imaging of cyanotic heart diseases

1. Imaging of Congenital Heart Diseases
DR.RIYADH W AL ESAWI
Assist.prof.diagnostic radiology
Faculty of medicine/ Kufa University
DMRD, MSc, PhD

2. 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

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

4. Congenital Heart Disease

5. Cyanotic heart diseases
Increased Pulmonary Vascularity Decreased Pulmonary Vascularity
Total anomalous pulmonary venous connection
(TAPVC)
Tetralogy of Fallot
Truncus arteriosus Tricuspid atresia
Transposition of great arteries Pulmonary atresia
Single ventricle Pulmonary stenosis and atrial septic defect
Double outlet right ventricle Ebstein's anomaly

6. 1-TGA
Transposition of great artery is the most common cyanotic heart
disease in the neonatal group. Depending on chamber-vessel
connection there are two entities; D-TGA In approximately 60% of
the patients, the aorta is anterior and to the right of the
pulmonary artery (dextro-transposition of the great arteries [d-
TGA]). ,and L-TGA, the aorta is anterior and to the left of the
pulmonary artery (levo-transposition of the great arteries [l-
TGA]). Coronary arteries arise from aorta, from posterior cusps. Due
to atrio-ventricular concordance and ventriculo-arterial discordance,
systemic venous blood passes through the right heart to the aorta and
pulmonary venous blood passes through the left heart to the lungs.

7. From a practical standpoint, the presence or absence of associated
cardiac anomalies defines the clinical presentation and surgical
management of a patient with transposition of the great arteries. The
primary anatomic subtypes are
1- TGA with intact ventricular septum.
2- TGA with VSD.
3- TGA with VSD and left ventricular outflow tract obstruction.
4- TGA with VSD and pulmonary vascular obstructive disease.

8. • In one third of patients with TGA, the coronary artery anatomy is
abnormal, with a left circumflex coronary arising from the right
coronary artery (22%), a single right coronary artery (9.5%), a single
left coronary artery (3%), or inverted origin of the coronary arteries
(3%) representing the most common variants.
• Survival is dependent on the existence of a communication between
pulmonary and systemic circulations, PDA, VSD, ASD.

9. • Total repair of D-TGA involves performing the rerouting the
systemic return to left atrium through a channel (Senning procedure)
and directing pulmonary return to the right atrium. In Rastelli
procedure transventricular rerouting of the left ventricular outflow is
performed. Pulmonary trunk is divided and a conduit is placed within
right ventricle and the pulmonary trunk.
• Jantane procedure is a 3-stage arterial switch procedure.
• Imaging by MDCT and /or MRI is helpful in demonstrating a post-
procedural complications like stenosis at various sites of anastomosis
.
• In L-TGA there is a discordant atrio-ventricular and ventricular
arterial connection. Some patients may be asymptomatic or present
late. Treatment involves the double switch procedure (Senning of
mustard) or Rastelli’s repair.

10. Chest X-Ray- Egg on String
Chest radiograph obtained in a neonate
shows narrowing of the superior
mediastinum, enlargement of the
cardiac silhouette with abnormal
convexity of the right atrial border,
and increased vascular flow—typical
features of transposition of the
great arteries.

11. TGA WITH VSD

12. Egg on string seen in one third of TGA

13. biventricular angiogram

14. TGA. A two-month-old male cyanotic child,
suspected on echocardiography to have TGA,
large VSD, PS, diagnosed with transposition of
great arteries, VSD and PS: (a) diagrammatic
illustration of TGA with narrowing of the
pulmonary outflow; (b) Plain radiography
demonstrate mild biventricular cardiac
enlargement. Lung fields are unremarkable; (c)
Axial MIP image showing anteriorly located
large aorta (arrow) and posteriorly located
pulmonary artery ( open arrows); (d) CT axial
image demonstrates biventricular enlargement
with large ventricular septal defect (circle).
Venkatraman Bhat et al, J Clin Diagn Res. 2016 Jul; 10(7):

15. (e) Lateral MIP CT image
demonstrates anterior location of
aorta (arrow). There is a narrowing
of the pulmonary arterial origin
(open arrow) (f). Modified oblique
view shows large ventricular septal
defect, anteriorly located aorta
(arrow) and posterior pulmonary
artery with narrowing at the origin.
(triangle).
Venkatraman Bhat et al, J Clin Diagn Res. 2016 Jul; 10(7):

16. L-TGA

17. 2-TRUNCUS ARTERIOSUS
TA; Incidence 1 in 11000 live births
Truncus arteriosus is an uncommon cono-truncal anomaly characterized
by a single arterial vessel that originates from the heart, overrides the
ventricular septum, and supplies the all the three, systemic, pulmonary,
and coronary circulation.
Associated with DiGeorge syndrome and chromosome 22q11 deletion .
Echocardiography is adequate for diagnosis and surgical planning in
most of patients. MRI or MDCT may be needed for delineation of
branching pattern of pulmonary arteries, demonstration of
aortopulmonary collateral vessels, pulmonary venous abnormalities and
configuration of aortic arch.

18. Type I = pulmonary trunk arises from proximal truncus
Type II and III= no pulmonary trunk; branch PA arise from
posterior and lateral mid-segments of truncus.
Type IV=pulmonary circulation dependent on MAPCAs.
TA associated with right aortic arch, interrupted aortic arch, COA, PDA, Coronary
artery anomalies, Unilateral absence of pulmonary artery.
Collet and Edwards classification

19. Truncus arteriosus. (a) Diagrammatic
representation of vascular anatomy in truncus
arteriosus; (b) Plain radiograph shows moderate
cardiac enlargement with increased pulmonary
arterial markings, especially on right side(open
arrow); (c) echocardiography shows a single
trunk(open arrow) providing origin to
pulmonary artery and aorta; (d-f) Reconstructed
outflow CT images shows a single large
ascending aorta overriding large septal
defect(circle); (e) Reconstructed axial oblique
CT view shows pulmonary arteries arising from
posterior aspect of aorta (triangle).
Venkatraman Bhat et al, J Clin Diagn Res. 2016 Jul; 10(7):

20. A three -month-old male with breathing
difficulty, recurrent chest infection,
diagnosed with truncus arteriosus; (a)
Axial CT view shows configuration of
aortic arch (arrow) and origin of
pulmonary artery(arrow); (b) 3 D
reconstruction showing larger aorta
(arrow) and RPA and LPA arising
posteriorly (open arrows). Also, there is
an aberrant left subclavian artery
(triangle).
Venkatraman Bhat et al, J Clin Diagn Res. 2016 Jul; 10(7):

21. 3-Total anomalous pulmonary venous
connection -TAPVC
• Supracardiac
• Intracardiac
• infracardaic
• These anomalies involve drainage of the pulmonary veins to
the systemic circulation. Intracardiac TAPVC is associated
with atrial isomerism (polysplenia or asplenia).
• Anomalous pulmonary venous drainage can be associated
with obstruction, especially infracardiac variety.

22. • Plain radiography characteristically shows signs of severe pulmonary
oedema or congestion with normal cardiac size in obstructed TAPVC .
• In supracardiac TAPVC shows cardiomegaly with wide mediastinum
and plethoric lungs. (Snow man or Figure of ‘8’ heart).
• CT or MRI plays a greater role in imaging these anomalies .

23. SNOW MAN
Snowman heart (supracardiac
type only) due to prominent
veins (SVC, innominate) : most
common Increased pulmonary
vascularity.

24. Supracardiac TAPVC. A three-month-old female
presenting with cyanosis while feeding, abnormal vessel
on echography, diagnosed with TAPVC: (a) diagrammatic
representation of confluence of pulmonary veins into a
vertical vein, draining subsequently in to left innominate
vein; (b) Plain radiography demonstrating biventricular
cardiomegaly and plethoric lung fields. Widening of the
mediastinum is noted on left side (arrow); (c,d) Grey
scale and colour Doppler echocardiography demonstrate
ascending vertical vein (triangle and arrows) Both atria
are enlarged; (e) MIP image in coronal plane of contrast-
enhanced CT demonstrates confluence of all pulmonary
veins into vertical vein and innominate vein. (Triangles);
(f) Coronal CT MIP image demonstrates pulmonary
plethora, dilated MPA and vena-cavae (triangles).
Venkatraman Bhat et al, J Clin Diagn Res. 2016 Jul; 10(7):

25. Supracardiac TAPVC. A three-month-old
female presenting with cyanosis while
feeding, abnormal vessel on echography,
diagnosed with TAPVC (g, h) 3-D CT
reconstructed images viewed from
posterior aspect demonstrates anomalous
drainage of all the pulmonary veins into
vertical vein (arrow).
Venkatraman Bhat et al, J Clin Diagn Res. 2016 Jul; 10(7):

26. Intra-cardiac TAPVC. A two-month-old female
presenting with cyanosis while crying, shown to
have a septal defect on echocardiography
diagnosed with intra-cardiac total anomalous
pulmonary venous drainage: (a) Diagrammatic
illustration showing a confluence of pulmonary
veins draining into the enlarged coronary sinus;
(b) Echocardiography shows dilated coronary
sinus draining in to enlarged right atrium
(arrow); (c) Colour Doppler exam shows
enhanced flow in to right atrium via coronary
sinus; (d) Axial CT MIP image shows
confluence of pulmonary veins and dilated
coronary sinus, draining into right atrium (open
arrows).
Venkatraman Bhat et al, J Clin Diagn Res. 2016 Jul; 10(7):

27. A two-month-old female presenting with
cyanosis while crying, shown to have a septal
defect on echocardiography diagnosed with
intra-cardiac total anomalous pulmonary venous
drainage: (a) axial CT images the level of
pulmonary artery revealed grossly dilated MPA
(open arrow); (b) axial CT image demonstrates
grossly enlarged right atrium with a large
secundum septal defect (open arrow). There is
enlargement of the both ventricular cavities; (c)
Coronal CT MIP image shows confluence of
pulmonary veins (arrows); and (d) increased
pulmonary arterial vascularity.
Venkatraman Bhat et al, J Clin Diagn Res. 2016 Jul; 10(7):

28. Infra-cardiac TAPVC. 3-day-old female neonate
presenting with respiratory distress, diagnosed
with infra-diaphragmatic TAPVC to portal vein
on MDCT. (a) Diagrammatic representation of
vascular anatomy in infra-cardiac TAPVC.
Anomalous common pulmonary vein (CPV) is
shown draining to portal vein (colored red); (b)
Plain radiograph shows obscured cardiac
shadow with evidence of pulmonary oedema;
(c)Coronal MIP CT reconstruction show
anomalous CPV traversing the diaphragm and
joining portal vein (arrow). Minimal narrowing
noted at diaphragmatic level (triangle); (d,e)
Axial CT images shows confluence of
pulmonary veins (star) and site of union with
portal vein (open arrow).
Venkatraman Bhat et al, J Clin Diagn Res. 2016 Jul; 10(7):

29. A three-day-old female neonate
presenting with respiratory distress,
diagnosed with infra-diaphragmatic
TAPVC to portal vein on MDCT: (a) 3 D
rendered CT image viewed from
posterior aspect shows the course of
common pulmonary vein (arrows); (b)
Sagittal MIP CT reconstruction show
anomalous common pulmonary vein
traversing the diaphragm with minimal
narrowing at diaphragmatic level
(triangle).Venkatraman Bhat et al, J Clin Diagn Res. 2016 Jul; 10(7):

30. 4- Single Ventricle
Usually left ventricle
Rudimentary RV
No IVS

31. The chest X-ray shows an enlarged heart, long ventricular and long middle left arch, and
increased pulmonary vascular bed. The middle arch suggests the aorta emerging from the
right ventricle to the left. Four-chamber apical echocardiography highlights in A the double
inlet tract of single left ventricle with two atrioventricular valves, and in B, in the same
projection, moderate insufficiency of the right atrioventricular valve (mosaic of regurgitation).
Edmar et al,Arq Bras Cardiol. 2014 Jan; 102(1)

32. 5-Double Outlet Right Ventricle (DORV)
• DORV is a cono-truncal defect characterized by origin of both great arteries
predominantly from RV.
• A ventricular septal defect is invariably present; its location in relation to the
semilunar valves may be subaortic (50%), subpulmonary (30%), uncommitted,
or remote . A large majority (66%) of patients with DORV have some degree of
pulmonary stenosis or atresia. DORV has a complex spectrum of physiology
based on the location of VSD and flow of blood from ventricle to great arteries.
Surgical planning depends upon complexity of physiology, associated anomalies
and whether or not there is pulmonary arterial hypertension. CT imaging with
3D reconstruction of the septum and ventricular outflow provides accurate
characterization of types of VSD with good correlation with surgical
observation and autopsy.

33. DOUBLE OUTLET RIGHT VENTRICLE

34. DORV
(DORV) with aberrant right subclavian artery.
One-year-old male referred to exclude CHD,
diagnosed with a DORV in association with ARSA; (a)
Diagrammatic representation of vascular anatomy in
DORV, both great arteries arising from RV; (b) Plain
radiograph reveal cardiomegaly with oligemic lungs;
(c) Sagittal oblique MIP views shows dilated aortic
arch with normal branching. There is a small
PDA(triangle); (d)Axial MIP images shows
infundibular and valvular stenosis(arrows); (e)
Outflow view shows both aorta and PA arising from
RV, LV communicating with a large VSD(star); (f) CT
Coronal MIP view shows ARSA (open arrow) and
narrowing of origin of LPA (arrow).
Venkatraman Bhat et al, J Clin Diagn Res. 2016 Jul; 10(7):

35. DORV, anomalous conus branch.
One-year-old male presented with the cyanosis on
feeding and feeding difficulty, suspected as TOF on
echocardiography , diagnosed with DORV: (a)
Diagrammatic representation patient of vascular
anatomy in DORV; (b) Plain radiograph shows right
ventricular cardiac configuration, oligemic lungs and
right aortic arch (arrow); (c) Outflow view shows
both aorta and PA arising from RV, LV
communicating with a large VSD(arrow); (d) Axial
CTA reveals normal size pulmonary arteries and right
aortic arch (arrow) (e) Axial CT at aortic root show
conus branch crossing RTOT(open arrow); (f)
Coronal CT reconstruction shows tight infundibular
stenosis (open arrow).
Venkatraman Bhat et al, J Clin Diagn Res. 2016 Jul; 10(7):

36. Cyanotic heart diseases with decreased pulmonary
vascularity

37. 1-TOF• - Most common of cyanotic HD, 6-10%
• Predominant pathology of TOF is the
outflow narrowing of the right ventricle
and varying degree of the narrowing of
pulmonary artery and branches. Other
components include ventricular septal
defect and overriding of aorta.
• CLINICAL:
cyanosis by 3-4 months
• squanting when fatiqued
• episodes of loss of conscious
• Associated with;
• Pulmonary infundibular or valvular stenosis.
• Right aortic arch anomalies, vascular rings or
slings 25%

38. • Hemodynamics:
RV outflow obstructionRV hypertrophy
• Radiographic features:
• Boot-shaped heart (RV enlargement)
• Right AA
• Small PA
• Treatment:
– Total corrective repair
– Blalock-Taussig shunt or
modified one
TOF

39. Plain radiography typically shows
-upturned cardiac apex due to right
ventricular hypertrophy.
-Lungs oligemia
-Prominent right aortic arch is
noted in up to 25% of patients.
TOF

40. Variants of TOF
Pentalogy of Fallot; tetralogy +ASD
Pink tetralogy; VSD with mild pulmonary
stenosis
Trilogy of Fallot; PA stenosis, RVH, patent
foramen ovale
Courtesy CristopherMeyer, MD

41. Venkatraman Bhat et al, J Clin Diagn Res. 2016 Jul; 10(7):
A three-month-old female presented with
cyanosis on feeding, ;
(a) Diagrammatic representation of cardiac
anatomy in TOF;
(b) Plain radiograph shows moderate cardiac
enlargement with upturned apex (RV
configuration) (triangle) and decreased
pulmonary arterial markings;
(c) Echocardiography long axis view
demonstrates a large malaligned
perimembraneous VSD (arrow);
(d) Colour Doppler examination of RV outflow
demonstrates narrowing of pulmonary artery
with turbulent flow.

42. A three-month-old female presented with
cyanosis on feeding, diagnosed as Tetralogy on
echocardiography
(e) axial CT images shows a large subaortic
ventricular septal defect (arrow) and RVH;
(f) Sagittal CT demonstrates large
aorta(triangle) with normal branching pattern;
(g) axial CT image at a slightly higher level
shows severe narrowing of pulmonary
infundibulum and proximal pulmonary artery
(arrow). Right and left pulmonary arteries are
relatively small. Also, there are enlarged
collateral arteries in chest wall (open arrow)
h) 3D reconstruction of aorta shows enlarged
major aorto-pulmonary collateral arteries
(MAPCA) (arrows).
Venkatraman Bhat et al, J Clin Diagn Res. 2016 Jul; 10(7):

43. A 21-year-old female presented with
palpitations, right ventricular outflow
obstruction on echocardiography diagnosed
with PS; (a) Diagrammatic representation of
cardiac anatomy in TOF (b) CXR shows
normal cardiac size and configuration.
Pulmonary arterial markings are decreased; (c)
RVOT reconstructed CT; and (d) axial CT
image at a slightly higher level shows severe
narrowing of pulmonary infundibulum,
pulmonary valve and proximal PA (arrows).
RPA and LPA are relatively small; (e)
Reconstructed CT view shows a large sub-
aortic VSD (arrow) and large overriding aorta.
There is biventricular enlargement and
Venkatraman Bhat et al, J Clin Diagn Res. 2016 Jul; 10(7):

44. Pulmonary Atresia.
An eight-month-old male with cyanosis at birth, feeding
difficulty; failure to demonstrate pulmonary artery on
echocardiography diagnosed with pulmonary atresia: (a)
Diagrammatic representation of cardio-vascular anatomy
in pulmonary atresia; (b) Plain radiograph shows mild
cardiac enlargement with upturned apex (RV
configuration) and decreased pulmonary arterial
markings. Collateral vessel around right hilar region are
prominent (open arrow); (c) axial CT images shows a
large sub-aortic ventricular septal defect (arrow) and
RVH; (d) Reconstructed outflow CT view shows
overriding of aorta and large sub-aortic VSD (arrow).

45. An eight-month-old male with
cyanosis at birth, Diagnosed with
pulmonary atresia: (e) Axial CT
slightly at a lower level shows blind
ending pulmonary infundibulum
(angled arrow). Main pulmonary artery
is absent. Right and left pulmonary
arteries reconstituted by collaterals
(triangles); (f) 3D reconstruction of
aorta shows enlarged collateral arteries
(arrow) (MAPCA).

46. • Pulmonary atresia with bronchial
compression by collateral artery.
• A three-year-old female presenting with
exertional dyspnoea, diagnosed with a
pulmonary atresia on echocardiography:
(a) Diagrammatic representation of
vascular anatomy in Pulmonary atresia;
(b) Plain radiograph shows mild cardiac
enlargement with upturned apex(arrow)
(RV configuration) and decreased
pulmonary arterial markings.

47. TOF absent Pulmonary valve: A One-day-old female
child presenting with respiratory distress, cyanosis,
diagnosed with TOF with absent pulmonary valve on
echo and CT: (a) Diagrammatic representation of vascular
anatomy in TOF with absent pulmonary valve: (b) Plain
radiograph shows hyperinflated left lung (arrow) due to
partial left bronchial compression; (c) Coronal 3-D
airway reconstruction demonstrates left bronchial
compression(triangle); (d) Axial MIP CT image shows
gross dilatation of pulmonary arteries with relative
narrowing at valve level (arrow); (e) CT coronal MIP
view shows severe dilatation of RPA and LPA (triangles);
(f) Axial CT images reveals subaortic VSD (circle).

48. Pseudotruncus:
Pseudotruncus refers to severe form of TOF in which pulmonary
artery and their proximal branches are completely atretic,
disconnecting right ventricles from pulmonary artery . Large
collateral arteries from the aorta provide blood-flow to pulmonary
circulation by reconstituting the more distal pulmonary branches.

49. • Pseudotruncus. A 10-year-male patient
presenting with exertional dyspnoea, investigated
for cardiac disease diagnosed with severe
pulmonary atresia: (a) Diagrammatic
representation of vascular anatomy in severe
pulmonary atresia (pseudo-truncus); (b) axial CT
images shows a large ascending aorta (arrow)
and absent pulmonary artery. Large collateral
artery is seen arising from descending aorta
(open arrow); (c) Reconstructed coronal CT view
shows collateral arteries reconstituting
pulmonary arteries.(triangles); (d) 3D
reconstruction of aorta shows enlarged collateral
arteries (open arrow) (MAPCA).
Venkatraman Bhat et al, J Clin Diagn Res. 2016 Jul; 10(7):

50. 2-Ebstein’s Anomaly
Distal displacement of TV, atrialization of RV, enlarged R atrium
Incidence < 1% of all CHDs
Associated with maternal lithium intake especially in first 8 weeks of
gestation.
80% with ASD with right to left shunt.
Imaging features; oligemic lungs.
Box shape heart due dilated rt.atrium
Normal size left atrium
Small aorta, bulge of MPA.

51. On plain radiography Ebstein’s anomaly has the classic appearance of
‘box shaped heart’. Large heart size is common in Ebstein’s anomaly
due to massively enlarged right atrium, which can also cause a anterior
bulge in lateral chest radiograph. Adequate evaluation can be
performed by echocardiography and MR imaging. Typically there is
caudal displacement, dysplasia of septal and posterior leaflets of
tricuspid valve, dilatation of the right atrium and atrialized portion of
the RV, which may pulsate paradoxically in ventricular systole.
Associated cardiac anomalies and conduction defects can be observed.
Ebstein’s Anomaly

52. MR imaging is superior in demonstrating morphological features,
chamber contractility and measuring regurgitant tricuspid flow, which
has prognostic value.
Management of Ebstein’s anomaly depends upon the age of patient,
severity of the malformation, degree of right ventricular outflow tract
obstruction and dynamic status of pulmonary vascular resistance. The
surgical approach depending on the exact context involve ligation of a
patent ductus arteriosus, placement of a systemic to pulmonary shunt,
tricuspid valve repair or reducing flow across tricuspid valve .

53. Ebstein anomaly, first
described by German
physician Wilhelm Ebstein
in 1866, accounts for 0.5%–
0.7% of cases of congenital
heart disease

54. Ebstein’s Anomaly

55. Ebsteins Anomaly. A 17-year-old male
presenting with cyanosis and diagnosed
with Ebsteins anomaly on Echo and MR:
(a) Diagrammatic representation of cardiac
anatomy in Ebsteins anomaly; (b) Plain
radiograph shows enlarged cardiac shadow,
RA enlargement (arrow) and pulmonary
oligmia; (c) Echocardiography
demonstrating enlarged RA with apical
displacement of septal tricuspid leaflet
(arrow); (d) Axial bright blood MRI
examination shows dilated RA displaced
dysplastic tricuspid valves (open arrow);
(e) Sagittal images shows large RA cavity
and RV outflow tract(circle); (f) Coronal
images illustrates displaced tricuspid valve
(arrow).Venkatraman Bhat et al, J Clin Diagn Res. 2016 Jul; 10(7):

56. Complete agenesis of tricuspid valve
Associations:
ASD (100%), D-TGA (35%), VSD
Hemodynamics:
RAASDLALV
RA, LA and LV are enlarged
RV and PA are small
If TGA is present  increased PA
Treatment:
PG to maintain PDA
Fontan procedure (RA  PA)
3-Tricuspid atresia

57. Tricuspid Atresia
• Tricuspid atresia is the third most common congenital cyanotic heart
disease, accounting for 2 % of all congenital cardiac disorders .
• This is caused by embryological fusion of the dorsal and ventral
endocardial cushions too far to the right lateral position. There is
resultant obstruction to outflow from the RA to the RV; hence, both
atrial septal defect (ASD) and ventricular septal defect (VSD) or a
patent ductus arteriosus (PDA) are necessary for survival. There are
three subtypes: I, normal great arterial arrangement (70 %; II, D-
transposition of the great arteries; III L-TGA.

58. X-rays from a female with tricuspid atresia, normally related great arteries, a nonrestrictive ventricular septal
defect, and a large ostium secundum atrial septal defect. A, At age 11 years, pulmonary vascular resistance was
below systemic, pulmonary vascularity was increased, the left ventricle (LV) was enlarged, and the pulmonary
trunk (PT) and right atrium (RA) were dilated. B, At age 19 years, the pulmonary vascular resistance was supra
systemic, pulmonary vascularity was normal, and the pulmonary trunk and right atrium remained dilated, but the
left ventricle was no longer enlarged. Overlying breast tissue accounts for prominent lower lung field radio
densities.

59. X-rays from an 18-year-old man with tricuspid atresia, complete transposition of the great arteries, a nonrestrictive
ventricular septal defect, and supra systemic pulmonary vascular resistance. A, Pulmonary vascularity is
diminished, and the dilated hypertensive posterior pulmonary trunk is border-forming (PT). The right cardiac
silhouette is hump-shaped because a prominent superior border is caused by an enlarged right atrium (RA) and a
receding inferior border, which is caused by a hypoplastic right ventricle. B, Left anterior oblique projection
highlights the hump-shaped right superior border

60. Tricuspid atresia. (a) Four-chamber steady-state
free precession (SSFP) image shows absent
tricuspid valve, which has been replaced by a
fatty wedge of tissue in the right AV groove
(arrow), a hypoplastic RV and dilated right
atrium (RA). (b) Tricuspid atresia in a patient
with dextro-transposition of the great arteries.
The RV is hypoplastic. Note that this has been
treated with a Fontan shunt (arrow)
Soham et al, Insights Imaging 2016 Oct; 7(5): 649–667

61. 4-PULMONARY ARTERY ATRESIA
• Pulmonary artery atresia (or sometimes known as pulmonary
atresia) there is complete disruption between the right ventricular
outflow tract (RVOT) and the pulmonary trunk.
Incidence is 1 in 10,000 births.
• Dr Vincent Tatco and Dr Yuranga Weerakkody, et al.

62. Pulmonary atresia can be classified into three types .
1- pulmonary atresia with intact interventricular septum (PA-IVS)
2- pulmonary atresia with VSD (PA-VSD)
3- complex pulmonary atresia (pulmonary atresia with complex cardiac
malformation)
Variants
• unilateral pulmonary artery atresia (UPAA)
• isolated unilateral pulmonary artery atresia

63. Radiographic features
Plain radiograph
PA-VSD
normal or mildly enlarged heart with poor or diminished pulmonary arterial
vascular markings.
asymmetrical vascular suggest stenosis within pulmonary arterial tree
there can be plethora due to horizontal arteries forming aortopulmonary
collaterals .
mottled appearance as the lung periphery may suggest pleuro-pulmonary
collateral formation .
PA-IVS
severe cardiomegaly from massive right atrial dilatation
Echocardiography
Echocardiography can characterize intracardiac anatomy. Has a limited role in
assessing pulmonary artery anatomy.

64. • MDCT
MDCT is better than echocardiography for pulmonary artery anatomy. It allows
evaluation of the following .
length of pulmonary atresia
presence of pulmonary artery confluence
• seagull appearance of the confluent pulmonary arteries and abbreviated pulmonary trunk
size of main, right, and left pulmonary arteries at the origin and at the hilum
presence of branch pulmonary artery stenosis
sources of pulmonary blood flow to each lung, including the number of
bronchopulmonary segments supplied by native pulmonary arteries and the
distribution of major aortopulmonary collateral arteries (MAPCAs)
Cardiac MRI
Allows direct visualization of the anomaly. Cine sequences may show a dilated
non contracting right ventricle 5. MR angiography allows detection of
aortopulmonary collaterals and patent ductus arteriosus if present.

65. Associations
tetralogy of Fallot: PA-VSD is considered by some authors
as a severe form of tetralogy of Fallot
truncus arteriosus (Collett and Edwards type IV) or
pseudotruncu : recognized to be a form of PA-VSD rather
than truncus arteriosus .
​heterotaxy syndrome: pulmonary atresia is present in two-
fifths of right isomerism cases.

66. Pulmonary stenosis in heart
failure
•J Somerville and V Grech Images Paediatr Cardiol .12(1); Jan-Mar 2010

67. Pulmonary atresia with VSD
•J Somerville and V Grech Images Paediatr Cardiol .12(1); Jan-Mar 2010

68. CARDIAC MALPOSITION
Situs solitus

69. Situs inversus
Arch on right
Apex on right
Stomach on right
Bibasilar reticulation
Situs inversus, bronchiectasis,
sinusitis==Kartagenars syndrome

70. Situs ambigus
Arch on right
Apex on left
Stomach on right
associations
1-Interruption of IVC
2-Azygous continuation
3-ASD
4-Abdominal heterotaxy
1
2

71. Abdominal heterotaxy== polysplenia

73. Situs Solitus
“normal”
Situs
Inversus
Situs Ambigous
Atrial situs

74. Situs abnormalities and herotaxia

75. Situs ambigus
•J Somerville and V Grech Images Paediatr Cardiol .12(1); Jan-Mar 2010

76. Right aortic arch
Deviation of trachea to
right
Descend of tracheal on
right
No deviation of
esophagus

77. • Vascular rings & slings
• Cor Triatrium
• Hypoplastic left heart (Shone) syndrome
• Cardiosplenic syndromes (Isomerism)
• Azygous continuation of IVC
• Double SVC & IVC
• Coronary anomalies
Other CHD

78. • Complete encirclement of the trachea & esophagus by the AA & its branches or
PA
• Symptoms are related to tracheal compression, less commonly esophageal
compression
• Types:
• Double AA
• Rt arch + Aberrant LSA
• Lt arch + Aberrant RSA
• Pulmonary sling: Aberrant left PA
passing between trachea & esophagus
Vascular Rings & Slings

80. DOUBLE AORTIC ARCH

81. Double Aortic Arch
•Widened superior mediastinum
•Bilateral esophageal indentation, with
the right usually higher

83. Left aortic arch with ARSA

84. Aberrant right subclavian artery .
Most common arch anomaly
Posterior esophageal indentation

85. Right aortic arch common with Fallot.
Aberrant left subclavian artery
Posterior esophageal indentation

86. Pulmonary sling. Aberrant left pulmonary artery arising from right PA
Passes between trachea and esophagus
Anterior esophageal indentation.

87. Pulmonary sling

88. • Incidence 0.1% of CHD
• Incomplete insertion of pulmonary veins into LAobstructionPVH
• Appears like a septum within normal sized LA
• classic cor triatriatum (cor triatriatum sinistrum / sinister): left atrium commoner
• cor triatriatum dextrum/dexter, right atrium.
Cor triatrium

89. • Hypoplastic LA, LV, Mitral valve, Aortic valve & Aorta
• Associated with ASD & PDA, infants die within 1st week as PDA closes
• CHF, severe pulmonary edema
Hypoplastic left heart ; Shones syndrome

92. Anomalous left coronary artery (malignant
course)

93. Myocardial bridging

94. Anomalous origin of the non-dominant LCX from the proximal most
segment of the RCA. Its proximal segment runs a retro-aortic course.
Mustafa et al,The Egyptian Journal of Hospital Medicine (July 2018) Vol.
72 (4), Page 4270-4277

95. Congenital and atherosclerotic coronary artery disease;
Anomalous origin of the dominant LCx from the right coronary sinus by a common ostium
with the RCA. Its proximal segment runs a retro-aortic course.
Mustafa et al,The Egyptian Journal of Hospital Medicine (July 2018) Vol.
72 (4), Page 4270-4277

96. Anomalous origin of the LMT from the right coronary sinus by a separate ostium
from the RC sinus, running an inter-arterial course and showing an average
caliber.
Mustafa et al,The Egyptian Journal of Hospital Medicine (July 2018) Vol.
72 (4), Page 4270-4277

97. Congenital and atherosclerotic coronary artery disease;
Anomalous origin of the RCA from the left coronary sinus by a separate ostium from the LMT. Its proximal
segment runs a short inter-arterial course without significant caliber attenuation.
Possible myocardial bridges at the mid and distal LAD segments.
Mustafa et al,The Egyptian Journal of Hospital
Medicine (July 2018) Vol. 72 (4), Page 4270-4277

98. Congenital coronary fistula; LCX to the right atrium.
Mustafa et al,The Egyptian Journal of
Hospital Medicine (July 2018) Vol. 72
(4), Page 4270-4277

100. GREAT THANKS