Pediatric Cardiology

1. Pediatric -
Cardiovascular
Ardiana Annisa

2. Congenital Heart Disease
Obstructive
lesions
- Coarctatio aortic
- Pulmonar stenosis
Acyanotic
lesions
- Ventrikel septal defect
- Atrial septal defect
-Patent Ductus Arteriosus
- Partial Anomalous
Pulmonary Venous
Return
Cyanotic
lesions
- Tetralogy of Fallot
- Transposition of Great
Arteries
- Truncus arteriosus
- Total Anomalous
Pulmonary Venous
Return
- Tricuspid atresia
- Pulmonary atresia
- Hypoplastic left heart
syndrome

3. Congenital Heart Disease
INCREASED
BLOOD FLOW
•- Atrial Septal
Defect
•- Ventrikel
septal defect
•- Patent Ductus
Arteriosus
OBSTRUCTIVE
DEFECTS
- Coarctation of
aorta
- Aortic stenosis
- Pulmonary
stenosis
DECREASED
BLOOD FLOW
- Tetralogy of
Fallot
- Tricuspid
atresia
MIXED DEFECTS
- Transposition
of great vessels
- Total
anomalous
pulmonary
venous
connection
- Hypoplastic
heart syndrome

4. Coarctatio Aorta
: a deformity of the aortic media and intima  a prominent posterior infolding of the aortic lumen.
Characteristically occurs : at or near the junction of the aortic arch and the descending thoracic aorta.
Infolding may extend laterally and cause eccentric narrowing of the lumen at the level where the ductus or ligamentum
arteriosus inserts anteromedially. Luminal narrowing in turn obstructs the flow of blood from the left ventricle.
Drawing shows a focal constriction of the aorta (1) just
beyond the origin of the left subclavian artery (2) and the
ligamentum arteriosum (3). The contour of the aorta is
deformed by both pre- and poststenotic dilatation, and the
left subclavian artery is dilated. 4 = left common carotid
artery, 5 = innominate artery, 6 = right heart structures, 7 =
left heart structures, 8 = pulmonary artery.
Two classic radiologic signs associated with aortic coarctation are the
figure-of-three sign and the reverse figure-of-three sign. The aortic
segment affected by coarctation has a shape that resembles the
number 3 on frontal chest radiographs (Fig 6a– 6d). The number 3 is
formed by dilatation of the left subclavian artery and aorta proximal to
the site of coarctation, indentation of the site, and dilatation of the
aorta distal to the site. This sign is seen in 50%– 66% of adults with
aortic coarctation. The reverse figure-of-three sign, a mirror image of
the number 3, is observed on the left anterior oblique view during
barium esophagography in patients with aortic coarctation

5. - Characterized by : abnormal opening in the atrial septum allowing communication between
the right and left atria. Due to the low pressures of the atria, the lesion is typically
asymptomatic until adulthood despite 2-4 times the normal pulmonary blood flow.
Atrial Septal Defect
Classification
according to their location within the septum:
• secundum ASD
• usually an isolated abnormality
• primum ASD
• associated with cleft anterior mitral valve leaflet (partial
atrioventricular septal defect)
• sinus venosus
• associated with anomalous right pulmonary venous return to
the superior vena cava or right atrium
• coronary sinus type ASD ("unroofed coronary sinus")
<1%
A patent foramen ovale (PFO) is a form of atrial septal defect.
Plain radiograph
•can be normal in early stages
•increased pulmonary flow (pulmonary
vascularity)
• enlarged pulmonary vessels
• upper zone vascular prominence
• vessels visible to the periphery of the
• eventual signs of pulmonary arterial
•chamber enlargement
• right atrium
• right ventricle
• Normal LA
• Aortic arch is small to normal

6. Venticular Septal Defect
Classification according to location
•membranous/perimembranous
• including the Gerbode defect
•inlet/inflow
•outlet/subarterial
•muscular/trabecular
defects in the interventricular septum that allow a hemodynamic communication between
the right and left ventricles. It typically results in a left-to-right shunt.
Plain radiograph
- The chest radiograph can be normal with a small
VSD.
- Larger VSDs  cardiomegaly (LA enlargement
although the right and left ventricle can also be
enlarged).
- A large VSD may also show features of pulmonary
arterial hypertension, pulmonary edema, pleural
effusion, and increased pulmonary vascular
markings.
MRI
May also show added
functional information (e.g.
quantification/shunt severity)
in addition to anatomy. Some
muscular defects can give a
"Swiss cheese" appearance
owing to their complexity.

7. Patent Ductus Arteriosus
persistent patency of the ductus arteriosus, a normal connection of the fetal
circulation between the aorta and the pulmonary arterial system that develops from the
6th aortic arch. Normally , functional closure 48 hours after birth.
Plain radiograph
Chest radiographic features may vary depending
on whether it is isolated or associated with other
cardiac anomalies and with the direction of shunt
flow (right to left or left to right).
- Cardiomegaly (predominantly LA and LV
enlargement if not complicated).
- Obscuration of the aortopulmonary window
- Features of pulmonary edema may be evident.

8. CT
MDCT can non-invasively provide
detailed anatomical information 1.
Krichenko classification based on
CT angiography:
type A: conical ductus, prominent
aortic ampulla with narrowing at
pulmonary artery end
type B: window, short and wide
ductus with blending of pulmonary
artery
type C: long tubular ductus with no
constrictions
type D: multiple constrictions with
complex ductus
type E: elongated ductus with
remote constriction
NOTES : A tortuous morphology that does not fit in the Krichenko classification is usually
observed in premature children and some authors proposed to classify it as type F or fetal
type. Type F ductus is larger, longer, tapers minimally from the aortic to pulmonary end, with a
tortuous connection to the pulmonary artery giving a hockey-stick appearance

10. Partial Anomalous Pulmonary Venous Return
The scimitar sign  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. 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.
Drawing shows
the pattern of blood flow
(arrows). The luminal
diameter
of the scimitar vein (1),
which may drain all or part
of the
right lung (2), enlarges as
the vein descends below the
dia- phragm (3) to empty
into the inferior vena cava
(4). Occasion- ally, the vein
may empty directly into the
right atrium (5).
Scimitar syndrome :
(a) hypoplasia of the right lung with dextroposition of the heart
(b) hypoplasia of the right pulmonary artery
(c) anomalous arterial supply of the right lower lobe from the
abdominal aorta.

11. Tetralogy of Fallot
- First described in 1888 by French physician
Etienne-Louis Arthur Fallot
- 4 Components :
(a) ventricular septal defect
(b) infundibular pulmonary stenosis
(c) overriding aorta
(d) right ventricular hypertrophy.
Embryologic : a single defect, an anterior malalignment of
the conal septum  VSD, RV outflow tract obstruction, and
overriding aorta.
RVH  long-standing untreated disease.
Drawing depicts the pattern of blood
flow (arrows) with the characteristic
ventricular septal defect (1), infundibular
pulmonary stenosis (2), overriding aorta
(3), and right ventricular hypertrophy
(4). The oxygen-rich blood in the left
side of the heart (5) mixes with oxygen-
poor blood in the right side of the heart
(6) before it proceeds to the aorta (7).
CXR  shape of a wooden shoe or boot (in
French, coeur en sabot)
 uplifting of the cardiac apex because of RVH
and concavity of the MPA.
 The shadow of the pulmonary arterial trunk
is almost invariably absent, and blood flow to
the lungs is usually reduced.
 The right ventricular infundibulum often
forms a slight bulge in the upper left heart
border, while the middle left heart border is
usually concave.

12. Transposition of Great Arteries
Normal 
- Aorta : anterior to and at the right of the pulmonary
artery
TGA 
- 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
- Most common cyanotic congenital heart lesion
- Most common in infants of diabetic mothers
- 90% of those affected and rarely is associated with a
syndrome or an extracardiac malformation
Transposition of the great arteries  a ventriculoarterial
discordance in which the aorta arises from the morphologic right
ventricle and the pulmonary artery arises from the morphologic
left ventricle
Drawing shows the pattern of blood flow
(arrows) through the heart with transposition
of the great arteries. The aorta (1) arises from
the right ventricle (2), and the pulmonary
artery (3) arises from the left ventricle (4).
Communication between the systemic and
the pulmonary circulation—an interatrial
septal defect (5), an interventricular septal
defect (6), or both—sustains life by allowing
oxygenated blood from the left atrium (7) to
mix with deoxygenated blood from the right
atrium (8) before it flows via the right
ventricle to the aorta and via the left ventricle
to the pulmonary artery.

13. Total Anomalous Pulmonary Venous Return
pulmonary veins fail to drain into the left atrium and
instead form an aberrant connection with some
other cardiovascular structure
Type of TAPVR:
- I : supracardiac level
- II : cardiac level
- III : infracardiac level
- IV :anomalous venous connections at two or more levels.
I : SUPRACARDIAC LEVEL
CXR  snowman
- Head of the snowman  The dilated vertical vein on the
left, the innominate vein on the top, and the superior
vena cava on the right
- The body of the snowman The enlarged right atrium.
Typically, four anomalous pulmonary veins (two from each
lung) converge directly behind the left atrium and form a
common vein, known as the vertical vein, that passes
anterior to the left pulmonary artery and the left pulmonary
artery and the left main bronchus to join the innominate
vein
Less common  anomalous drainage to the left
brachiocephalic vein, the right superior vena cava, or the
azygos vein occurs.
Venous obstruction in type I TAPVR is uncommon, but
extrinsic obstruction may occur if the vertical vein courses
between the left pulmonary artery anteriorly and the left
main bronchus posteriorly.

14. II : CARDIAC LEVEL
- Involves a pulmonary venous connection at the cardiac level.
- The pulmonary veins join either the coronary sinus or the right atrium.
III : INFRACARDIAC OR INFRADIAGPHRAGHMATIC LEVEL
The pulmonary veins join behind the left atrium to form a common vertical descending vein, which courses anterior to
the esophagus and passes through the diaphragm at the esophageal hiatus.
This vertical vein usually joins the portal venous system but occasionally connects directly to the ductus venosus, the
hepatic veins, or the inferior vena cava.
Type III TAPVR is virtually always accompanied by some degree of obstructed venous return. The obstruction of
pulmonary venous flow causes cyanosis and, often, early and severe congestive heart failure. In addition,
lymphangiectasia sometimes results from the obstruction of venous return through the vein that extends below the
diaphragm.
The heart size is usually normal, but there is severe interstitial pulmonary edema, thymic atrophy, and depression of
the diaphragm.
IV : ANOMALOUS VENOUS CONNECTIONS AT TWO OR MORE LEVEL
Vertical vein drains into the left innominate vein, and the anomalous vein or veins from the right lung drain into either
the right atrium or the coronary sinus. This pattern generally is associated with other major cardiac lesions.
The radiologic appearance of TAPVR varies according to the site of abnormal venous drainage and whether the flow is
obstructed. The structure in which the anomalous vein terminates appears dilated; termination at the level of the
coronary sinus, superior vena cava, or azygos vein leads to dilatation of that structure and produces characteristic
abnormalities in the imaging appearance.
All the systemic venous and pulmonary venous blood enters the right heart, and the only path for its exit to the left
heart is a communication in the atrial septum, usually a large atrial septal defect or patent foramen ovale. This right-to-
left shunt is essential for survival. The right heart is prominent in TAPVR because of the increased flow volume, but the
left atrium remains normal in size.
In infants affected by TAPVR, cyanosis and congestive heart failure typically develop in the early neonatal period.
Approximately one-third of those with TAPVR also have other associated cardiac lesions; many have heterotaxy
syndrome, particularly asplenia (1– 8).

15. Eibstein Anomaly
Drawing shows the pattern of
blood flow (arrows) caused by
downward displacement of the
tricuspid valve (1), with resultant
formation of a common chamber
(3) consisting of the right ventricle
(2) and the dilated right atrium (4),
and by the right- to-left shunt of
blood through a defect at the atrial
level (5). 6 􏰁 left atrium, 7 􏰁 left
ventricle, 8 􏰁 aorta, 9 􏰁 pulmonary
artery.
- Wilhelm Ebstein in 1866
- 0.5%– 0.7% of cases of congenital heart disease.
- Association  abnormality and oral lithium therapy during
pregnancy
- Both the aorta and the pulmonary trunk are smaller than
normal
- The pulmonary flow may vary from normal to borderline to
diminished. Normal  acyanotic; borderline or diminished
 cyanotic.
Ebstein anomaly : downward displacement of the septal leaflets and posterior leaflets of the tricuspid valve into the
inflow portion of the right ventricle  the formation of a common right ventriculoatrial chamber  tricuspid
regurgitation.
Insufficiency of the tricuspid valve  dilatation of the right ventricular outflow tract and all proximal right heart
structures
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.
CXR :
RA enlargement; the right atrium may be huge and fill the
entire right hemithorax.
LA Normal, but the left cardiac contour has a shelved
appearance because of the dilated RV outflow tract
The aorta is small, and the pulmonary trunk, which normally
appears as a discrete convex bulge, is absent. This
combination of features produces a cardiac silhouette that
has been described as box shaped

17. cyanotic congenital heart anomaly in which a single trunk supplies both the pulmonary and systemic circulation, instead of a
separate aorta and a pulmonary trunk. It is usually classified as a conotruncal anomaly.
almost always associated with a ventricular septal defect (VSD)
Truncus Arteriosus
Classifications
Collett and Edwards system
•type I: (most common) both aorta and main pulmonary artery arise
from a common trunk
•type II: pulmonary arteries arise separately from the posterior aspect
of trunk, close to each other just above the truncal valve (negligible
main pulmonary artery segment)
•type III: (least common) pulmonary arteries arise independently from
either side of the trunk
•type IV: neither pulmonary arterial branch arising from the common
trunk (pseudotruncus), currently considered a form of pulmonary
atresia with a VSD
Van Praagh system
•type A1: identical to the type I of Collett and Edwards
•type A2: separate origins of the branch pulmonary arteries from the
common trunk
•type A3: origin of one branch pulmonary artery (usually the right) from
the common trunk, with other lung supplied either by collaterals or a
pulmonary artery arising from the aortic arch
•type A4: presence of an associated interrupted aortic arch

18. Plain radiograph
-
plethora : collateral formation
mediastinum.
- an appearance is similar to D-
loop transposition of great

19. Tricuspid Atresia
- agenesis of the tricuspid valve and right ventricular inlet.
- almost always an obligatory intra-atrial connection through either an ASD or PFO in order for
circulation to be complete
- A small VSD is often also present
- may also be associated with TGA
Plain radiograph
Chest radiographic features may vary depending on the presence and extent of
a VSD or TGA. May demonstrate decreased pulmonary vascularity (i.e. oligaemic
appearance). Cardiac size may be normal or enlarged.
CT and MRI
Allows direct visualization of the anomaly and may typically show a fatty and/or muscular
separation of the right atrium from the right ventricle. Cine MRI can offer functional
information in addition to anatomy.

20. Pulmonary atresia
congenital cardiovascular anomaly in which there is complete disruption between the right ventricular outflow
tract (RVOT) and the pulmonary trunk.
Classification
It can be classified into three types 1:
- PA with intact interventricular septum (PA-IVS)
- PA with VSD (PA-VSD)
complex pulmonary atresia (pulmonary atresia
with complex cardiac malformation)
Plain radiograph
PA-VSD
•normal or mildly enlarged heart with poor or diminished
pulmonary arterial vascular markings 3
•asymmetrical vascular suggests stenosis within
pulmonary arterial tree 3
•there can be plethora due to horizontal arteries forming
aortopulmonary collaterals 3
•mottled appearance as the lung periphery may suggest
pleuro-pulmonary collateral formation 3
PA-IVS
•severe cardiomegaly from massive right atrial
dilatation 10

21. Endocardial Cushion Defects
The gooseneck-shaped deformity in endocardial cushion
defect is caused by a deficiency of both the conus and sinus
portions of the interventricular septum, with narrowing of
the left ventricular outflow tract.
Normally, the endocardial cushion  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
Drawing (anteroposterior
view) of an endocardial
cushion defect shows the
concavity of the medial
margin of the left ventricle
(1) below the mitral valve
and resultant narrowing of
the left ventricular outflow
tract (2).
The concavity of the interventricular septum below the mitral valve, along with the elongation and narrowing of the
left ventricular outflow tract, produces a characteristic shape that has been compared to a sitting goose with an
elongated neck on the anteroposterior projection in left ventricular angiography

23. Pediatric Cardiac Tumors
- More commonly benign
- The most common cardiac tumors in fetuses and neonates  Rhabdomyoma,
teratoma, and fibroma.
- Sarcomas are the most common primary malignant cardiac tumors in both
children and adults.
- Metastatic cardiac tumors are less common in children than in adults and include
sarcoma, lymphoma, testicular cancer, and Wilms tumor.

24. A strong association exists between cardiac rhabdomyomas and tuberous sclerosis,
an auto- somal dominant disease characterized by benign hamartomas in multiple
organ systems (7). Cardiac rhabdomyomas may precede skin lesions, such as
hypopigmented “ash-leaf ” macules, and neuroradiolo gic findings, which include
subependymal nodules and cortical tubers, by months or years.
Rhabdomyoma
(a) Transverse US image obtained in a fetus
at 22 weeks of gestation shows a hyperechoic
mass (*) in the interventricular septum. L =
lung. (b, c) Axial half-Fourier acquisition
single-shot turbo spin-echo (HASTE)
(Siemens Healthcare, Erlan- gen, Germany)
(b) and coronal true fast imaging with steady-
state precession (FISP) (c) fetal MR images
obtained at 34 weeks of gestation in the same
patient as in a show a large mass (*) arising
from the interventricular septum, consistent
with a rhabdomyoma. L = lung. (d) Oblique
postnatal echocardiogram shows a large
echogenic rhabdomyoma (*) in the ventricular
septum that fills almost the entire right
ventricle (RV) and a portion of the left
ventricle (LV). On serial echocardiograms
obtained through 2 years of age (not shown),
the rhabdomyoma has been progressively
decreasing in size.

25. Fibroma
Fibromas often are single lesions that do not regress, unlike rhabdomyomas.
Therefore, cardiac fibromas generally are surgically resected.
A fibroma generally is a well-defined mass that is hypo to isointense relative to myocardium on T1WI
and hypointense on T2WI.
Demonstrate slow progressive diffuse or heterogeneous enhancement after administration of
intravenous contrast material, with a hypointense core due to decreased blood supply that is
discernible from the surrounding myocardium
Fibroma in a 4-month-old female infant with a
heart murmur. (a–c) Transaxial black blood (a),
short-axis two-chamber cine (b), and transaxial
contrast-enhanced (c) MR images show a large
isointense and slightly hypointense mass (*)
centered in the right ventricular free wall. It
exerts mass effect on the right ventricular outflow
tract (RVOT).The mass is encapsulated (arrows
in c) and slowly enhances diffusely. A moderate
pericardial effusion is also seen (arrowheads in
b). LV = left ventricle. (d) Two-dimensional gray-
scale US image and color flow map show a large
right ventricular mass (*) filling the right ventricle
and causing flow obstruction through the
tricuspid valve.The patient underwent surgical
resection, and pathologic analysis confirmed

27. Cardiac CTA
Evaluation for :
1. anomalous coronary arteries
2. congenital aortic anomalies such as vascular
rings, interruption, and coarctation
3. anomalous pulmonary venous return
4. branch pulmonary artery stenosis
5. pulmonary artery atresia with major arterio-
pulmonary collateral vessels (MAPCAs)
6. aortic root dissection
7. post operative complications of congenital
heart disease repairs
8. Absolute contraindication to cardiac MR
imaging.
Radiation doses and contrast volume
used in CTA are much less than required
for catheterization.
The contrast dose is usually 1.5-3 ml/kg
with 2ml/kg usually sufficient.
If the contrast bolus has passed the
vessel of interest (delayed acquisition) or
if the contrast has not reached the vessel
of interest (premature triggering) then
the resultant images will be suboptimal.

28. REPORTING
TEMPLATE

29. MRI Cardiac
Indications :
- Evaluation of both congenital and acquired
cardiac conditions as well routine
postoperative surveillance of anomalies
following surgical correction or palliation.
- The cardiac MRI examination :
- an evaluation of cardiovascular
morphology
- quantification of right and left ventricular
function
- quantification of intravascular flow or
obstruction
- tissue discrimination (masses/ myocardial
fibrosis)
The entire cardiac MRI examination :
- Take between 60 and 90 minutes
- Frequently general anesthesia or conscious
sedation is necessary in the pediatric
population.
- MRI in patients with claustrophobia and non
MRI compatible implantable
devices/hardware is contraindicated.

30. TERIMA KASIH