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CONGENITAL ACYANOTIC

HEART DISEASES

PARVATHY R

Subdivisions of heart tube & their fate


Fetal heart develop b/n 2nd to 8th week of intrauterine life.

Folding of cardiac tube

UNDERSTANDING THE CARDIAC


SILHOUETTE - PA

LEFT ATRIUM
DOES NOT
FORM ANY
PART OF
CARDIAC
SILHOUETTE
IN A NORMAL
PA VIEW

3 MOGULS OF HEART
LEFT MEDIASTINAL OUTLINE STARTING AT THE AORTIC KNOB.

1. AORTIC KNUCKLE
PROMINENT KNOB = ANEURYSM/HYPERTENSION
ABSENT KNOB = LOOK TO THE RIGHT

2. PULMONARY ARTERY
EXCESSIVE CONVEXITY - POSTSTENOTIC
DILATATION/COPD/PAH/L2R SHUNTS/PERICARDIAL DEFECTS.
SEVERE CONCAVITY - R2L shunts.

3. PROMINENT LEFT ATRIAL APPENDAGE (90%) RHEUMATIC CARDITIS

4. JUST ABOVE THE CARDIOPHRENIC ANGLE- VENTRICULAR


ANEURYSM.

5. AT THE CARDIOPHRENIC ANGLE - PERICARDIAL CYSTS /PROMINENT


FAT PADS/ADENOPATHY.

UNDERSTANDING THE CARDIAC


SILHOUETTE - LATERAL
RETROSTERNAL SPACE:
ANT. MED MASSES
RV

RETROTRACH. SPACE:
VASCULAR
ESOPHAGEAL
THRYOID

RETROCARDIAC SPACE:

HIATUS HERNIA
LV ENLARGEMENT
ESOPHAGEAL DISORDERS
PNEUMONIA

CHAMBER ENLARGEMENT

LEFT ATRIUM

7 CM

1. ATRIAL ESCAPE
2. WALKING MAN SIGN

90 DEG

RIGHT ATRIUM
Atrial bulge too far to the right of the spine
(more than 5.5 cm from the midline on a wellpositioned PA radiograph).
Right atrial convexity to exceed 50% of the
mediastinal or cardiovascular shadow.

LEFT VENTRICLE

RIGHT VENTRICLE

ATRIAL SEPTAL DEFECT

ATRIAL SEPTAL DEFECT

ATRIAL SEPTAL DEFECT

Commonest CHD in adults.


M:F :: 1:6
Types
Ostium secundum type (60% )
Ostium primum (part of
endocardial cushion defect and
is always associated with cleft
mitral valve)
Sinus venosus ( associated
with PAPVC )
Coronary sinus ( least
frequent)

Normal

Right atrium open


looking into left
atrium through
ASD

Looking through
ostium primum
defect
at cleft mitral valve

Proximity of ostium
primum defect to
tricuspid valve

Right atrium open looking


into left atrium through
ASD

CHEST X-RAY FINDINGS

Pulmonary arterial over circulation


( plethora )

Enlargement of RA and RV

Enlargement of main and hilar


pulmonary arterial segments

Small ascending aorta and aortic arch


due to RV hypertrophy & dilatation with
associated rotation, arch of aorta
comes to lie in anterioposterior
position than in normal so knuckle
appear small

ASSOCIATIONS

Aberrant pulmonary veins


Pulmonary valve stenosis
Eisenmenger reaction
Lutembacher syndrome

ASSOCIATIONS
HOLT ORAM syndrome
ostium secundum ASD,
conduction defects and
skeletal abnormalities of
upper extremity (absent
thumb, absent / dysplastic
radius)
CHD a/w upper limb anomaly rather than lower limb anomaly.

ELLIS VAN CREVELD


SYNDROME

Polydactyly, syndactyly, short


nails, single atrium

Echocardiography
Cornerstone of the diagnosis of CHD
2D imaging shows the defect in almost all the cases
Ostium primum & secundum are easily
differentiated.
Sinus venosus are difficult to visualize.
Right side chamber enlargement is well seen

Echocardiography

Doppler study
M mode shows paradoxical motion of the interatrial
septum
Color flow :
In localizing the defect
To diagnose any valvar regurgitation
To R/O Venous anomalies

MR ADJUNCT TO ECHO

ENDOCARDIAL CUSHION DEFECT


(ATRIOVENTRICULAR SEPTAL DEFECT)
40% of ECD patients have
down syndrome
Endocardial cushion tissue
contributes to the formation
of ventricular septum, lower
part of atrial septum and
septal leaflets of mitral and
tricuspid valves.
Incompetence of both AV
valve & ASD, VSD.

CHEST X-RAY FINDING


Skeletal features of
trisomy 21 , such as
11 ribs , double
manubrial
ossification center
and tall vertebral
bodies
All cardiac chambers
will be enlarged
Increased pulmonary
vascularity

DOUBLE MANUBRIAL OSSIFICATION CENTER AND 11 PAIR OF RIBS

VENTRICULAR SEPTAL DEFECT


Most common congenital cardiac
anomaly (20%) ( after bicuspid
aortic valve)
Types
Membranous 70%
Muscular/ Trabecular 20%- Swiss
cheese septum.
(Maladie de Roger)
Atrioventricular 5% (inlet)
Supracristal 5% (outlet) seen in
TOF

inlet

RADIOLOGICAL
FINDINGS
Small VSD NORMAL
Medium to large VSD

Pulmonary arterial over


circulation.
Enlargement of LA, LV &
RV.
Enlargement of main and
central pulmonary arterial
segments
Small thoracic aorta
Right aortic arch is seen in
2% VSD

VSD - LATERAL
VIEW
LA & RV ENLARGEMENT

ECHOCARDIOGRAPHY
Echo is vital in the differential
diagnosis of these conditions.

ECHOCARDIOGRAPHY

Apical four-chamber view

Color Doppler

MRI : has been used with success


to demonstrate the VSD ( adjunct to echo)

Flow jet (*) across the defect into the right ventricle,
indicating a left-to-right shunt.

MRI

MEMBRANOUS TYPE

CARDIAC CT

AORTO PULMONARY SHUNT

Most Frequent Type PDA


OTHERS

artery

Aorto pulmonary window


Aortic sinus fistula
Coronary artery arising from pulmonary
Coronary artery fistula into right heart
Post surgical shunts.

Patent Ductus
Arteriosus

General

Higher incindence in:


Trisomy 21
Trisomy 18
Rubella

Predominance in females 4:1

Anatomy

Ductus connects pulmonary artery to


descending aorta just distal to left subclavian
artery

Physiology

In fetal life, shunts blood from pulmonary


artery to aorta
At birth, increase in arterial oxygen
concentration increases constriction of ductus
Functional closure- 24 hrs
Anatomic closure- 2 months(90%) 1yr(99%)

Pathophysiology

Ductus may persist


Due to defect in muscular wall of ductus or due to
chemical defect in response to oxygen
Anatomic persistence of ductus beyond 4 months
is abnormal
Blood is shunted from aorta to pulmonary arteries

CHEST X-RAY FINDNIGS

Increased pulmonary vascularity.


Enlargement of LA and LV later
Enlargement of main and central
pulmonary arterial segments.

All the above features are


identical to those seen in VSD ,
specific features of PDA are,
Enlargement of aorta and aortic
arch.
Filling up of the angle between the
aortic arch and the pulmonary artery
DUCTUS BUMP : most specific sign

Ductus
bump

IMAGING - RADIOGRAPH

Calcifications
Punctate calcifications at site of closed ductus
is a normal finding
Linear or railroad track calcification at the site
of ductus may be seen in adults with PDA

ECHOCARDIOGRAPHY

Color Doppler in a parasternal short axis view

MRI

MRI : sagittal section

CARDIAC CT

ASSOCIATIONS

Other left to right shunts especially VSD, ASD


Coarctation of aorta
Complete interruption of aortic arch and
hypoplastic left heart syndrome
Eisenmenger reaction

Development of AP septum

AORTOPULMONARY WINDOW

Underdevelopment or
perforation of the
septum dividing the
fetal truncus

Has well-formed aortic


and pulmonary valves

Appearances may be
identical with PDA or
VSD
PAH occurs early.

moderate cardiomegaly, enlargement of aorta and pulmonary arteries and


increase pulmonary vascularity

MRI

Axial and coronal steady-state free precession MRI image

AORTIC SINUS FISTULA

A complication of aneurysmal dilatation of one


of the sinuses of Valsalva.
Most commonly the right coronary or non
coronary aortic sinus perforates.
Radiological appearances are usually
indistinguishable from VSD, ASD or PDA

CORONARY ARTERY SHUNT OR


FISTULA

Usually right coronary artery is involved


Affected artery is always dilated, tortuous and
often aneurysmal
Selective coronary arteriography is
confirmatory

Memory Aid

PARTIAL ANOMALOUS PULMONARY VENOUS


CONNECTION
Some but not all pulmonary veins connects to
systemic circulation rather than to LA.
PAPVC is an acyanotic lesion in comparison to TAPVC
Divided into
Supra cardiac
Cardiac.
Infracardiac seen in scimitar syndrome

SCIMITAR SYNDROME(HYPOGENETIC LUNG


SYNDROME , VENOLOBAR SYNDROME)

More common in girl.


Always almost right sided

1.

Anomalous
pulmonary
SHAPED)

2.

Hypoplasia of lung volume


loss, mediastinal shift

3.

Hypoplasia
of
pulmonary artery.

4.

Anomalous systemic
supply to lower lobe

right lower lobe


vein ( SCIMITAR

ipsilateral
arterial

SCIMITAR SYNDROME

ACYANOTIC HEART DISEASE WITHOUT


SHUNT (NORMAL PULMONARY
VASCULARITY)
Congenital Aortic Stenosis
Congenital Pulmonary Stenosis
Coarctation of Aorta

CONGENITAL AORTIC STENOSIS


Types

Subvalvular
Valvular
Supra valvular
Associated with Williams syndrome

RADIOLOGICAL FEATURES

Cardiomegaly.
LV hypertrophy.
Poststenotic dilatation of aorta in valvular AS.
Small ascending aorta in supravalvular form.

SUPRA
VALVULAR

Small ascending aorta

VALVULAR

Poststenotic dilatation of aorta

CONGENITAL PULMONARY STENOSIS

TYPES
Infundibular ( subvalvular) common in TOF
Valvular (classic PS)
Supravalvular stenosis (60%)

CONGENITAL PULMONARY STENOSIS

Normal pulmonary
vascularity and cardiac size

Post stenotic dilatation of left


pulmonary artery (jet is
angled towards the left
pulmonary artery)

Right pulmonary artery is


normal/smaller in size

RV enlargement may elevate


cardiac apex

Calcified pulmonary arteries


in adults rarely.

COARCTATION OF AORTA
Narrowing at level of distal arch / descending aorta.
Associated with bicuspid aortic valve (75%), cerebral
aneurysms (5-10%) and Turner syndrome (20% have
coarctation)

Post-ductal coarctation

2 M old boy present with heart failure.


Discrepancy b/n diameter of ascending & descending
aorta.

NOTCHING OF RIBS
Due to collateral flow through dilated, tortous ,
pulsatile posterior intercostal arteries
Anterior ribs are spared because anterior intercostal
artery do not run in costal grooves.
Seen > 10 yrs
Not seen in first two ribs as first and second posterior
intercostal arteries arise not from aorta but from
costocervical trunk of subclavian.
Usually bilateral, medial third of postr.ribs not involved

NOTCHING OF RIBS
When coarctation narrows the orifice of LSA , only
right sided notching seen
When there is anomalous origin of RSA distal to
coarctation collaterals fail to develop in right
hemithorax so only left sided notching seen.

PSEUDOCARCTATION OF AORTA
Buckling or kinking of aorta in the vicinity of
ligamentum arteriosum, resulting in elongation,
tortuosity of
distal aortic arch and proximal
descending aorta
Plain X-ray
Figure 3 sign
No rib notching

COR TRIATRIATUM
Obstructing membrane
between the true LA
cavity and the
diverticulum.
Severe pulmonary
venous congestion and
oedema.
The right ventricle is
hypertrophied, the left
ventricle (LV) is of normal
size

COR TRIATRIATUM
Mild pulmonary
edema pattern with
enlarged slightly
hazy pulmonary
vessels.
Cardia is not overtly
enlarged.

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