Congenital heart diseases2020

Imaging of congenital heart diseases
DR.RIYADH W AL ESAWI
DMRD, MSc, PhD

Introduction
• The heart is the first organ to form and become functional ,
emphasizing the importance of transport of material to and from
the developing infant. It originates about day 18 or 19 from the
mesoderm and begins beating and pumping blood about day 21 or
22. It forms from the cardiogenic region near the head and is visible
as a prominent heart bulge on the surface of the embryo. Originally,
it consists of a pair of strands called cardiogenic cords that quickly
form a hollow lumen and are referred to as endocardial tubes.

• These then fuse into a single heart tube and differentiate into five
parts, the truncus arteriosus, bulbus cordis, primitive
ventricle, primitive atrium, and sinus venosus, starting about
day 22. The primitive heart begins to form an S shape within the
pericardium between days 23 and 28. The internal septa begin to
form about day 28, separating the heart into the atria and
ventricles, although the foramen ovale persists until shortly after
birth. Between weeks five and eight, the atrioventricular valves
form. The semilunar valves form between weeks five and nine.

CONGENITAL HEART DISEASE
• Definition:
• Congenital heart diseases are structural or functional circulation
system anomalies which provide clinical symptoms at birth or
in other life periods.

MAIN CATEGORIES OF CONGENIAL HEART
DISEASES
1.Diseases with left-to-right shunt.
2.Diseases with prevailing right-to-left shunt.
3. Valves stenosis and valves or ventricules hypoplasia.
4.Anomalies of great vessels.
5. Position anomalies.
6. Other congenital syndromes.

Another division:
Cyanotic HD with decreased pulmonary flow
• Tetralogy of Fallot (ToF), pulmonary atresia with/without ventricular septum
defect (PA + VSD), tricuspid atresia (TA), Ebstein anomaly.
with increased pulmonary flow
• Transposition of great arteries (TGA), common ventricle (CV), anomalous
pulmonary venous connection (PAPVC, TAPVC) .
with pulmonary stasis
• TAPVC with pulmonary veins stenosis, cor triatriatum, mitral stenosis

• Another division:
Non cyanotic HD with pulmonary vein stasis
Critical aortic coarctation, mitral stenosis, pulmonary veins’ stenosis, hypoplasia
of the left heart syndrome (HLHS)
with increased pulmonary flow
Ventricle septum defect (VSD), patent ductus arteriosus (PDA), atrial septum
defect (ASD), atrio-ventricular septum defect („common canal” – AVSD), truncus
arteriosus comunis (TAC), aorto-pulmonary window (A-P W)

• Another division:
Non cyanotic HD with normal pulmonary flow
Aortic stenosis (AS), coarctation of aorta (CoA), pulmonary stenosis (PS),
vascular ring, corrected TGA

About 80% of all congenital heart diseases are following eight:
ASD, VSD, PDA, AS, PS, CoA, ToF, TGA,

Protocols of the examination in CT and MR:
- general anesthesia in children
- very good venous access (central)
- maximum short time
- maximum temporal and spatial resolution
- optimalization of the parameters

MR TECHNIQUES
For morphological information
- „Black blood” spin-echo (SE)
- „White blood” fast gradient echo, steady-state free precession (SSFP)
- Contrast enhansed angiography MR

For functional information
-Cine imaging fast gradient echo
-Velocity-encoded cine (VEC) MRI
ECG gated (non-gated SSFP in children), slice thickness 5 mm (3 mm), gap
between slices 1 mm, respiratory compensation, gadolinium-enhanced for
3D

INDICATIONS FOR MR IN CHD
1.Complex CHD
2. Different anomalies of aorta
3. Post-operative patient
The first choice in older children and adults

PROTOCOL FOR CT
- flow?
• from 0.5 ml/sec (newborn)
- delay?
• test bolus!
- ml of non-ionic contrast?
• up to 3 ml/kg b.w. in newborn
• up to 2 ml/kg b.w. the rest

Test-bolus to determine correct timing of contrast enhancement (left)
and region of interest placement in the ascending aorta (right).

Sequential Analysis
1: Atrial Situs.
2: Ventricular Morphology.
3: Ventriculoarterial connection.
4: Other abnormalities.
Anatomical considerations

ATRIAL SITUS
LEFT ATRIUM
Receives the 4 pulmonary veins
ANATOMICAL CONSIDERATIONS

Atrial Situs
Right Atrium
Receives:
• SVC
• IVC
• Coronary Sinus
ANATOMICAL CONSIDERATIONS

Ventricular Morphology
Right Ventricle

-VSD
-ASD
-ECD
-PDA
-Aorto-pulmonary window
-PAPVC
Acyanotic heart diseases with increased pulmonary vascularity

1- ASD
ASDs are seen in 1 per 1,500 live births and account for
30–40 % of all adult congenital heart disease
There are five types of ASD according to their
location within the septum:
1-Secundum ASD. 60-90% of all ASDs. usually an
isolated abnormality.
2-Primum ASD. 5-20% ...
3-Sinus venosus. 5% ...
4-Coronary sinus type ASD ("unroofed coronary
sinus") <1%
5-common atrium, combination of two or more defects.

ASD associated with;
Down syndrome –ASD primum
Holt Oram syndrome ASD secondum
Lautembacher syndrome –ASD+ Mitral stenosis
ASD is more common in females, small defect usually
asymptomatic presented in 4th and 5th decades.

Chest x -ray findings
• Cardiomegaly RT.side.
• Normal left heart
• RA enlargement.
• RV enlargement.
• Full pulmonary conus.
• Increased pulmonary vascular
marking, Plethoric lung fields

Panels (a) and (b) Large secondum ASD (black arrowheads) associated with right atrial (panels a and b)
and right ventricular enlargement (panel a), indicating volume overload. Note a good tissue rim, exceeding
3 mm in all directions (black arrows) in both panels.
Marilyn J. Siegel2, Tomasz Miszalski-Jamka3, 4 and Robert Pelberg1
ASD
SECONDUM

Primum atrial septal defect (ASD). An axial CT shows
the ASD (black arrowhead) located between the level of
the fossa ovalis and atrioventricular valves. This defect
occurs in the lower part of the atrial septum close to the
ventricular inlet valves.
ASD PRIMUM

Maximum intensity projections of the sinus venosus atrial septal defect .
Here panels (a), (b), and (c) are axial views each showing 1 of the 3
anomalous pulmonary veins (black arrows, white arrows) entering the
superior vena cava (asterisk). Panel (c) illustrates the SVC straddling both
atria (black arrowhead). The persistent left-sided SVC is again noted by
the black asterisk. White asterisk superior vena cava.
Sinus Venosus ASD
Always with PAPVC

A- Axial steady-state free precession (SSFP) image of 43-year-old woman shows sinus venosus defect (straight arrow)
between superior vena cava (SVC) and left atrium (LA). Note also anomalous right upper lobe pulmonary vein (curved
arrow) opening into SVC.
B-Short-axis SSFP image of 54-year-old woman shows inferior sinus venosus defect (straight arrow) between inferior vena
cava (IVC) and left atrium (LA), with jet (curved arrow) extending from LA to IVC.
A
B
Prabhakar Rajiah1 and Jeffrey P. Kanne2October 2011, Volume 197, Number 4

UNROOFED ASD
Represents an unroofed coronary sinus atrial septal defect in a 33 year old patient with repair in childhood who now presents
with right sided heart failure and evidence of an intracardiac shunt. Panel (a) demonstrates a persistent left sided superior vena
cava entering the coronary sinus. A calcified septal closure patch which does not cover the entire defect is also demonstrted
(arrow). Panel (b) demonstrates an intra-atrial communication via the unroofed coronary sinus.

Axial and sagittal SSFP images show complete unroofing of coronary
sinus (arrow) and direct communication between it and left atrium
(LA). Prabhakar Rajiah1 and Jeffrey P. Kanne2October 2011, Volume 197, Number 4

COMMON ATRIUM
Common atrium (confluence of two or more
types of ASD defects) is characterized by
complete absence of atrial septum and occurs in
association with other complex defects,
frequently heterotaxy syndrome. It is very rarely
seen in the adult population .

PATENT FORAMEN OVALE
Patent foramen ovale (PFO) is the most common form of interatrial
communication. It is caused by a failure of fusion of the flap valve of
the fossa ovalis. The prevalence of PFO declines progressively with
age (34 % up to age 30 years, 25 % for age 30–80 years, and 20 %
older than 80 years).
Two types of PFO exist. The first is the incompetent valve type and
results in right-to-left shunting only when right atrial pressure exceeds
that of the left atrium such as during a Valsalva maneuver.
The second type is called the stretched type and is due to high left
atrial pressure (such as seen in left heart failure) which stretches the
fossa ovalis flap valve to the point of incompetence, resulting in a left-
to-right shunt. While a single defect is most common in PFO, multiple
fenestrations may also be seen.

A- Four-chamber steady-state free precession (SSFP) image of 61-year-old woman shows patent foramen ovale
(arrow) in midportion of interatrial septum.
B- SSFP image of 37-year-old woman shows small patent foramen ovale (straight arrow) with shunting of blood
(curved arrow) from left atrium (LA) to right atrium (RA).
A B

ATRIAL SEPTALANEURYSM
Atrial septal aneurysm (ASA) is associated with a PFO in
30 % of the cases . ASA is defined as an abnormal bulging
of the interatrial septum with an excursion of at least 10
mm and a base span of at least 15 mm.
This is thought to be due to redundancy of the valve of the
fossa ovalis and/or excessive mobility of the atrial septum
with ballooning into the right or left atrial chamber. ASA
may be associated with mitral valve prolapse and atrial
arrhythmias.

Four-chamber steady-state free
precession (SSFP) image of 42-
year-old woman shows normal
appearance of atrial septal occluder
device (arrow). No residual shunt is
seen.

2-VSD
•VSD is the overall second most common congenital cardiac
disease (20%) and is often associated with other congenital
cardiac anomalies, such as tetralogy of Fallot, transposition of
the great arteries, atrioventricular canal, and a single ventricle.
VSD may also be acquired after acute myocardial infarction,
chest trauma, cardiac interventions, or endocarditis.

VSDsmay be classified into four main categories according to their location and
the appearance of the margins of defects.
Type I defects are also known as outlet defects. 5% of all VSDs and are located in the
outlet portions of the left and right ventricles. The superior edge of the VSD is the
joined annulus of the aortic and pulmonary valves. Because the aortic and pulmonary
valves are in fibrous continuity, this type of defect may also be referred to as a supra-
cristal, sub-pulmonary, infundibular, or conoseptal defect.
Type II defects are also called infra- cristal, subaortic, perimembranous, or
paramembranous defects. These defects are the most common type of VSD,
constituting 75% of all cases. They occur around the membranous septum and are
associated with a muscular defect. The defect is just inferior to the aortic valve, and
the annulus of the tricuspid valve contributes to the rim of the defect.

• Type III defects, also called atrioventricular (AV) canal, AV septal, or
inlet septal defects, are located in the posterior region of the septum
beneath the septal leaflet of the tricuspid valve. These defects account
for 10% of all VSD types.
• Type IV is muscular defect account for 10% of all VSDs . They may
be single, but are commonly multiple. the multiple defects occur in the
apical trabecular septum.

Hemodynamics:
LVRVPALALV
Enlarged RV, LV, LA and PA
In case of Eisenmenger syndrome
there is right ventricular to left
ventricular flow.
Clinical:
Smallasymptomatic
Largecongestive heart faliure by 2-3 months
VSD

Chest X ray finding;
Small VSD, normal chest Xray.
Larger VSDs may show cardiomegaly
(particularly left atrial 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
VSD

Chest examination in a 26-year-old man with a muscular VSD. A, Posteroanterior examination shows dilation of the main
pulmonary artery (MP) and hilar right (1 arrow) and left (2 arrow) pulmonary arteries. The upper (3 arrow) and lower (4
arrow) lobe pulmonary arteries are sharp and dilated as well, indicating shunt vascularity. Narrowing of the superior
mediastinum (5 arrow) and displacement of the heart toward the left indicate right heart volume loading. B, Lateral
examination shows filling of the retrosternal space, indicating right heart and main pulmonary artery enlargement. The left
bronchus (1 arrow) is displaced, and the inferior retrocardiac space is filled, indicating left atrial and ventricular
enlargement.

6-year-old boy with a membranous VSD. A, Posteroanterior examination showing typical signs
of shunt vascularity and cardiac enlargement. Right heart enlargement is indicated by the
leftward displacement of the heart from the midline. B, Lateral examination shows filling of the
retrosternal clear space (indicating right heart and pulmonary artery enlargement), and filling of
the superior and inferior retrocardiac space, indicating left atrial and left ventricular
enlargement.

VSD
1- increased vascularity
2- LA enlargement
3-LV enlargement
4-RV enlargement1 2
3
4

Posteroanterior chest examination in a 38-year-old man with an unoperated
membranous VSD.

TTE in subcostal four-chamber view shows a membranous VSD (arrow)

Small defect  Expectant (closes spontaneously)
Large defect  Septal occluding device
A- FETAL SUBAORTIC VSD WITH OVER-RIDING -- B --MUSCULAR VSD
A B

Patent ductus arteriosus or arteriosum (PDA) is a
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.
Epidemiology
PDAs occur in ~1 in 2000 full-term neonates with a F:M of
2:1
3-PDA

Normally closes functionally at 48 hrs &
anatomically at 4 wks
Hemodynamics:
AortaPALALV
LA, LV, Aorta & PA are enlarged
Associations:
Pulmonary atresia, COA
Highest incidence in premature infants with RDS
Clinical:
F > M
Small  asymptomatic; Large  CHF by 2-3
months
Treatment:Indomethacin, Catheter closure or

PDA
1
1-increased vascularity
2-LA enlargement
3-LV enlargement
4-RV normal
4
3
2

Patent ductus arteriosus. Volume render CT and Sagittal steady-state free
precession image of 32-year-old man shows patent ductus arteriosus
(straight arrow) connecting aortic isthmus with main pulmonary artery
(PA), with high-velocity jet (curved arrow) present throughout cardiac

PDA with jet flow from aorta to PA
Sagittal coronal

4-Aorto-pulmonary window
• An aortopulmonary window defect is an abnormal
communication between the ascending aorta and main
pulmonary artery. The presence of both aortic and pulmonic
valves and of an intact ventricular septum distinguishes it
from truncus arteriosus.
• The pulmonary artery and left heart chambers are typically
enlarged. Other associated anomalies include VSD, aortic
coarctation, and patent ductus arteriosus. Aortopulmonary
window defect requires surgical repair during infancy.

aortopulmonary window
defect. Axial SSFP image of
27-year-old woman shows
communication (arrow)
between aorta (AO) and main
pulmonary artery (PA)
consistent with
aortopulmonary window
defect.

Aortopulmonary shunts.A Line
diagram illustrates various
surgically created
aortopulmonary shunts.

Coronal MR angiography image of 21-
year-old man after surgical repair of
tetralogy of Fallot shows absence of right
subclavian artery (arrowheads), which
was taken down for Blalock-Taussig
shunt. Note normal left subclavian artery
(arrow).

5-Endocardial Cushion Defect, AVSD, AVC

In an atrioventricular defect (i.e., an endocardial cushion defect),
defects of the atrial ostium primum and ventricular inlet septum are
associated with a common atrioventricular valve and abnormal
arrangement of the valve leaflets. The various types of atrioventricular
defects include a complete type (ostium primum ASD, inlet VSD, and
cleft mitral and tricuspid valves); a partial type (ostium primum ASD,
no interventricular shunt, normal atrioventricular valves); and a
common atrium . Direct shunting from the left ventricle into the right
atrium may develop. In the most severe form, all four chambers
communicate causing left-to-right and right-to-left shunting
Gerbode defect

Gerbode defect
A Gerbode defect usually congenital and could be acquired, the acquired defects
are seen below the level of insertion of the tricuspid valve in the interventricular
septum and are caused by surgery, infective endocarditis, or myocardial
infarction .

• Hemodynamics:
A-V septal defect
RA, RV, LA, LV & PA are enlarged, Not Aorta
• Radiographic features:
CXR: cardiomegaly, enlarged PA
Angiography: goose-neck deformity of LVOT on RAO view
• Associations:
Down $ (40%)
• Clinical:
congestive heart faliure by 2-3 months
• Treatment:
Surgical closure before 2 years

Increased flow, Global cardiac enlargement right > left
AVSD

AVSD. Axial SSFP image of 2-year-
old girl shows atrioventricular septal
defect with ostium primum atrial
septal defect (arrowhead) and inlet
ventricular septal defect (arrow).

Gooseneck deformity
• 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. 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.

Gooseneck deformity
Endocardial cushion defects,
which account for 4% of all cases
of congenital heart disease,

6-Anomalous pulmonary venous return
• In total anomalous pulmonary venous return (TAPVR) all of
the pulmonary veins drain into the right atrium or a systemic
venous channel at supracardiac, cardiac, or infra-
diaphragmatic levels. Connections may rarely exist at more
than one level. The supracardiac type most commonly
involves a vertical vein that connects to the brachiocephalic
vein. In the cardiac type, the anomalous vein drains directly
into the right atrium or coronary sinus. The infra-
diaphragmatic type typically drains into the portal vein.
Venous obstruction may be present.

• In PAPVR, one to three pulmonary veins drain into the
systemic venous circulation, either directly into the right
atrium or into systemic veins, resulting in a partial left-to-
right shunt. PAPVR is usually an incidental finding occurring
in 0.7% of the population .
• It most commonly affects the left upper lobe (47%), draining
into the left brachiocephalic vein , followed by the right upper
lobe (38%), right lower lobe (13%), and left lower lobe (2%)
.

• PAPVR is bilateral in 4% of patients. A sinus venosus ASD
occurs in 42% of people with PAPVR of the right upper lobe .
MRI can easily identify and quantify a sinus venosus ASD
and associated anomalous pulmonary veins that are otherwise
occult on echocardiography. Transverse plane acquisitions
and 3D MRA or 3D whole-heart SSFP sequences can depict
these lesions. Flow into the right atrium can be confirmed and
quantified with velocity-encoded phase-contrast MRI .

• Scimitar syndrome (i.e., hypo genetic lung or venolobar
syndrome) is characterized by anomalous drainage of part of
or of the entire right lung into the IVC, coronary sinus, right
atrium, azygos vein, portal vein, or hepatic vein in addition to
hypoplasia of the right lung and right pulmonary artery. The
anomalous pulmonary vein curves medially toward the
diaphragm and the IVC, giving the characteristic appearance
of a scimitar .

• Hemodynamics:
- Some (not all) pulmonary veins drain into RA
or systemic veins
- RA, RV & PA are enlarged
• Types:
Supracardiac (SVC, Azygous vein) resemble
Cardiac (RA, coronary sinus) ASD
Infracardiac (IVC, portal vein) Scimitar $
• Associations:
ASD (sinus venosus defect)
Hypogenetic lung $ -- Scimitar $

Three-dimensional volume-rendered
image of 28-year-old woman shows
anomalous drainage of right superior
pulmonary vein (straight arrow) into
superior vena cava (curved arrow). RA =
right atrium.

Axial steady-state free precession
image of 43-year-old woman shows
vertical vein (straight arrow) that
drains left upper lobe into left
brachiocephalic vein (curved
arrow).

• Curved vessel at the right cardiophrenic angel. (curved tubular
structure running parallel to the right heart).
• Hypogenic right lung.
• Increased pulmonary vascularity.
• Radiologically similar to ASD.
SCIMITAR SYNDROME

Scimitar syndrome. Chest x ray, CT MIP, and MRA

• Valvular pulmonary stenosis
• Peripheral pulmonary stenosis
• Aortic stenosis
• Coarctation of aorta
• Interruption of aortic arch
ACYANOTIC CONGRNITAL HEART DISEASES WITH NORMAL
VASCULARITY

• Types:
Vavular (dome-shaped, dysplastic  Noonan $)
Peripheral (main PA, bifurcation, multiple peripheral)
• Hemodynamics:
Obstruction of RV outflow
RV is enlarged (hypertrophy)
Post-stenotic dilatation of Main & left PA in Valvular type
Dilated main &/or left PA, may show mural calcifications
RV hypertrophy
PULMONARY STENOSIS

Valvular pulmonary stenosis
Peripheral pulmonary stenosis
(maternal Rubella, Williams $)
PULMONARY STENOSIS

1-Thickened cusps
2-RV hypertrophy
3- Dilated main and left PA
4- Normal right PA
Pulmonary valve stenosis

AORTIC STENOSIS
• Types:
Sub-vavular
Valvular (bicuspid valve, single horseshoe-shaped valve)
Supravalvular  Williams $ (MR, AS and PS)
• Hemodynamics:
Obstruction of LV outflow
LV is enlarged (hypertrophy)
Post-stenotic dilatation of Aorta in Valvular type
Cardiomegaly (LV hypertrophy), Dilated ascending aorta

AORTIC STENOSIS
Supra-valvular type
(hourglass deformity of ascending aorta)

COA
Coarctation of the aorta accounts for 5%–10%
of congenital cardiac lesions and is usually sporadic.
However, it occurs with increased frequency
among patients with Turner syndrome,
20%–36% of whom are affected. Clinical manifestations vary from
congestive heart failure in infancy to hypertension with differential
pressures between the upper and lower extremities in adulthood.

COA
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. The reverse figure-of-
three sign, a mirror image of the number 3, is observed on the left
anterior oblique view during barium esophagography. This sign is
seen in 50%–66% of adults with aortic coarctation

Types:
Infantile type (diffuse, pre-ductal)
Adult type (localized, post or peri-ductal)
Hemodynamics:
Pre-ductal: R-L shunt via PDA or VSD
Post-ductal: L-R shunt via PDA
Collaterals to descending aorta via IMA, periscapular through intercostals
Associations:
Coarctation $: COA, PDA, VSD
Turner $, Marfan $
Bicuspid aortic valve
Circle of Willis Aneurysm
Clinical:
BP difference between arms and legs
Diffuse type: congestive heart faliure in noenates
Treatment:
Resection & anastomosis, patch angioplasty, balloon angioplasty
COARCTATION OF AORTA

• Aortic figure 3
• Reverse figure 3 of Barium
filled esophagus
• Inferior rib notching (3rd to 8th,
>8 yrs, dt dilated intercostal
arteries)
• LV hypertrophy
COARCTATION OF AORTA

COA
Aortic Caliber change local narrowing turbulent flow

3 figure sign due to pre and post dilatation, reverse 3 sign , dilatation of arch
vessels, collateral circulations (IMA& intercostal)

MRI is the gold standard imaging modality in follow
up post operative.

Coarctation of Aorta (COA)
Inferior rib notching is absent in case of Aberrant SCA with its
origin after COA, thus no collaterals on this side…

50-80% associated with bicuspid aortic valve

I AA
According to the Celoria-Patton classification of IAA ;
type A is defined as interruption distal to the left subclavian artery (42%
of cases);
type B, the interruption is between the left common carotid artery and
subclavian artery (53%).
type C, the interrupted segment is between the innominate artery and
left common carotid artery (4%).

• Occluded Aortic segment
• Mostly at the arch
• Associated with VSD, PDA, DORV
• No aortic knob, large PA
• Neonatal pulmonary edema
Interrupted Aortic Arch

IAA type 1
• 30 Y ,F,HYPERTENSIVE

Just a kink figure 3
No mediastinal widening
No rib notching
No functional stenosis
High riding aortic arch
No collateral
50-80% bicuspid aortic valve
Buckle in descending aorta
Pseudo coarctation of aorta

•‫خير‬ ‫بالف‬ ‫وانتم‬ ‫عام‬ ‫وكل‬ ‫مبارك‬ ‫عيدكم‬
• ‫والسداد‬ ‫بالنجاح‬ ‫لكم‬ ‫تمنياتنا‬

Congenital heart diseases2020

More Related Content

What's hot

Similar to Congenital heart diseases2020

More from RiyadhWaheed

Recently uploaded

Congenital heart diseases2020