
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.
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
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.
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
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.
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.
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.
