Congenital & General Flashcards
Ped) 1. A 14-year-old boy with a heart murmur has an echo showing ASD. An abnormality of the SVC is suggested and investigated with CT. Which is the most common congenital abnormality of the SVC?
A. Double SVC
B. Single left-sided SVC
C. Partial Anomolous Pulmonary Venous Return (PAPVR)
D. Hemiazygous continuation
E. Total Anomolous Pulmonary Venous Return (TAPVR)
A. Double SVC
Occurs in 0.3% of the population, but more common in patients with congenital heart disease, particularly with ASD. Persistent left SVC is much rarer and can be confused with lymphadenopathy.
(Ped) 31. In Scimitar syndrome, a chest radiograph would show:
A. A curved vessel at the left medial costophrenic angle
B. Hypoplasia of the left lung
C. Prominent left atrium
D. Decreased pulmonary vascularity
E. Radiographic features similar to Atrial Septal Defect (ASD)
E. Radiographic features similar to Atrial Septal Defect (ASD)
Part or all of the hypogenetic lung, which is almost always right-sided, is drained by an anomalous vein. There is increased pulmonary vascularity.
(Ped) 32. In an acyanotic child with an enlarged heart and an enlarged main pulmonary artery, which is the diagnosis?
A. Transposition of the great vessels
B. Ventricular Septal Defect (VSD)
C. Truncus arteriosis
D. TAPVR
E. Tricuspid atresia
B. Ventricular Septal Defect (VSD)
The other options are causes of an enlarged heart in a cyanotic child with a concave main pulmonary artery.
d) 46 A neonate with complex congenital heart disease has been referred for a cardiac MRI in order to obtain more precise anatomical and function information.
Which of the following factors is the most problematic for MR imaging in this age group?
(a) Breath holding cannot be achieved
(b) Imaging takes over 1 hour
(c) The size of the coronary vessels
(d) Neonates cannot lie still
(e) Neonates have slow heart rates
(c) The size of the coronary vessels
The small size of the neonatal heart and vessels is a challenge, which requires high resolution scans using a small FOV and small surface coils to maximise SNR. Neonates/ infants are typically anaesthetized and intubated, this stops spontaneous movement and allows breathholding sequences to be performed. Some targeted studies may be as short as 10 minutes. Neonates have a fast heart rate, which necessitates high temporal resolution for accurate assessment of ventricular volume and flow.
Ped) 21) A 10 year old presents with increasing dyspnoea on exertion. A chest radiograph shows dilatation of the left pulmonary artery with mural calcification. The right pulmonary artery and lungs are normal. An ECG shows right ventricular hypertrophy. What is the most likely diagnosis?
a. aneurysm of pulmonary artery
b. patent ductus arteriosus
c. right pulmonary atresia
d. pulmonary stenosis
e. Swyer–James syndrome
d. pulmonary stenosis
Pulmonary stenosis is often asymptomatic, but it presents when there is a high gradient across the pulmonary valve. There is dilatation of the pulmonary trunk and left pulmonary artery (post-stenotic dilatation), but the right pulmonary artery is unaffected, as the jet preferentially enters the left pulmonary artery. Calcification of the pulmonary arterial wall is highly suggestive.
Pulmonary atresia is associated with a small hemithorax and mediastinal shift towards the affected side.
Swyer– James syndrome presents with a hyperlucent lung with reduced vascularity and a small hilum. Neither of these conditions is associated with dilatation of the pulmonary arteries.
Ped) 40) At 6 months old, an infant with a cardiac murmur, learning disability, physical handicap, and abnormal facies is investigated after a urinary tract infection. Ultrasound scan reveals unilateral ureteric obstruction by an echo-bright focus in the bladder wall at the vesicoureteric junction. On the same examination, a bladder diverticulum is also noticed. Choose the most likely unifying diagnosis.
a. Marfan’s syndrome
b. Maffucci’s syndrome
c. Morquio’s syndrome
d. Williams’ syndrome
e. Mikity–Wilson syndrome
d. Williams’ syndrome
The features of idiopathic hypercalcaemia of infancy (Williams’ syndrome) include elfin-like facies, neonatal hypercalcaemia that can form renal stones, learning disability, physical retardation, colonic and bladder diverticula, aortic and pulmonary valve stenosis, cardiac septal defects, osteosclerosis and metastatic calcification.
Marfan’s syndrome is an autosomal dominant, connective tissue disorder that, in addition to skeletal manifestations, may involve the cardiovascular system, particularly the mitral valve and ascending aorta.
Morquio’s syndrome is the commonest of the mucopolysaccharidoses, with multiple skeletal manifestations occurring within the first 18 months of life.
WilsonMikity syndrome is similar to bronchopulmonary dysplasia, occurring in normal preterm infants breathing room air, but is seldom seen now due to the use of mechanical ventilation.
Maffucci’s syndrome is a dysplasia characterized by enchondromatosis and multiple soft-tissue haemangiomas. Learning disability and urinary tract abnormalities are not features of any of these conditions.
Ped) 45) What is the commonest chest radiograph finding seen in tetralogy of Fallot?
a. pulmonary plethora
b. bulging right heart border
c. concavity at the level of the pulmonary trunk
d. elevated cardiac apex
e. bulging of the superior left heart border
d. elevated cardiac apex
Tetralogy of Fallot accounts for 8% of congenital heart disease and consists of ventricular septal defect, overriding aorta, right ventricular hypertrophy and right ventricular outflow obstruction. On the chest radiograph, the most common finding is elevation of the cardiac apex due to right ventricular enlargement. This, with concavity at the level of the pulmonary trunk due to reduced pulmonary blood flow, produces the classic boot-shaped heart. A slight bulge in the upper left heart border may be seen due to the right ventricular infundibulum.
Other associated features sometimes seen are aortic enlargement and right-sided aortic arch.
@# 57) A 35-year-old man presents following a chest injury. A chest radiograph shows a smooth, curvilinear, tubular opacity adjacent to the right heart border. No other abnormality is seen. The accident and emergency team are requesting a CT of the chest. What is the most likely diagnosis?
a. pulmonary contusion
b. pneumothorax
c. pericardial injury
d. extralobar sequestration
e. partial anomalous pulmonary venous return
e. partial anomalous pulmonary venous return
The appearances are classic of partial anomalous pulmonary venous return, which occurs in 0.3–0.5% of cases of congenital heart disease and is associated with atrial septal defects and hypogenetic lung.
Contusions are seen as illdefined opacities on CT, often with rib fractures.
Anterior pneumothorax would cause increased conspicuity of the heart border.
Pericardial injury produces a thick, irregular, shaggy, soft-tissue density adjacent to the heart border.
Extralobar sequestration produces a triangular-shaped opacity adjacent to the diaphragm.
Ped) 98) A 4-month-old boy presents with cyanosis. Examination reveals right ventricular heave and systolic murmur. A chest radiograph shows a bulging right heart border and widening of the superior mediastinum, creating a ‘snowman’ appearance. What is the most likely diagnosis?
a. Fallot’s tetralogy
b. total anomalous pulmonary venous return
c. partial anomalous pulmonary venous return
d. transposition of the great vessels
e. coarctation of the aorta
b. total anomalous pulmonary venous return
Total anomalous pulmonary venous return presents in the first year of life with cyanosis.
It is due to failure of the pulmonary veins to drain into the left atrium, with drainage instead into another vascular structure.
There are four types – the commonest, type I (supracardiac), has the four pulmonary veins draining into one common vein, the vertical vein, which drains into the left brachiocephalic vein.
This dilated vein, along with the dilated left brachiocephalic vein and superior vena cava, causes widening of the superior mediastinum, which is the ‘head’ of the ‘snowman’.
The body is formed by enlargement of the right atrium, producing a rounded appearance of the lower mediastinum.
There may be increased pulmonary vascularity.
Partial anomalous pulmonary venous return presents later in life with symptoms similar to atrial septal defect.
Transposition of the great vessels presents in the first 2 weeks of life with cyanosis. Chest radiograph shows an ‘egg-on-string’ appearance of the mediastinum with increased pulmonary vascularity.
Infantiletype coarctation presents with symptoms and signs of congestive heart failure in infancy. The classic figure-3 sign seen in coarctation is often hidden by the thymus in infants.
Ped) 23 Which of the following classifications of atria/viscera positioning relative to the midline is associated with the lowest frequency of congenital heart defects?
(a) Situs ambiguous
(b) Situs inversus/dextrocardia
(c) Situs inversus/levocardia
(d) Situs solitus/dextrocardia
(e) Situs solitus/levocardia
(e) Situs solitus/levocardia
Normal anatomy is situs solitus/levocardia. The frequency of associated CHO is: situs solitus/levocardia < 1 %, situs inversus/dextrocardia 4%, situs solitus/dextrocardia 95%, situs inversus/levocardia 95%, situs ambiguous (heterotaxy syndromes) 50-100%.
37- With regard to cardiac CT imaging, which of the following statements is true?
(a) Prospective gating enables motion to be evaluated
(b) Retrospective gating reduces dose in cardiac imaging
(c) The coronary arteries are best evaluated during systole
(d) Ejection fraction evaluation requires retrospective gating
(e) Pacemakers are a contraindication to cardiac CT
(d) Ejection fraction evaluation requires retrospective gating
Prospective gating images the heart during a short period of diastole and whilst reducing dose, only images the arteries.
Evaluation of cardiac function requires imaging throughout the cardiac cycle and retrospective gating, but this has a dose penalty.
Dose may be reduced by modulating mAs during systole.
Ped) 48 A 4 week old baby is cyanosed and has a heart murmur. The CXR reveals pulmonary oedema with widening of the superior mediastinum to produce a ‘snowman’ (‘cottage loaf’) appearance. What is the likely type of total anomalous pulmonary venous connection (TAPVC) present?
(a) Cardiac
(b) lnfracardiac
(c) Mixed
(d) Scimitar syndrome
(e) Supracardiac
(e) Supracardiac
TAPVC describes return of the pulmonary veins to the RA rather than the LA; there are 3 types: supracardiac, cardiac and infracardiac. Supracardiac is the commonest; the veins drain into a common left sided trunk which runs superiorly and drains into the left innominate vein then the SVC, widening the superior mediastinum to give the ‘snowman’ (‘cottage loaf’ or ‘figure 8’) appearance. Scimitar syndrome is a type of PAPVC.
@# 18 A 2 day old neonate presents with difficulty in breathing. On examination there are widespread crepitations. The CXR shows florid pulmonary oedema. Which of the following congenital heart anomalies would be the most likely cause of these appearances?
(a) Atrial septal defect
(b) Hypoplastic Left Heart Syndrome
(c) Pulmonary atresia
(d) Tetralogy of Fallot
(e) Ventricular septal defect
(b) Hypoplastic Left Heart Syndrome
The overall incidence of CHD is 1%, the most common are structural defects bicuspid aortic valve and MVP.
The most common types of CHD presenting in the first month of life are: hypoplastic left heart syndrome (35%), TGA (25%), coarctation (20%), multiple defects (15%), pulmonary atresia/stenosis (10%), severe Tetralogy of Fallot’s (10%).
HLHS describes underdevelopment of LA, LV, MV, AV and aorta, survival requires a large ASD and PDA to allow admixing of the left and right circulations.
CXR shows marked pulmonary oedema and right heart enlargement.
HLHS and the ‘5 Ts’ present at 0-2 days, but HLHS is more common and more likely to produce marked CCF.
Coarctation and AS present at 7-14 days; VSD and PDA presents in infancy and ASD in adulthood.
20 With regard to cardiac MRI, which of the following is not true?
(a) In balanced-steady state free precession images stationary blood is bright
(b) Gradient echo sequences are not flow-sensitive
(c) In balanced-steady state free precession images flowing blood is bright
(d) Triple inversion recovery sequences are sensitive for myocardial oedema
(e) T1-weighted spin-echo sequences show blood as black
(b) Gradient echo sequences are not flow-sensitive
Cardiac MRI demands rapid and complex sequences which are evolving continually.
Gradient echo sequences are flow sensitive and may be used to show jets or regurgitation.
22 Regarding coronary artery fistulas, which of the following statements is true?
(a) They are seen in 1 % of cases undergoing coronary angiography
(b) They account for 5% of congenital cardiac abnormalities
(c) They are seen more commonly in men
(d) They are most commonly congenital
(e) They most commonly drain in to the right atrium
(d) They are most commonly congenital
CAF have a prevalence of 0.0002% in the population, are seen in <0.25% patients undergoing angiography and account for approximately 0.3% of congenital cardiac abnormalities.
Approximately 50% arise from the RCA, 42% from the LCA, 5% from both;
drainage is almost always into a low pressure venous structure, most commonly right ventricle (41%) and right atrium (26%) and the vast majority are single.
There is no predilection for race or sex.
@# Ped) 33 A neonate is cyanosed. A CXR is performed which shows slight cardiac enlargement and oligaemia of the pulmonary vasculature. Which of the following is the most likely diagnosis?
(a) Single ventricle
(b) Total anomalous pulmonary venous connection
(c) Transposition of the great arteries
(d) Tricuspid atresia
(e) Truncus arteriosus
(d) Tricuspid atresia
The list given is that of the ‘5 Ts’ of cyanotic heart disease with increased pulmonary vasculature.
The differential diagnosis for cyanosis and pulmonary oligaemia is tricuspid atresia, Tetralogy of Fallot, Ebstein’s anomaly and pulmonary atresia.
In tricuspid atresia there will only be increased pulmonary flow if there is an associated VSD but no pulmonary stenosis (a frequent association); the majority have reduced pulmonary blood flow.
Ped) 51 In a neonate with Tetralogy of Fallot, which of the following findings would not be expected at echocardiography?
(a) Atrial septal defect
(b) Aorta over-riding the interventricular septum
(c) Obstructed right ventricular outflow tract
(d) Right ventricular hypertrophy
(e) Ventricular septal Defect
(a) Atrial septal defect
‘Tetralogy of Fallot’ consists of the 4 features listed in answers (b) - (e).
Variations on the condition include ‘Trilogy of Fallot’ (PA stenosis, RVH and patent foramen ovale)
and ‘pink Tetralogy’ (VSD with mild PS).
The presence of ASD with the other features makes the diagnosis one of ‘Pentalogy of Fallot’.
61 Which of the following statements regarding MUGA studies is not true?
(a) Acquisition requires a minimum of 16 frames per cardiac cycle
(b) It is a more accurate measure of ejection fraction than echocardiography
(c) Images are acquired in the LAO position
(d) Images are gated to the r-wave of the cardiac cycle
(e) Attenuation correction is applied to reduce errors
(e) Attenuation correction is applied to reduce errors
Attenuation correction is not used in MUGA imaging. MUGA images sum all the data from points on a cardiac cycle and these may be viewed as a cine loop.
Ped) 3. A two week old neonate presents with central cyanosis and respiratory distress. Plain chest radiograph reveals pulmonary plethora. Which is the most likely underlying congenital heart disease?
a. VSD
b. Tetralogy of Fallot
c. PDA
d. Pulmonary stenosis
e. Total anomalous pulmonary venous return (TAPVC)
- e. Total anomalous pulmonary venous return (TAPVC)
VSD and PDA will both result in pulmonary plethora and distress, but are left-to-right shunts and acyanotic. Pulmonary stenosis results in pulmonary oligaemia and may or may not be cyanotic depending on the presence of an intracardiac defect with shunt reversal.
Tetralogy of Fallot is a congenital cyanotic heart disease with pulmonary oligaemia unless associated with the development of aorto-pulmonary collaterals.
Apart from TAPVC, the admixture lesions such as truncus arteriosus, tricuspid atresia, transposition of great vessels, single ventricle and common atrium are some other causes of cyanosis with pulmonary plethora.
Ped) 52. A four month old infant presents with cyanosis and repeated squatting episodes. Following an echocardiogram, a diagnosis is made. Which of the following is unlikely on a plain radiograph?
a. Boot-shaped heart
b. Fullness in the pulmonary artery
c. Right-sided aortic arch
d. Pulmonary oligaemia
e. Enlarged aorta
- b. Fullness in the pulmonary artery
All the above are features of TOF except for fullness of the pulmonary artery, there is usually a prominent concavity in the region of the pulmonary artery.
- A 45 year old man with a known atrial septal defect (ASD) presents with breathlessness and mild cyanosis. Clinical examination reveals a loud second heart sound and a prominent parasternal heave, but no signs of heart failure. Echocardiogram demonstrates a shunt reversal. Which of the following is not a usual feature on the imaging?
a. Paucity of peripheral pulmonary vasculature
b. Enlarged central PA
c. Right ventricular hypertrophy
d. Dilated pulmonary veins
e. Linear calcification of the main pulmonary arteries
- d. Dilated pulmonary veins
This patient has developed Eisenmenger syndrome with reversal of his longstanding left-to right shunt across the ASD. All of the features listed except dilated pulmonary veins are generally present.
d) QUESTION 15 A 3-month-old infant presents with a history of failure to thrive. On examination, the infant is tachypnoeic with no clinical evidence of central or peripheral cyanosis. A chest radiograph shows enlarged central and peripheral pulmonary vessels throughout both lungs. Which one of the following is a potential diagnosis?
A Pulmonary stenosis
B Tetralogy of Fallot
C Total anomalous pulmonary venous return
D Tricuspid atresia
E Ventricular septal defect (VSD)
E Ventricular septal defect (VSD)
VSD is the only option that presents as acyanotic heart disease with pulmonary plethora.
Ped) QUESTION 53 A 3-month-old infant with Tetralogy of Fallot is awaiting surgery. A preoperative chest radiograph is performed when the child has no concurrent illness. Which one of the following features are you most likely to see?
A Boot-shaped heart
B Enlarged hila
C Pulmonary plethora
D Rib notching
E Splaying of hila
A Boot-shaped heart
The boot shaped heart is seen secondaiy to the right ventricular hypertrophy.
Ped) QUESTION 54 A neonate who is cyanotic at birth undergoes an echocardiogram. This detects apical displacement of the septal leaves of the tricuspid valve and an apical septal defect. Which one of the following is the most likely diagnosis?
A Bacterial endocarditis
B Ebstein’s anomaly
C Eisenmenger’s syndrome
D Tetralogy of Fallot
E Tricuspid atresia
B Ebstein’s anomaly
Ebstein’s anomaly is a congenital abnormality of the tricuspid valve. It results in tricuspid regurgitation and atrialisation of the right ventricle. It is usually associated with an atrial septal defect (ASD), and a right-to-left shunt with cyanosis.
@# 15 A patient undergoing echocardiography for an acyanotic shunt had the following findings on imaging: dilated left atrium and ventricle, dilated right ventricle, undilated right atrium and undilated aorta. What is the most likely cause of the shunt?
a Ostium primum atrial septal defect (ASD)
b Ventricular septal defect (VSD)
c Patent foramen ovale
d Ostium secundum ASD
e Patent ductus arteriosus (PDA)
15 Answer B: Ventricular septal defect (VSD)
The following features can differentiate between the position of acyanotic shunts:.
