An approach to Congenital heart disease by Dr. Prashant Gupta

1. AN APPROACH TO CONGENITAL
HEART DISEASE
Dr. Prashant Gupta
NAMS, BIR HOSPITAL

2. Development of primitive heart tube

8. Introduction
• The incidence of congenital heart disease in live births is
estimated at between 0.5-1.5%.
• Many of the recorded abnormalities are relatively simple
with only a small proportion of cases having complex
abnormalities.
• This relatively low incidence means that many
radiologists will see only a small number of congenital
heart disease cases each year however it is essential that
all radiologists should be sufficiently well informed to
recognize possible cardiac problems and guide further
investigations.

9. Role of a radiologist
• Recognition that a congenital heart disease is present.
• Detailed diagnosis by echocardiography with the possible
addition of transesophageal echocardiography, MRI, cardiac
catheterization and angiography.
• Detailed evaluation of investigation results.
• Management which can be continued medical management,
palliative surgery, corrective surgery or interventional
catheter techniques.
• Follow up of patients with/without interventions.

10. An approach
• In recent years a reasonably standardised approach has been achieved in
the description of congenital cardiac abnormalities.
• Although this is not necessarily used in the description of very simple
abnormalities, it is invaluable in the description of complex abnormalities
as it avoids confusion or ambiguity.
• This approach has five major descriptive steps:
1. Situs
2. Cardiac connections
3. Looping
4. Positions
5. Malformations.
@SLC PM {slc pass navako PM Oli}

11. Situs
• The abdominal and thoracic viscera are
asymmetrical, and for this reason normal situs
can be recognized by obvious features such as
the liver and inferior vena cava lying on the
right side and the spleen and the heart on the
left side.

12. • The very high association with the inferior
vena cava draining into the right atrium has
led to the development of the term
visceroatrial situs. This essentially means that
the atrial situs in almost all cases conforms to
the situs of the upper abdominal viscera,
irrespective of the situs or position of the
remainder of the heart.

13. • It is also important to recognize the presence
of asymmetry in the lungs, which is usually
apparent in the form of bronchial situs. The
right main bronchus is shorter, wider and
more vertically orientated than the left main
bronchus, which is usually at least 1.5 times as
long as the right from bifurcation to first major
branch. The bronchial situs nearly always
corresponds to the visceroatrial situs.

15. Situs solitus
This describes the normal
situation with normal
visceroatrial situs (liver,
inferior vena cava and right
atrium on the right side)
and normal bronchial situs.
The `position' of the cardiac
mass and/or cardiac apex is
not directly related to
visceroatrial situs and may
not correspond with it. The
apex can occasionally be
directed toward the right
side even with normal
visceroatrial situs, this
sometimes being described
as isolated dextrocardia or
dextrorotation of the heart.

16. Situs inversus
In this condition there is
complete reversal of the
visceroatrial situs and the
bronchial situs.
In most cases of situs
inversus the cardiac apex
lies on the right side, but
occasionally it will lie on
the left (isolated
levocardia=> situs
inversus with
levocardia).

17. Situs ambiguous
This describes a situation in which
the left and right-sided nature of
abdominal or thoracic organs and the
atria are not clearly distinct.
1.` Bilateral right-
sidedness'
In this condition there is a midline liver
running across the upper abdomen, the
spleen is absent, the stomach is usually
centrally positioned, and the bronchial
anatomy shows right-sided morphology of
both major bronchi. Both atrial chambers
have right-sided characteristics and, not
surprisingly, there is a frequent association
with abnormalities of pulmonary venous
drainage.
2. ‘Bilateral left-sidedness‘
is also associated with a midline liver,
often smaller but there is frequently
polysplenia and bilateral left atrial
morphology, and the two main bronchi
both show left morphology. There is assoc
abnormal systemic venous drainage.

19. Cardiac connections
• Once the cardiac chambers have been identified in
morphological terms it should be possible to state which
vessel or chamber is connected to which.
• Two types of discordance
– Ventriculoarterial discordance (the aorta arises from the
morphologically right ventricle and the pulmonary artery
arises from the morphologically left ventricle)
– Atrioventricular discordance (when the morphologically
right atrium drains into the morphologically left ventricle
and vice versa.

20. Looping/ Topology
• This term relates to the ventricular loop which
has been formed during cardiac development.
If the heart is well enough developed to have
two ventricles, each with an inlet and an
outlet, and an interventricular septum lying
between them, then it will be possible to
define the loop.
• D-loop and L-loop configurations are
stereoisomers (mirror images) of each other,
the difference between the two types of loop
being analogous to the difference between the
left hand and right hand.

21.  The normal ventricular D-loop can be
understood most simply by using the analogy
of the right hand rule in which the
morphological right ventricle is likened to a
right hand.
 The inflow is represented by the thumb, the
outflow is represented by the fingers, and the
interventricular septum will lie on the palmar
side of the hand.

22.  If, however, the morphologically right
ventricle is configured in such a way that the
relationship of inflow, outflow and
interventricular septum can only be
represented by a left hand, this infers that the
ventricle is actually a stereoisomer of a
normal D-loop ventricle, and is an L-loop
ventricle.
 Transposition of great vessels =>anatomically
corrected or corrected transpositioned

24. Position
• Although the position of the heart in the chest is the first thing to
be seen on a chest radiograph, the absolute position is of secondary
importance in describing the fundamental nature of the congenital
heart abnormality.
• The position is of practical importance in planning surgical
procedures.
• Criss-cross heart: a complex positional variation in which there
is an additional twist of the ventricular mass resulting in ventricles
lying in unexpected positions given the particular situs and
connections.
• It is important to note the position of the aortic arch relative to the
trachea. Some congenital anomalies are associated with higher
than normal incidence of right sided arch.

26. Malformations
• This refers to the specific deformities or
abnormalities within the heart, such as
stenotic or atretic valves, abnormal
communications.
• These malformations are often the most
obvious abnormality, and they are commonly
used as the overall descriptive term for a
particular abnormality (eg- VSD, pulmonary
atresia, coarctation of aorta).

27. • In many cases the malformations are the only
abnormality and in this situation the full
description of the situs, connection, looping
and position is omitted.
• This is acceptable in normal practice, provided
that the full descriptive nomenclature is used
as soon as the congenital heart abnormality is
anything other than straightforward.

28. Classification of Congenital Heart
Disease
Acyanotic Heart Disease
a. Increase pulmonary blood flow
b. Obstruction of blood flow
Cyanotic Heart disease
a. Decrease pulmonary blood flow
b. Mixed blood flow .

29. Acyanotic Heart Disease
Move blood from arterial …to…venous system
A Cyanotic
Increased in pulmonary
blood flow
1. ASD
2. VSD
3. AVC
4. PDA
Obstruction of blood flow form
ventricle
1. Pulmonary stenosis
2. Aortic stenosis
3.Coarctation of the Aorta

30. Cyanotic Heart Defect
Cyanotic
Decreased pulmonary
blood flow
Mixed blood flow
1. Transposition of
the great Arteries
2. Total pulmonary
venous return
3. Truncus Arteriosus
4. Hypoplastic left
Heart Syndrome
1. Tricuspid Artesia
2. Tetrology of Fallot

31. An approach to a chest x ray of a child
with a congenital heart disease

32. Step 0 : technical assessment
• Before you even begin it is essential to assess the
technical adequacy of the chest x-ray. The film
should not be rotated, over or under exposed and no
excessive lordotic or kyphotic angulation should be
present. An adequate inspiratory effort must also
have been obtained.

33. Step 1 : pulmonary vasculature
• Establishing whether pulmonary vasculature
is normal, congested (active or passive) or
decreased is essential in narrowing the
differential.
• .

34. What we are going to evaluate?
• Right descending pulmonary artery, main pul.
Artery (Pul. Bay)
• Distribution of flow in the lungs
Upper vs lower lobes
Central vs peripheral
• Findings: Normal
Pulmonary arterial hypertension
Pulmonary venous hypertension
Increased flow
Decreased flow

37. Main
Pulmonary
Artery

40. Pulmonary artery
• The pulmonary artery may be normal, increased or decreased in
size.
• A small or inapparent pulmonary artery can be due to either it
being small due to decreased pulmonary flow as a result of
pulmonary outflow obstruction or it being abnormally located as
is the case in truncus arteriosus and transposition of the great
arteries.
• An enlarged pulmonary artery may represent:
– post stenotic dilatation
– increased pulmonary blood flow
• left to right shunts
• pulmonary valvular insufficiency
– pulmonary arterial hypertension

41. If we draw a
tangent line
from the apex of
the left ventricle
to the aortic
knob (red line)
and measure
along a
perpendicular to
that tangent line
(yellow line)
The distance
between the
tangent and the
main pulmonary
artery (between
two small green
arrows) falls in a
range between
0 mm (touching
the tangent line)
to as much as
15 mm away
from the tangent
line

42. • Increased
pressure
• Increased flow
Main pulmonary
artery projects
beyond tangent

44. 15F,
16M

45. Small pulmonary artery
• Truncus arteriosus
• Tetralogy of Fallot
Main pulmonary artery is
more than 15 mm
from tangent

46. Venous hypertension
• Stage 1: cephalisation of the blood flow (14-19 mm of hg)
• Stage 2: interstitial edema ,pleural effusion (20-25)
– Kerly lines (1-3mm, upto 1cm, subpleural,
costophrenic),
– peribronchial cuffing
– Background haze
– Septal and interstitial oedema
• Stage 3 : Alveolar oedema (>25)
– Bat wing appearance

47. RDPA >16

48. •ENLARGEMENT OF THE CENTRAL (HILAR) PULMONARY, LOBAR
AND SEGMENTAL PULMONARY ARTERIES
•DIAMETER OF AN EN FACE PULMONARY VESSEL EXCEEDING
THE DIAMETER OF ITS COMPANION BRONCHUS
•THE VISUALIZATION OF EN FACE VESSELS (SHORT VESSELS)
BELOW THE LEVEL OF THE TENTH POSTERIOR RIB

50. Normal pulmonary vasculature
Unhelpful- does not narrow the differential.
It may represent milder or earlier forms of
congenital heart defects, or alternatively
represent abnormalities that do not result
in altered pulmonary blood flow or
pressures, such as simple valvular
abnormalities or coarctation of the aorta .

51. Congested pulmonary vasculature =>PLETHORA
Congested pulmonary vasculature can be active or passive, and
represents increased blood flow and increased pulmonary
venous pressure respectively.
– Active congestion is therefore seen in left to right shunts
when right ventricular output is approximately 2.5 times
that of the left ventricle . Although the vessels are
enlarged, are seen more peripherally than normal and may
be tortuous, in contrast to passive congestion the margins
remain distinct as there is little interstitial oedema.
– Passive congestion is due to elevated pulmonary venous
pressure and reflects left cardiac dysfunction or
obstruction.

53. Decreased pulmonary vasculature
Oligaemia of the pulmonary vasculature
represents decreased blood flow through the
pulmonary circulation, usually as a result of
right ventricular outflow obstruction with
associated right to left shunt.
If the proximal pulmonary arteries are enlarged,
with pruning of the peripheral vascular
markings, then pulmonary arterial hypertension
should be considered

55. Step 2 : aorta
• The aorta may altered in size, location
or shape.

56. Size
• The aorta may be normal, increased or decreased in
size.
• An enlarged aortic knob may represent:
– post stenotic dilatation
– increased blood flow (CYANOTIC HEART DISEASE)
• truncus arteriosus
• valvular insufficiency
• Severe tetralogy of Fallot
– systemic hypertension
• A small aortic knob usually represents reduced blood
flow typically due to ASD or VSD. It may also be
primarily hypoplastic in hypoplastic left heart syndrome.

57. Position
• Although most right sided aortic arches are
incidental with only ~10% being associated with
congenital heart disease, in the setting of mirror
anatomy the vast majority do have cardiac
anomalies, most frequently tetralogy of fallot .

58. • Assuming that the heart is in normal situs solitus
(laevocardia) position,
• right-sided aortic arch (M/C cyanotic heart disease)
– is seen in about 30–50% of patients with
persistent truncus arteriosus,
– 10–30% of patients with tetralogy of Fallot (being
more frequent with severe RV outflow
obstruction),
– 15–20% of patients with tricuspid atresia,
– 5% of patients with transposition (UTGA) of the
great vessels and occasionally in VSD.
• The aortic arch, trachea and main bronchi are more
clearly seen on a high-kV, well-penetrated grid film

59. • The side of the aortic arch can usually be
inferred to be on that side of the trachea
where there is more mediastinal soft tissue
at the level of the 3rd and 4th dorsal
vertebrae.
• In doubt, a barium swallow will demonstrate
a concave indentation and displacement of
the oesophagus away from the side of the
aortic arch.

60. Shape
• The most common abnormality of shape is the
so-called figure of 3 sign seen in aortic
coarctation.

61. Aortic knob
should
measure
< 35mm
Aortic Knob
The first bump on the left-
side is the aortic arch. We
can measure the knob from
the lateral border of air in
the trachea to the edge of
the aortic knob.

62. 42mm
Enlarged with:
l Increased pressure
l Increased flow
l Changes in aortic wall
Aortic Knob

63. Step 3 : cardiac size and shape
• Finally the heart itself may be abnormal in size or demonstrate
alterations in shape representing underlying chamber
enlargement or anatomic anomalies.
• It is also important to assess for the correct orientation of the
heart by looking for the liver / stomach below the diaphragm
and reviewing side markers.

64. Step 4 : Spine, rib cage and sternum
• The vertebrae should be assessed for congenital anomalies
including scoliosis which is present in 6% of patients with a
congenital heart defect, but only 0.4% of the normal
population.
• Ribs may demonstrate notching in coarctation of the aorta,
or may be only number 11 in patients with Down syndrome
• Down syndrome children may also show hypersegmented
sternums.

65. Ventricular Septal Defect
• VSD – is an abnormal opening in the
ventricular septum, which allows free
communication between the Rt & Lt
ventricles. Accounts for 25% of CHD.

67. VSD types {sutton}
• Perimembranous (M/C type)
• Muscular defects
– Inlet /Basal
– Outlet
– Apical (mid-muscular)

68. Gerbode defect: basal septum (right AV septum) defect between the LVOT
and right atrium; confused perimembranous VSD; a/w TR

69. Ventricular Septal Defect

70. CXR
Findings vary depending on the size of the
VSD.
• Normal in a person with a small VSD.
• Moderate to large VSDs
 Cardiomegaly with enlargement of the
left atrium, left ventricle/right ventricle,
and pulmonary arteries and increased
pulmonary blood flow (Prominent of
main/hilar/peripheral pulmonary artery,
pulmonary plethora. Later PAH).
 Congestive heart failure occurs
frequently in infants
• Pulmonary hypertension may be
evident in older Children or young
adults.

71. Atrial Septal Defect
• ASD is an opening in the atrial septum
permitting free communication of blood
between the atria. Seen in 10% of all CHD.

73. Atrial Septal Defect
• Two primary types of ASDs occur and are classified by their
relationship to the fossa ovalis.
 Ostium secundum defects (which comprise 80% to 90% of
all ASDs) occur in the region of the fossa ovalis.
 Ostium primum defects occur caudal to the fossa ovalis at
the base of the atrial septum, are usually large defects, and
almost always are associated with other types of structural
heart disease.

74. Additionally, two defects—
1. Sinus venosus septal defects- located posterior to the
fossa ovalis and occur as a result of a deficiency in the
sinus venosus septum, which separates the right
pulmonary veins from the superior vena cava and
from the posterior aspect of the right atrium.
Associated with partial anomalous pulmonary venous
return.
2. Unroofed coronary sinus is rare and occurs as a result
of a partial or complete absence of the wall between
the inferior left atrium and the roof of the coronary
sinus.
Associated with drainage of a left superior vena cava to the
coronary sinus or left atrium

75. Plain film (CXR)
• can be normal is early stages +/- when the ASD is small
• signs of increased pulmonary flow (shunt vascularity)
– enlarged pulmonary vessels
– upper zone vascular prominence
– vessels visible to the periphery of the film
– eventual signs of pulmonary arterial hypertension
• chamber enlargement
– right atrium
– right ventricle
– note : left atrium is normal in size
– note : aortic arch is small to normal

76. D/D: Patent foramen ovale:
persisting interatrial pressure difference;
Echo/color doppler mapping - presence of turbulence
and size less than 3 mm
Frontal (A) view of the chest show mild cardiomegaly, a prominent pulmonary artery
(arrowhead), and increased pulmonary vascularity without left atrial dilation

77. Patent Ductus Arteriosus
• Persistence of normal fetal vessel joining the
pulmonary artery to the aorta
• Closes spontaneously in normal term infants at 3-5
days of age
• Epidemiological facts
– Accounts for about 10% of all cases of CHD
– Higher incidence of PDA in infants born at high altitudes
(> 10,000 feet)
– More common in females

78. Patent Ductus Arteriosus

79. Patent Ductus Arteriosus

80. • Left to right shunting at the arterial level causes dilation of the
pulmonary vasculature.
• The main pulmonary artery is dilated which may be noted by
prominence of the main pulmonary artery segment at the left
heart border just below the aortic arch on the AP view.
• The left atrium and ventricle become dilated due to increased
pulmonary venous blood return to the left atrium resulting in
cardiomegaly.
• Left atrial dilation may cause widening of carina angle

82. Tetralogy of Fallot

84. Tetralogy of Fallot
• Most common cyanotic
lesion (7 to 10% of all CHD)
• Typical features
– Cyanosis after the neonatal
period
– Hypoxemic spells during
infancy
– Right-sided aortic arch in 25%
of all patients
– Systlic ejection murmur at the
upper LSB

85. r
a
RA LA
RV
L V
P
V
Lungs
Aorta
PA STENOSIS
 Pulmonic stenosis
Increased pressure in
the right ventricle
• septal defect
Right to left shunt
Cyanosis
TETRALOGY OF FALLOT

87. • On chest radiographs in those affected by this
syndrome, the heart has the shape of a wooden
shoe or boot (in French, coeur en sabot).
• This deformity is due to uplifting of the cardiac
apex because of right ventricular hypertrophy
and concavity of the main pulmonary artery.
• The shadow of the pulmonary arterial trunk is
almost invariably absent, and blood flow to the
lungs is usually reduced.

89. TGA – L and D type
• The estimated incidence is at ~ 1 in 5000
births.
• Transposition of the great arteries is
an isolated abnormality in 90% of those
affected and rarely is associated with a
syndrome or an extra-cardiac malformation.
• Common a/w with ASD, VSD, PDA, pulmonic
stenosis (precludes arterial switch operation)
• It is most common in infants of diabetic
mothers

90. • Occurs as a result of ventriculo-arterial
discordance with the aorta arising from the
right ventricle and the pulmonary trunk from
the left ventricle. It can be sub divided into
two main types depending on the positional
relationship of the aortic valve with the
pulmonary valve.
– D type transposition of the great arteries
– L type transposition of the great arteries -
congenitally corrected TGA

91. D type TGA: The great arteries have an anteroposterior relationship, which gives the
narrow pedicle; pulmonary valve lower in position than aortic valve

92. L type TGA: The aorta arises more towards left side – long left heart
border

94. • In the normal anatomy, the aorta is anterior to and at the
right of the pulmonary artery; in transposition of the great
arteries, the pulmonary artery is situated to the right of
its normal location and is obscured by the aorta on
frontal chest radiographs.
• This malposition, in association with stress-induced
thymic atrophy and hyperinflated lungs, results in the
apparent narrowing of the superior mediastinum on
radiographs, the most consistent sign of transposition of
the great arteries.
• The cardiovascular silhouette varies from normal in the
first few days after birth to enlarged and globular, with the
classic appearance described as an egg on a string.

96. L type TGA
• Atrio-ventricular discordance
• Ventriculo-arterial discordance
= anatomically corrected or corrected transposition
• Usually asymtomatic
• Right ventricle posteriorly and left ventricle
anteriorly; morphology also switched – RV is
not support the systemic pressure so may
have RV failure

97. Management
• Initial pallliative: Rashkind balloon septostomy
– catheter - atrial mixing {under echo control}
• Definitive surgery – Mustard operation

98. TAPVR
• Total anomalous pulmonary venous return
(TAPVR) is a cyanotic congenital heart anomaly
with an abnormal drainage anatomy of the entire
pulmonary venous system.
• In TAPVR all systemic and pulmonary venous
blood enters the right atrium and nothing drains
into the left atrium. A right-to-left shunt is
required for survival and is usually via a
large patent foramen ovale (PFO) or less
commonly atrial septal defect (ASD).

99. • TAPVR occurs when the pulmonary veins fail
to drain into the left atrium and instead form
an aberrant connection with some other
cardiovascular structure.
• Such abnormalities account for approximately
2% of cardiac malformations and are best
differentiated according to the site at which
the anomalous pulmonary veins terminate.

100. PA
RA LA
RV LV
Systemic
veins
Lungs
Aorta
To systemic circulation
PV

103. • Type I TAPVR. (a, b) Chest radiograph obtained in a neonate (b the same
as a with a superimposed drawing) reveals the classic snowman sign,
sometimes referred to as a figure-of-eight sign.
• The dilated vertical vein on the left, the innominate vein on the top, and
the superior vena cava on the right form the head of the snowman; the
body of the snowman is formed by the enlarged right atrium.

104. Partial Anomalous Pulmonary
Venous Return
• It is essentially a combination of pulmonary
hypoplasia and partial anomalous pulmonary
venous return (PAPVR).
• It almost exclusively occurs on the right side.
• Haemodynamically, there is an acyanotic left to
right shunt. The anomalous vein usually drains
into
– inferior vena cava - most common
– right atrium
– portal vein

105. • The scimitar sign is produced by an anomalous
pulmonary vein that drains any or all of the lobes of
the right lung.
• The so-called scimitar vein curves outward along the
right cardiac border, usually from the middle of the
lung to the cardiophrenic angle, and usually empties
into the inferior vena cava but also may drain into
the portal vein, hepatic vein, or right atrium.

106. • Although the diameter of the scimitar vein
depends on whether it drains the entire right
lung or only a portion of it, the diameter
generally increases as the vein descends. The
characteristic appearance of the vein has led
to its comparison to a scimitar, a sword with a
curved blade that traditionally was used by
Persian and Turkish warriors.

109. Scimitar syndrome

110. Ebstein’s Anomaly
• Ebstein anomaly is characterized by the downward
displacement of the septal leaflets and posterior
leaflets of the tricuspid valve into the inflow portion
of the right ventricle. This displacement results in the
formation of a common right ventriculoatrial
chamber and causes tricuspid regurgitation.
• Atrialized RA (massively enlarged) and smaller RV
(with infundibular narrowing) which is ineffective to
pump the blood to Pulmonary trunk.

111. • The right atrium becomes enlarged, and a
right to- left shunt (through a patent foramen
ovale or atrial septal defect) is seen in most
patients.
• Cyanosis is caused primarily by the right-to-
left shunt, and increased right atrial pressure
causes a greater right-to-left shunt and more
severe cyanosis.

113. • The most consistent imaging feature is right atrial
enlargement; the right atrium may be huge and fill the entire
right hemithorax.
• The left atrium is normal in size, but the left cardiac contour
has a shelved appearance because of the dilated right
ventricular outflow tract.
• Only cyanotic congenital malformation of the heart in which
both the aorta and the pulmonary trunk are smaller than
normal
• This combination of features produces a cardiac silhouette
that has been described as box shaped.

115. Persistent Truncus Arteriosus
• Failure of division of the
truncus arteriosus into the
aorta and pulmonary artery
• A single vessel drains both
ventricles and supplies the
systemic, pulmonary,
coronary circulation
• High ventricular septal
defect
• high flow = Very ill patients
• Mixed blood flow –
CYANOTIC HEART DISEASE

116. Persistent Truncus Arteriosus
p

117. Type I: A single common pulmonary artery with a well-
developed main pulmonary artery segment may arise
from the common truncus before it divides into left and
right pulmonary arteries
Type 2 : Length of the main pulmonary artery segment
is negligible, but the two pulmonary arteries arise close
together just above the truncal valve.
Type 3 : Left and right pulmonary arteries arise
independently from the main truncus at a higher level,
usually one from each side of the main artery. Least
common form.
Types of PTA

118. • Common mixing across VSD
• VERY Large pulmonary artery
flow
• Most cases: Fully developed pul.
artery segment doesn’t develop in
its usual postion, so chest xray
shows:
1. Pulmonary plethora
2. Narrow mediastinum(as TGA)
• Right aortic arch (in 35%)

119. Atrioventricular septal/canal defects/
Endocardial cushion defects
• The endocardial cushion normally forms the lower portion of the
atrial septum, the upper portion of the interventricular
septum, and the septal leaflets of the mitral valve and the
tricuspid valve.
• 4% of CHDs
• Left to right shunt
• a/w Down’s sydnrome; 11 pair ribs

120. • (A) Normal relationships of the interventricular and interatrial septa with the
atrioventricular valves. The atrioventricular valves are inserted
• into the septum primum (thin line). ra = right atrium; la = left atrium; r y = right
ventricle; Iv = left ventricle. (B) Ostium secundum ASD. The atrioventricular valves
and left ventricular outflow tract are normal. (C) Ostium primum ASD. The septum
primum is absent and the atrioventricular valves are inserted in a low position into
the crest of the muscular interventricular septum. (D) Total AVSD. A large common
valve separates the atrial cavities from the ventricular cavities. There is an ostium
primum ASD and a large VSD in continuity.

122. Coarctation of aorta
• Shelf like narrowing of aorta just distal to the
origin of left SCA.
• CXR:
• Small or irregular contour of upper descending aorta
• Rib notching (after 5 years of age) – prominent
intercostal collaterals.
• Right sided rib notching in coarctation proximal to left
SCA.
• a/w biscuspid aortic valve.
• Most severe: interruption of AORTA ARCH.

123. 3 types of interruption of aortic arch
• Type A: distal to left SCA
• Type B: in between left CCA and Left SCA {right sided
rib notching}
• Type C: in between innominate and left CCA
• PDA supplies lower half of body if not closed
But symptomatic if closure of PDA

124. • Echo
• via suprasternal approach; any associated PDA
• MRI
• Can be diagnostic
• And for F/U
• Cardiac catheterization
• Severe CoA – may be difficult to assess AA
• Trans-septal puncture approach or brachial artery approach
• Pulmonary artery contrast injection
• Treatment
• Surgical repair
• Balloon dilatation for recurrent of native Coarctation

125. Single ventricle/ primitive ventricle
1. Double inlet ventricle : both tricuspid and
mitral valve empty in to a single chamber
2. Common inlet valve: single large A-V valve
enters a large ventricular chamber
3. Atresia of one valve: may be
indistinguishable from situation 2
4. Very large VSD: with little residual septum
– Symptoms depend on Pulmonary stenosis
– If present cyanotic if not plethoric

126. • Treatment
• Often palliative only – shunts or pulmonary
artery handing
• Surgical repair
– TCPC (Total cavo-pulmonary communication)
procedure
– Direct communication of the right atrial appendages to the
main pulmonary artery

127. Hypoplastic left heart syndrome
• In severe aortic stenosis – poor
development of LV; may be a/w mitral valve
stenosis
• Entire systemic pumping by RV; blood
bypassed via PDA
• Usually fatal after closure of life sustaining
PDA

128. Sinus of valsalva fistula
• Enlargement of one of the sinuses of valsalva in aortic root=>
commonly in the right sinus
• May rupture into the right atrium=> left to right shunt
• CXR
– Typical features of left to right shunt; aneurysmal sinus only may not
be visible
• Echo
• Aortic root angiogram
• D/D: perimembranous VSD: defect below the aortic valve
(sinus of valsalva fistula above the fistula)

129. Double outlet ventricle (DOV)
• Both aorta and pulmonary trunk arise from
morphological RV,
• Almost always a/w VSD
• DORV with subaortic VSD
• DORV with subpulmonic VSD (Taussig Bing anomaly)
• Doubly committed VSD
• Non-committed VSD (not located near aorta or PA)

130. References
1. Textbook of radiology and imaging, David
Sutton
2. Classic imaging signs of congenital
cardiovascular abnormalities, RSNA
3. Various internet sources

131. Thank You