Simple radiological approach and pathophysiology of congenital heart disease

1. Radiographic Presentation of Various
Congenital Heart Disease
Dr. Tarique Ajij,
Resident,
Department of Radio-Diagnosis,
MCH, Kolkata

2. Clues for Evaluation of an Infant with
suspected CHD
1. On History and Physical Examination
color)
 Acyanotic
 Cyanotic
2. Chest roentgenogram: Pulmonary
blood flow may me
 Normal
 Increased/Plethora
 Decreased/Oligemia
3. Electrocardiogram: shows ventricular
hypertrophy
 Right
 Left
 Biventricular
4. Final diagnosis
 Precordial examination
 Echocardiography
 CMRI

4. Atrial Septal Defect
Defect occur in any portion of the
atrium
- Ostium secundum (at fossa
ovalis)
- Ostium primum (ECD) (lower
atrial septum)
- Sinus venosus (upper atrial
septum)

5. Atrial Septal Defect
CXR
Right. V & A enlargement
Large pulm. Artery
↑ed pulm. vascularity

6. Ventricular Septal Defect
 The most common cardiac
malformation
 Defect occur in any portion of the
septum
 Majority membranous
 Muscular – Single or Swiss-cheese
defect
Pathophysiology
 Lt to Rt shunt
 Restrictive if defect is small
(0.5cm2)
 Non-restrictive - large defect (>
1cm2)
- Right and left vent. Pressure
equalized

7. Ventricular Septal Defect
 Small VSD:
Small VSD: normal CXR
 Significant shunt (Qpulm/Qaorta >2):
enlargement of heart, pulmonary
arteries, and LA
 Eisenmenger physiology
 Enlarged pulmonary arteries
 Cardiac and LA enlargement may
decrease RVH
 Peripheral pulmonary arteries become
constricted (“pruning”)
 Calcified PA (rare)

8. Patent Ductus Arteriosus
Functional closure soon after birth
 Aortic end of the ductus distal to
the origin of left subclavian artery
and the other end at bifurcation of
pulmonary artery.
 Male to female ratio 1:2
Pathology – Deficiency of mucoid
endothelial layer & muscular media
in term infant.
Pathophysiology
Lt to Rt shunt
- size
- ratio of pulm. to systemic
resistance
Reversal of shunt

9. Patent Ductus Arteriosus
Small PDA:
1. normal CXR
Large PDA:
1. Increased pulmonary vascularity
2. Enlargement of LA, LV
3. Eisenmenger physiology may develop in
longstanding, severe disease.
 All the above features are identical to those
seen in VSD; specific features to suggest PDA
are:
1. Unequal distribution of pulmonary arterial blood
flow, especially sparing of left upper lobe
2. Enlargement of aorta and AA
3. PDA may be seen as faint linear density
through the PA (occasionally calcifes).

10. AVSD
 40% of ECD patients have trisomy 21
(Down syndrome)
 Types
 Partial AV canal (ostium primum
defect ± cleft in anterior mitral or
septal tricuspid leaflets)
 Transitional AV canal (ostium primum
defect, cleft in both AV valves, defect
in superior IVS)
 Complete AV canal (ostium primum
defect, cleft in both AV valves, large
defect in IVS: either
common AV valve or separate mitral
and tricuspid valves)

11. AVSD
CXR
 Cardiomegaly
 Increased pulmonary vascularity
 Screen for other trisomy 21 findings:
11 ribs, multiple manubrial ossification
centers

12. Pulmonic Stenosis
4 types
1. Valvular
2. Infundibular
3. Supra valvular
4. Peripheral
Pathophysiology
RV outlet obstruction → Pressure work
↓
Rt vent. hypertrophy

13. Pulmonic Stenosis
 CXR:
1. Poststenotic dilatation of main
and/or left PA (jet through stenosed
valve dilates PA)
2. Right PA of normal size
3. RV (hypertrophy)

14. Aortic Stenosis
 3 types
1. Valvular - the commonest
2. Supra valvular
3. Subvalvular (subaortic)

15. Aortic Stenosis
 Plain film
• Cardiomegaly: LV hypertrophy
• Poststenotic dilatation of aorta is
present only in valvular AS.
• CHF

16. Coarctation of the Aorta
 Occur at any site from the arch of
aorta to iliac bifurcation
 98% juxta ductal
 Types
• Infantile type (diffuse type,
preductal): tubular hypoplasia
• Adult type (localized type,
postductal, periductal): short
segment; common

17. Coarctation of the Aorta
 Hemodynamics
• Preductal type has concomitant R-L
shunting via PDA or VSD.
• Postductal coarctation has L-R flow
through PDA.
• Collaterals to descending aorta
Internal mammary-intercostals
Periscapular arteries-intercostals

18. Coarctation of the Aorta
Plain film
 Aortic figure-3 configuration 50%:
 Prestenotic dilatation of aorta
proximal to coarctation
 Indentation of aorta caused by the
coarctation
 Poststenotic dilatation
 Inferior rib notching:
 Secondary to dilated intercostal
arteries
 Only ribs 3 to 8 involved
 Only in children >8 years
 Reverse-3 sign of barium-filled
esophagus
 Prominent left cardiac border from
left ventricular hypertrophy (LVH)
 Normal pulmonary vascularity

19. Tetralogy of Fallot
 Consists:
1. Rt ventricular outflow obst.
2. Ventricular septal defect
3. Dextroposition of the aorta
4. Right ventricular hypertrophy

20. Tetralogy of Fallot
 Plain film
• Boot-shaped heart (enlarged RV):
coeur en sabot
• Right AA, 25%
• Small or concave PA

21. Tetralogy variants
Pink Tetralogy
• VSD with mild pulmonic stenosis
Pentalogy of Fallot
• Tetralogy ± ASD
Trilogy of Fallot
• PA stenosis, RVH with patent foramen
ovale

22. Ebstein Anomaly
Plain film
• Large squared heart (box-shaped heart) due to:
Left side: horizontal position of RV outflow
tract
Right side: RA enlargement
• Decreased pulmonary vascularity

23. Tricuspid Atresia
Plain film
• May be normal
• No TGA: similar appearance as Fallot
• If TGA is present there is:
Increased pulmonary flow
Cardiomegaly
Narrow vascular pedicle

24. TGA
 TGA is the most common CHD
presenting with cyanosis in the first 24
hours of life.
 Types
 D-TGA
 Aorta originates from RV.
 PA originates from LV.
 Normal position of atria and ventricles:
VA concordance
 L-TGA
 Aorta originates from RV.
 PA originates from LV.
 VA concordance
 Abnormal position of atria and ventricles:
VA concordance.

25. D-TGA
Two independent circulations exist:
 Blood returning from body → RV →
blood delivered to body
 Blood returning from lung → LV →
blood delivered to lung
 This circulatory pattern is
incompatible with life unless there
are associated anomalies that permit
mixing of the two circulations (e.g.,
ASD, VSD,
or PDA).

26. D-TGA
Plain film
 “Egg-on-side” cardiac contour:
 narrow superior mediastinum due to
hypoplastic thymus (unknown cause)
 Abnormal relationship of great
vessels
 Right heart enlargement
 Pulmonary trunk not visible because
of its posterior position

27. L-TGA
 Large vessels and ventricles are
transposed (AV and VA discordance).
 Poor prognosis because of associated
cardiac anomalies.
 If isolated, this is an acyanotic lesion.
 Plain film:
 Pulmonary trunk and aorta are not
apparent because of their posterior
position.
 LA enlargement
 Abnormal AA contour because of the
leftward position of the arch
 Right pulmonary hilus elevated over left
pulmonary hilus

28. Truncus Arteriosus
 Results from failure of formation of the spiral
septum within the truncus arteriosus.
 As a result, a single vessel (truncus) leaves
the heart and gives rise to systemic,
pulmonary, and coronary circulation.
 The truncus has 2 to 6 cusps and sits over a
high VSD.
 Types
 Type 1: (most common): short main PA
from truncus
 Type 2: two separate PAs from truncus
(posterior origin)
 Type 3: (least common): two separate PAs
from truncus (lateral origin)
 Type 4: (pseudotruncus) PA from
descending aorta = pulmonary atresia with
VSD; findings of a tetralogy of Fallot
combined with pulmonary atresia

29. Truncus Arteriosus
 Hemodynamics
Admixture lesion with both L-R
(truncus → PA) shunt and R-L (RV →
VSD → overriding aorta) shunt
Plain film
• Enlargement of aortic shadow (which actually
represents the truncus)
• Cardiomegaly due to increased LV volume
• Increased pulmonary vascularity
• Pulmonary edema, occasionally present
• Right AA, 35%

30. TAPVC
 Pulmonary veins connect to systemic
veins or the RA rather than to the LA.
TAPVC exists when all pulmonary veins
connect anomalously. The anomalous
venous return may be obstructed or non-
obstructed
Types
 Supracardiac connection (50%) Supracardiac
TAPVC is the most common type; infrequently
associated with obstruction.
• Left vertical vein
• SVC
• Azygos vein
 Cardiac connection (30%)
• RA
• Coronary sinus
• Persistent sinus venosus
 Infracardiac connection (15%); majority are
obstructed.
• Portal vein
• Persistent ductus venosus
• IVC (caudal to hepatic veins)
• Gastric veins
• Hepatic veins
 Mixed types (5%)

31. TAPVC

32. TAPVC