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Radiologic Diagnosis of Heart

Diseases
An Atlas of Cardiac X-rays

PART 5
Radiological feature of common congenital cardiac
malformations
Extracardiac structures simulating cardiac disease

Dr. Khairy Abdel Dayem


Professor of Cardiology
PART 5 Radiological feature of common congenital
cardiac malformations
 The cardiac malpositions
 Atrial septal defect
 Ventricular septal defect
 Patent ductus arteriosus
 Pulmonary stenosis
 Coarctation of aorta
 Fallot’s tetralogy
 Transposition of great arteries
 Ebstein Anomaly of the Tricuspid valve
 Total anomalous pulmonary venous drainage

Extracardiac structures simulating cardiac disease


Radiological Features of
Common Congenital Heart
Diseases
In theThe Cardiac
normal Malpositions
situs solitus, (Fig.the
the heart occupies 84)left side of
the thorax on the same side as the stomach, (Fig. 85). The liver is
present on the right side of the abdomen.
In situs inversus totalis all body structures are mirror image of
the normal and the heart and stomach are on the right side and the
liver on the left side. If the X-ray is inverted it will appear entirely
normal, (Fig. 86).
In isolated dextrocardia, (Fig. 87) the heart exists on the left
side of the thorax but the viscera are in their normal position (situs
solitus). This creates a discordance between the position of the
systemic veins which follow the liver, and the heart. The atria
usually follow the veins and there is atrio ventricular discordance.
In isolated levocardia the heart remains in the left side of the
chest while the viscera are inverted, (Fig. 88). The atrium follow the
liver. Here too, there is atrio-ventricular discordance.

Fig. (84): The position of cardiac Fig. (85): Normal left sided cardiac
chambers, the liver and stomach in position in situs solitus in an infant
various malpositions.
Fig. (86): Dextrocardia in situs inversus totalis
Fig. (87): Isolated lexocardia in a case Fig. (88): Isolated dextrocardia. Note
of situs inversus. Note that the that the stomachis situated in the left
stomach is on the right side. side of abdomen (situs solitus)
Atrial Septal Defect (ASD)
When there is a defect in the interatrial septum the blood will
flow from the LA to the RA (left to right shunt). This is because
the LA empties into the thick less compliant LV while the right
atrium empties into the thin more distensible RV.

Additional oxygenated blood will reach the RA and RV and


pulmonary arteries. Pulmonary plethora will result, (Fig. 89).

If the pulmonary blood flow is excessive, pulmonary arteriolar


vasoconstriction will occur and causes pulmonary hypertension.
This will decrease then abolish the increased blood flow in the
lungs. It will also cause further hypertrophy and dilatation of the
RV, RA and pulmonary arteries.

Ultimately the shunt will be reversed resulting in Eisenmenger


Syndrome.
Fig. (89): X-ray signs of ASD: dilated RA, RV, PA and
pulmonary plethora. N = normal x-ray
X-ray Picture (Fig 89 & 90):
 Plethoric lung fields.

 Dilatation of the right atrium, right ventricle and pulmonary


artery.

 Small aortic arch due to low cardiac output.

 Marked pulsations of the pulmonary artery and its branches


seen during fluoroscopy, i.e. hilar dance.

 As pulmonary hypertension develops the plethora will


decrease then disappear and the picture will be transformed
into that pulmonary hypertension.
Fig. (90): X-ray of ASD with left to right shunt
Ventricular Septal Defect (VSD)
When there is a defect in the interventricular septum the blood
will be shunted from the LV to the RV causing increased flow in
the pulmonary artery and pulmonary plethora. The excessive
blood returning from the lung will pass through the LA and will be
pumped by the LV into the aorta. Thus, LA and LV dilatation will
occur.

If pulmonary hypertension sets in, the RV will enlarge and the


pulmonary plethora will decrease and be transformed into
pulmonary oligemia.

In the final stage the X-ray shows only manifestations of


pulmonary hypertension.
X-ray Picture:
1. X-ray is normal in cases with small defects.

2. Large defects result in:

a) Pulmonary plethora, i.e. overfilled large and tortuous


pulmonary arteries.

b) Large main pulmonary artery.

c) Left and right ventricular enlargement.

d) Left atrial enlargement.


Fig. (91): Two cases of small (left) and large (right) VSD. Note the left
ventricular enlargement and the pulmonary plethora.
Patent Ductus Arteriosus (PDA)
The ductus arteriosus is normally present in the fetus. It
connects the aorta (at the junction of the arch with the descending
aorta) with the pulmonary artery (at the junction of the main
pulmonary artery with its left branch). It normally closes during the
first month after birth. If the ductus does not close, the following
hemodynamic changes will occur.

1. The blood flows through the ductus from the aorta to the
pulmonary artery, i.e. left to right shunt.

2. As pulmonary artery receives blood both from the shunt and


the right ventricle, pulmonary artery dilatation and
pulmonary plethora occur.
3. The excessive flow returns to the left atrium, the left ventricle
and the aorta resulting in:

a) Dilatation of the left atrium.

b) Dilatation and hypertrophy of the left ventricle (volume


over-load).

c) Dilatation of the ascending aorta and aortic arch.

4. If the shunt is big pulmonary vasoconstriction and hypertension


occur.

5. When the pressure in the pulmonary artery exceeds that of the


aorta, the shunt will be reversed, i.e. right to left, and differential
cyanosis occurs. This is Eisenmenger’s syndrome.
Fig. (92): X-ray findings in patent ductus arteriosus. Note the enlarged pulmonary
artery and left ventricle and the pulmonary plethora. The aortic arch is prominent
X-ray Picture:
1. X-ray is normal in cases with small ductus.

2. In moderate or large ductus the following signs appear (Fig. 92):

a) Pulmonary plethora.

b) Enlargement of the left atrium, left ventricle and the aorta.

c) Systolic dilatation of the pulmonary artery and its main


branches seen in the hilum by fluoroscopy: hilar dance.

d) Ductus itself can be seen as a line bridging the space


between the aorta and the pulmonary artery on the left
border of the heart, (Fig. 93). It may even be calcified, (Fig.
94).
Fig. (93): The patent ductus is seen in the space between the aorta (AO)
and pulmonary trunk (PT) in the X-ray (left) and angiogram (right)
Fig. (94): Two cases of calcified ductus
Pulmonary Stenosis
Pulmonary stenosis may be caused by:
a. Congenital fusion of pulmonary valve cups, I.e. congenital
valvular pulmonary stenosis.
b. Congenital excessive hypertrophy of the muscles surrounding
the outflow tract of the right ventricle (I.e. the infundibulum)
below the level of the pulmonary valve, i.e. congenital
infundibular pulmonary stenosis.

X-ray Picture:
1. Pulmonary oligemia occurs in moderate and severe cases and
results in reduced pulmonary vascular markings.
2. Right ventricular enlargement is proportional to the severity of
the stenosis. Right atrial enlargement may also occur.
3. If the stenosis is valvular, the jet of blood coming out of the
narrow orifice hits against the pulmonary artery wall and causes
its weakening and dilatation. This is post-stenotic dilatation of
the pulmonary artery.

Fig. (95): Two cases of pulmonary stenosis with post stenotic dilatation of the PA
Coarctation of The Aorta
Coarctation of the aorta is narrowing of the aorta usually at the
junction of the arch with the descending aorta just distal to the left
subclavian artery. Because of the narrowing, pressure rises in the
ascending aorta and the aortic arch and its branches.
Anastomosis forms between the branches of he aorta proximal
and distal to the obstruction. The most important of these connect
the subclavian artery through its internal mammary branch to the
intercostal arteries which arise from descending aorta. The
intercostal arteries become enlarged and tortuous and erode the
lower border of the fourth to ninth ribs causing rib notching.
Appreciable anastomosis develops gradually by time. That is why
rib notching is not detectable except after the age of 10. Other
anastomosis develops around the scapula and another connects
the superior and inferior epigasric arteries (Fig. 96).
Fig. (96): Collateral circulation in coarctation of aorta
X-ray Picture:
1. Signs of left ventricular hypertrophy.

2. Large and high aortic arch. On the


left cardiac border two convexities
are present: The first is due to
dilated aortic arch and is followed
by another due to post-stenotic
dilatation of the aorta. This results
Fig. (97): X-ray picture of
in the characteristic 3 sign (Fig. 97). coarctation of aorta

3. Rib notching is the most specific sign (Figs. 98 & 99) and it
occurs in the fourth to the tenth ribs and appears by the age
of 10.
Fig. (98): X-ray of coarctation of aorta showing Fig. (99): Enlarged view of the
rib notching starting from the 4thth rib. The left ribs showing notching of their
border of the heart shows the 3 sign. lower borders
Fallots Tetralogy
Fallot’s tetralogy consists of:
1. Severe pulmonary stenosis which causes right ventricular
hypertrophy. The pulmonary stenosis is usually infundibular
but sometimes it is both valvular and infundibular.
2. Large ventricular septal defect which makes the pressure
equal in both ventricles.
3. The origin of the aorta is abnormally deviated to the right, i.e.
dextroposed (dextro = right) so that it lies partly over the right
ventricle, i.e. the aorta over-rides both ventricles.
Due to the severe pulmonary stenosis and the large ventricular
septal defect, the pressure in both ventricles is equal. There is no
left to right shunt. On the contrary, part of the blood pumped by the
right ventricle passes into the aorta causing its enlargement and
central cyanosis.
X-ray Picture (Figs. 100 & 101):
1. Right ventricular hypertrophy causes the apex to be
displaced outwards and becomes separated from the
diaphragm.

2. The aorta receives blood from both ventricles and the aortic
arch is enlarged. Sometimes the aortic arch is directed to the
right i.e. right-sided aortic arch (Fig. 102).

3. The pulmonary artery and its branches are diminished in size


due to the pulmonary stenosis (pulmonary oligemia). There
is a concavity in the region of the underdeveloped RV
outflow tract.

All the above factors result in a characteristic cardiac shadow,


i.e. coeur en sabot (Sabot = wooden shoe).
Fig. (100): Two cases of Fallot’s tetrology

Fig. (101): Fallots tetrology with near Fig. (102): Fallot’s tetrology with right
pulmonary atresia. The aorta is markedly sided aortic arch
enlarged
Transposition of Great Arteries
(TGA)
In TGA the aorta arises from the morphologically right ventricle
and the pulmonary artery from the left. The atrioventricular
connections are normal. Additional atrial or ventricular septal defect
or patent ductus are essential in order to allow oxygenated blood to
reach the body and venous blood to reach the lungs.

X-ray Picture (Fig. 103):


1. The x-ray commonly give the essential clue to the diagnosis of
TGA: Increased pulmonary arterial blood flow (plethora seen in
the x-ray) in a cyanotic child. Plethora is more evident in the
right lung because of the rightward direction of the main
pulmonary artery.
2. The thynus gland is absent and the vascular pedicle is
characteristically narrow in the frontal plane because the
pulmonary artery lies behind the aorta and not by its side.
3. The heart is enlarged and takes the shape of an “egg on side”
tilted so that its tip (the apex) is pointing downwards and to the
left.
 The presence of additional lesions e.g. pulmonary stenosis,
pulmonary hypertension, ductus, etc. may alter or add to the
typical picture.

Fig. (103): Typical picture of TGA.


Note the oval cardiac silhouette, the
narrow pedicle and the pulmonary
plethora.
Ebstein Anomaly of The Tricuspid
Valve
This is a rare anomaly in which the leaflets of the tricuspid valve
are adherent to the right ventricular wall. The level of the valve is
shifted towards the right ventricular apex. A portion of the right
ventricle is “atrialized” i.e. becomes hemodynamically part of the
right atrium. Tricuspid regurgitation is commonly severe.

X-ray Picture:
1. The most characteristic x-ray sign is enlarged right atrium
forming a prominent convexity on the lower two thirds of the
right cardiac borders.
2. The right ventricular infundibulum forms a prominence on the
left cardiac border at a level lower than that of the pulmonary
artery. This, together with the right atrial enlargement gives a
box like shape to the cardiac silhouette (Fig. 104).
3. The overall cardiac size may vary from huge (specially in
infants) to normal.

Fig. (104): Ebstein anomaly. The right


atrium is enlarged. The right ventricular
inlet is atrialized and the right ventricular
infindibulum is prominent.

4. The lungs vasculature is normal or reduced .


The differential diagnose of the x-ray film is pericardial
effusion, dilated cardiomyopathy and heart failure due to critical
valve lesion.
Total Anomalous Pulmonary Venous
Drainage
Anomalous pulmonary venous drainage may be total or partial
and may be supracardiac, cardiac or infracardiac according to the
level in which the pulmonary venous flow joins the systemic
circulation: i.e. either in:

 Superior vena cava, or

 Right atrium or coronary


sinus, or

 Inferior vena cava.

Fig. (105): The course of circulation in


total anomalous venous drainage
opening in the superior vena cava
In total anomalous pulmonary venous drainage the pulmonary
veins join a transverse common vein that ends in an anomalous
vertical channel which, in turn opens in the left side of the
innominate vein (Fig. 105).

X-ray Picture:
The x-ray resembles that of an atrial septal defect.

The commonest form of total anomalous venous connection


gives the “snow man” or “figure of 8” silhouette. The upper
portion of the 8 is formed by the dilated left vertical venous
channel and the right superior vena cava. The lower portion is
made by the dilated right atrium and right ventricle (Fig. 106).
Fig. (106): (Left): an angiogram and (right): X-ray of a case of total anomalous
venous drainage in which the common venous channel opens in the superior
vena cava. The X-ray shows the figure of 8 or the snow-man appearance.
Extracardiac Structures Simulating Cardiac Enlargement
Pathological masses in the anterior or superior mediastinum may be
superimposed on the cardiac shadow and simulate enlargement of various
cardiac chambers and great vessels. They include lymphomas, dermoid
cyst, retrosternal goiter, thymoma, etc.., (Figs. 107, 108, 109 & 110).

Fig. (107): Fig. (108): Fig. (109):


When such masses occur, can be suspected by the presence of a normal
cardiac size and normal pulmonary vasculation.
Fig. (111): Enlarged thymus gland in an infant