DAVID SUTTON PICTURES ONLY FOR STUDY PURPOSE CONGENITAL HEART DISEASE

1. 14
DAVID SUTTON

2. DAVID SUTTON PICTURES
DR. Muhammad Bin Zulfiqar
PGR-FCPS III SIMS/SHL

3. • Fig. 14.1 Situs variations. The diagram shows the
arrangement of bronchial, atrial and abdominal
anatomy in the usual arrangement, mirror image
arrangement and right and left isomerism.
(Courtesy of Professor R. Anderson.)

4. • Fig. 14.1 Situs variations. The diagram shows the
arrangement of bronchial, atrial and abdominal
anatomy in the usual arrangement, mirror image
arrangement and right and left isomerism.
(Courtesy of Professor R. Anderson.)

5. • Fig. 14.2 Plain radiograph of the mediastinum
showing normal bronchial sometimes the
azygos vein. anatomy. Arrows indicate the
length of left and right bronchi.

6. • Fig. 14.3 Chest radiograph of a patient with
visceroatrial situs solitus (gastric bubble
arrowed) and isolated dextrocardia.

7. • Fig. 14.4 Chest radiograph of a patient with
total situs inversus (gastric bubble arrowed).

8. • Fig. 14.5 Chest radiograph of an infant with situs
ambiguous showing a centrally positioned cardiac
apex and transverse liver (arrows). There were complex
intracardiac anomalies.

9. • Fig. 14.6 Diagram demonstrating the
difference between right-hand topology (D-
loop) and left-hand topology (L-loop). The two
arrangements are mirror images of each other.
(Courtesy of Professor R. Anderson.)

10. • Fig. 14.7 Chest radiograph showing an isolated
right-sided aortic arch (arrows indicate aortic
knuckle).

11. • Fig. 14.8 (A) Chest radiograph of an infant aged 1 day. The
heart is only slightly enlarged and the child was
asymptomatic. (B) Chest radiograph of the same infant
after 1 month. The heart size has increased and the
pulmonary vasculature is now plethoric. The child had
developed feeding difficulties, and a ventricular septal
defect was diagnosed.

12. • Fig. 14.9 Chest radiograph of a child with a
moderately large atrial septal defect. The
main pulmonary artery segment is large, and
the lung vessels large.

13. • Fig. 14.10 Posterior view of a 99mTc-
microsphere lung scan in a patient with
Eisenmenger's syndrome. Renal uptake (arrows)
is due to right-to-left shunting in the heart.

14. • Fig. 14.11 Corona) spin-echo gated MRI scan showing
a double-inlet left ventricle. The left ventricle is large
and is seen connected to the inflow from the right
atrium (RA). The right ventricle (RV) is rudimentary and
lies in a superior position, filling directly from the left
ventricle (LV) through a large VSD. Ao = aorta.

15. • Fig. 14.12 Two-dimensional echocardiogram
taken from the apex in a child with a s mall
perimembranous ventricular septal defect (VSD).
The margins of the defect act as distinct
echogenic structures. LV = left ventricle; LA = left
atrium.

16. • Fig 14. 13: (A) a parasternal long axis echocardiogram. The arrows
indicate the region for seeking a ventricular septal defect on
Doppler examination. LV = left ventricle; RV = right ventricular
outflow tract; AO = aortic root; LA = left atrium. (B) Parasternal
short-axis echocardiogram at the level of the aortic root. The
arrows indicate the area which should be examined just below the
aortic valve to detect a perimembranous ventricular septal defect.
T = tricuspid valve; A = aortic valve; P = pulmonary valve. (C) =
Apical four-chamber echocardiogram. The arrows indicate where a
muscular ventricular septal defect might be sought using Doppler
techniques. T = tricuspid valve; M = mitral valve.

17. • Fig. 14.14 Colour flow Doppler study taken in
an apical four chamber view. The arrows
indicate the orange flow pattern (toward the
transducer) of an apical muscular defect. LV =
left ventricle. RV = right ventricle.

18. • Fig. 14.15 (A) Left ventricular tine angiogram taken in a
right anterior oblique view. There is simultaneous
filling of the aorta and pulmonary artery, indicating the
presence of a ventricular septal defect which is not
profiled in this projection. (B) Simultaneous view of the
same angiogram but shown in the cranially angled LAO
view. An aneurysmal perimembranous VSD is profiled
(arrow).

19. • Fig. 14.16 Colour flow Doppler study taken
from the subcostal position in an adult with
mitral valve disease. The patent foramen ovale
is seen as an orange jet (toward the
transducer). This was an incidental finding. LA
= left atrium; RA = right atrium.

20. • Fig. 14.17 Chest radiograph of an elderly women with
an ASD and severe pulmonary hypertension. The main
pulmonary artery and hilar pulmonary arteries are very
large, with peripheral vascular attenuation.

21. • Fig. 14.18 Modified apical four-chamber
echocardiogram of a patient with a secundum
ASD. The right-sided chambers are
considerably enlarged. LA = left atrium; RA =
right atrium; LV = left ventricle; RV = right
ventricle.

22. • Fig. 14.19 M-mode echocardiogram of a patient
with an ASD and right ventricular volume
overload. There is 'paradoxical' motion of the
interventricular septum (arrows). LV = left
ventricle; RV = right ventricle.

23. • Fig. 14.20 Colour flow Doppler study in a
patient with a secundum ASD septal defect in
the same orientation as in Fig. 14.18. Flow
through the defect toward the tricuspid valve
is in red (toward the transducer).

24. • Fig. 14.21 (A) Photograph of a nitinol Amplatzer
ASD occluder device in the unexpanded
(deployment) position. (B) The same device
partially stretched; full extension of the device
allows deployment through a catheter delivery
sheath.

25. • Fig. 14.22 (A) Transoesophageal echocardiogram
showing an ASD prior to device closure. LA = left
atrium; RA = right atrium; RV = right ventricle. (B)
Balloon sizing of the defect is performed to determine
the appropriate size of device to use; the balloon
(arrows) lies in the left atrium. (C) The deployed nitinol
Amplatzer device lies in a satisfactory position (arrows)
occluding the ASD. (Courtesy Dr G. Stuart.)

26. • Fig. 14.22 (A) Transoesophageal echocardiogram
showing an ASD prior to device closure. LA = left
atrium; RA = right atrium; RV = right ventricle. (B)
Balloon sizing of the defect is performed to determine
the appropriate size of device to use; the balloon
(arrows) lies in the left atrium. (C) The deployed nitinol
Amplatzer device lies in a satisfactory position (arrows)
occluding the ASD. (Courtesy Dr G. Stuart.)

27. • Fig. 14.23 Suprasternal echocardiogram of a
patient with transposition of the great
arteries and a PDA (D) The great arteries are
parallel in this condition. AO = aorta; MPA =
main pulmonary artery. (Courtesy Dr R.
Martin.)

28. • Fig. 14.24 Continuous-wave Doppler study
taken from the parasternal
• position. Continuous flow through the PDA is
shown above the baseline-toward the
transducer. (Courtesy Dr R. Martin.)

29. • Fig. 14.25 (A) Chest radiograph of a child with a PDA
immediately before closure. The heart is large, and
there is pulmonary plethora. (B) Localised view of the
Rashkind duct occluder in position in the same patient.
(C) Chest radiograph in the same patient 24 hours after
ductal occlusion. The heart has decreased considerably
in size.

30. • Fig. 14.25 (A) Chest radiograph of a child with a PDA
immediately before closure. The heart is large, and
there is pulmonary plethora. (B) Localised view of the
Rashkind duct occluder in position in the same patient.
(C) Chest radiograph in the same patient 24 hours after
ductal occlusion. The heart has decreased considerably
in size.

31. • Fig. 14.26 Isotope perfusion study of a
patient with left pulmonary artery branch
stenosis. There is considerably less uptake of
isotope in the left lung.

32. • Fig. 14.27 Chest radiograph of a child with
pulmonary valve stenosis. The main pulmonary
artery and left pulmonary artery are considerably
enlarged, but pulmonary vascularity is otherwise
normal.

33. • Fig. 14.28 (A) Lateral view of a right ventricular
angiogram in a child with pulmonary valve
stenosis. The doming of the stenotic valve and
the central jet of contrast medium are seen.
There is post-stenotic dilatation of the main
pulmonary artery. (B) Lateral view of pulmonary
valve dilatation in the same patient. The
indentation in the balloon indicates that the valve
is not yet fully dilated.

34. • Fig. 14.29 Continuous-wave Doppler traces
taken from a patient immediately before and
after pulmonary valve dilatation. Peak velocity is
indicated (in m/s), and can be used in the
modified Bernoulli equation (pressure (in mmHg)
= 4 x velocity (in m/s')) to show a predilatation
pressure drop of 67 mmHg reduced to 21 mmHg.

35. • Fig. 14.30 Chest radiograph of an infant with
coarctation of the aorta. There is
cardiomegaly and evidence of left heart
failure.

36. • Fig. 14.31 Localised view of the ribs showing
notching (arrows) in an adult patient
presenting with coarctation of the aorta.

37. • Fig. 14.32 Oblique sagittal gated spin-echo MRI
scan of a child with coarctation of the aorta
(arrow). as = ascending aorta; da = descending
aorta. (Courtesy of the Trustees of the Bristol MRI
Centre.)

38. Fig. 14.33 (A) Digital subtraction study of a left
ventriculogram in the LAO projection. A severe
coarctation of the aorta is seen in the typical
position. (B) Late image from the study in (A)
shows delayed filling of the

39. • Fig. 14.34 Subclavian flap repair for
coarctation of the aorta. (Reproduced from
Jordan & Scott 1989, with permission.)

40. • Fig. 14.35 (A) Short-axis echocardiogram of a
normal aortic valve. showing three leaflets.
(B) Short-axis echocardiogram of a bicuspid
aortic valve.

41. • Fig. 14.36 (A) Parasternal long-axis
echocardiogram showing an obstructive
subaortic membrane. (B) Apical four-chamber
echocardiogram of the same case showing the
obstructive subaortic membrane. LV = left
ventricle; AV = aortic valve; SAS = subaortic
stenosis.

42. • Fig. 14.37 Chest radiograph of an infant with
tetralogy of Fallot. The trachea is indented by
the right-sided aortic arch (arrows), the
cardiac apex is angled upward, and the lung
fields are oligemic.

43. • Fig. 14.38 Colour flow Doppler study in a child with
tetralogy of Fallot. Parasternal long-axis view here is
right-to-left flow from the right ventricle (RV) to the
aorta (AO) The majority of the flow is encoded blue
(away from the transducer), but the fastest moving
central flow shows aliasing (orange). LV = left ventricle;
LA = left atrium.

44. • Fig. 14.39 Thallaum-201 scan in the left
anterior view. In this adult patient with
longstanding tetralogy of Fallot without
complete correction there is marked right
ventricular hypertrophy, with activity
equalling that in the left ventricular wall.

45. • Fig. 14.40 (A) Cranially angled LAO left ventriculogram
of a child with tetralogy of Fallot. There is early
passage of contrast to the right ventricle across the
VSD. Aortic override is seen. (B) Later image from the
same study. The right ventricle is now well filled. The
hypoplastic pulmonary arteries can be seen.

46. • Fig. 14.41 (A) Cranially angled anterior view of a right
ventriculogram of a patient with tetralogy of Fallot. Severe
infundibular stenosis is seen in systole (arrows). The hypoplastic
pulmonary annulus and main pulmonary artery can be seen. (B)
Diastolic image from the same study. The right ventricular
infundibulum is now much wider (arrow).

47. • Fig. 14.42 (A) The classic Blalock shunt. A =
aorta; RPA = right pulmonary artery; RSA = right
subclavian artery. (B) A modified Blalock shunt.
MBS = modified Blalock shunt. RCC = right
common carotid artery; LCC = left common
carotid artery; LSA = left subclavian artery. PAT =
pulmonary artery trunk. (Both diagrams
reproduced from Jordan Scott 1989, with
permission.)

48. • Fig. 14.43 (A) Anterior view of a selective
angiogram of a right Blalock shunt. The right
pulmonary artery is opacified. (B) Anterior view
of a pulmonary arteriogram in the same patient.
There is a severe stenosis at the site of insertion
of the Blalock shunt.

49. • Fig. 14.44 Transverse spin-echo MRI scan of
bilateral seromas (arrows) associated with
bilateral modified Blalock-Taussig shunts.

50. • Fig. 14.45 Frontal chest radiograph showing a
right-sided calcified seroma following a previous
modified Blalock-Taussig shunt.

51. • Fig. 14.46 (A) Normal great arterial connections in the
anterior view. The morphological left ventricle
(smooth outline) lies posteriorly to the morphological
right ventricle (wavy outline) as shown by the
interrupted line. (B) Connections in D-transposition of
the great arteries in the anterior view. Compare with
(A). The great arteries have an anteroposterior
relationship, which gives the narrow pedicle.

52. • Fig. 14.47 (A) Subcostal echocardiogram
showing a Rashkind balloon being drawn
from the right atrium (RA) to the left atrium
(LA) to rupture the atrial septum. (B)
Echocardiogram of the same patient, taken
immediately afterward, showing an ASD
created by the balloon septostomy. (Courtesy
Dr R. Martin.)

53. • Fig. 14.48 Chest radiograph of an infant with
D-transposition of the great arteries. The
pedicle (mediastinum) is narrow, and there is
cardiomegaly and pulmonary plethora.

54. • Fig. 14.49 (A) Normal cardiac connections in the anterior
view. The morphological left ventricle (smooth outline) lies
posterior to the morphological right ventricle (wavy
outline) as shown by the interrupted line. (B) Connections
in L-transposition of the great arteries ('corrected
transposition'). The morphological right ventricle (wavy
line) lies behind the morphological left ventricle (smooth
line) as shown by the interrupted line. The aorta has a
leftward origin, which accounts for the long curved left
heart border seen in some cases.

55. • Fig. 14.50 Chest radiograph of a patient with L-
transposition of the great arteries. There is a long
smooth curve to the left heart border due to the
abnormal leftward origin of the aorta.

56. • Fig. 14.51 Subcostal echocardiogram of a
patient with D-transposition of the great
arteries. The morphological right ventricle
(RV) is much larger than the morphological
left ventricle (LV), and the interventricular
septum (arrows) is curved toward the left
ventricle.

57. • Fig. 14.52 Modified apical four-chamber view in a patient with L-
transposition of the great arteries. The morphological left
ventricle (LV) lies anteriorly and the morphological right ventricle
(RV) lies posteriorly, as shown by the insertions of their respective
atrioventricular valves. The tricuspid valve in the right ventricle is
inserted more apically than the mitral valve. ant = anterior; post =
posterior; RA = right atrium; LA = left atrium. (Compare the valve
insertions with the diagrams in Figs 1 4.1 3C and 14.57A).

58. • Fig. 14.53 (A) Digital subtraction angiogram of a right ventricular injection recorded in the
left anterior oblique projection. The anteriorly placed morphological right ventricle gives
rise to the aorta, indicating D-transposition of the great arteries. (B) Digital subtraction left
ventriculogram of the same patient and in the same projection. The pulmonary artery arises
from the morphological left ventricle.

59. • Fig. 14.54 Mustard procedure for D-transposition of the great
arteries. A prosthetic intra-atrial conduit leads the systemic venous
return from the superior vena cava (SVC) and inferior vena cava
(IVC) to the left ventricle (LV) through the mitral valve (MV). Flow
from the pulmonary veins (PVs) passes over the conduit to reach
the right ventricle (RV) through the tricuspid valve (TV). RA = right
atrium; LA = left atrium, CS = coronary sinus.

60. • Fig. 14.55 (A) Anterior view of an angiogram performed with the
venous catheter (arrows) passed to the superior vena cava (svc) in
a patient with a previous Mustard operation. Contrast medium
flows to the systemic venous atrium (sva) before its passage to the
left ventricle. (B) Similar angiogram to that in (A), but there is
severe postoperative narrowing at the point of entry of the superior
vena cava to the systemic venous atrium. Flow bypasses the
obstruction through a dilated azygos vein.

61. • Fig. 14.56 (A) Cross-clamping of
the aorta prior to the great arterial
switch procedure for D-
transposition of the great arteries.
(B) Division of the great arteries
and excision of the origins of the
coronary arteries with a small
'button' of aortic wall. (C) Re-
anastomosis of the great arteries
and coronary arteries. Systemic
arterial blood flows into the
coronaries from the newly created
'aortic root', previously the main
pulmonary artery. (Reproduced
from Jordan & Scott 1989, with
permission.)

62. • Fig. 14.56 (A) Cross-clamping of the
aorta prior to the great arterial
switch procedure for D-
transposition of the great arteries.
(B) Division of the great arteries
and excision of the origins of the
coronary arteries with a small
'button' of aortic wall. (C) Re-
anastomosis of the great arteries
and coronary arteries. Systemic
arterial blood flows into the
coronaries from the newly created
'aortic root', previously the main
pulmonary artery. (Reproduced
from Jordan & Scott 1989, with
permission.)

63. • Fig. 14.56 (A) Cross-clamping of
the aorta prior to the great
arterial switch procedure for D-
transposition of the great
arteries. (B) Division of the great
arteries and excision of the
origins of the coronary arteries
with a small 'button' of aortic
wall. (C) Re-anastomosis of the
great arteries and coronary
arteries. Systemic arterial blood
flows into the coronaries from
the newly created 'aortic root',
previously the main pulmonary
artery. (Reproduced from Jordan
& Scott 1989, with permission.)

64. • Fig. 14.57 (A) Normal relationships of the interventricular and
interatrial septa with the atrioventricular valves. The
atrioventricular valves are inserted into the septum primum (thin
line). ra = right atrium; la = left atrium; rv = right ventricle; Iv = left
ventricle. (B) Ostium secundum ASD. The atrioventricular valves and
left ventricular outflow tract are normal. (C) Ostium primum ASD.
The septum primum is absent and the atrioventricular valves are
inserted in a low position into the crest of the muscular
interventricular septum. (D) Total AVSD. A large common valve
separates the atrial cavities from the ventricular cavities. There is an
ostium primum ASD and a large VSD in continuity.

65. • Fig. 14.57 (A) Normal relationships of the interventricular and
interatrial septa with the atrioventricular valves. The
atrioventricular valves are inserted into the septum primum (thin
line). ra = right atrium; la = left atrium; rv = right ventricle; Iv = left
ventricle. (B) Ostium secundum ASD. The atrioventricular valves and
left ventricular outflow tract are normal. (C) Ostium primum ASD.
The septum primum is absent and the atrioventricular valves are
inserted in a low position into the crest of the muscular
interventricular septum. (D) Total AVSD. A large common valve
separates the atrial cavities from the ventricular cavities. There is an
ostium primum ASD and a large VSD in continuity.

66. • Fig. 14.58 Subcostal echocardiogram showing
an ostium primum ASD defect lying between
the left atrium (la) and the right atrium (ra).
There is no ventricular septal defect between
the left ventricle (lv) and the right ventricle
(rv).

67. • Fig. 14.59 (A) Normal left ventricular angiogram in the
right anterior oblique projection. Diastolic inflow does
not wash out contrast medium lying below the aortic
root in the left ventricular outflow tract (Ivot). (B) Left
ventricular angiogram performed in a patient with an
AVSD. The normal left ventricular outflow region is
missing due to the absent septum primum, and so the
contrast medium in the subaortic region is washed out
by the incoming mitral flow. This produces the
frequently misinterpreted 'gooseneck‘ appearance.

68. • Fig. 14.60 Chest radiograph of a child with pulmonary
atresia and a VSD. There is a right-sided aortic arch
indenting the trachea which accentuates the concave
pulmonary bay. The left heart border does not show an
upturned apex as seen in Fig. 14.61.

69. • Fig. 14.61 Chest radiograph of an adult with long-term
palliation of pulmonary atresia. There is a right-sided
aortic arch and an upturned cardiac apex. The
vascularity in the right lung is more prominent due to
the presence of a right-sided shunt.

70. • Fig. 14.62 Transverse spin-echo MRI image
showing small pulmonary arteries (arrows) in
a patient with pulmonary atresia.

71. • Fig. 14.63 (A) Chest radiograph of a child with pulmonary
atresia and a left-sided aortic arch. (B) Chest radiograph of
the same patient following closure of the VSD and
insertion of an external valved conduit (arrows) from the
right ventricle to the main pulmonary artery. (C) Lateral
view of (B), showing the metallic frame of the prosthetic
valve in the conduit.

72. • Fig. 14.63 (A) Chest radiograph of a child with pulmonary
atresia and a left-sided aortic arch. (B) Chest radiograph of
the same patient following closure of the VSD and
insertion of an external valved conduit (arrows) from the
right ventricle to the main pulmonary artery. (C) Lateral
view of (B), showing the metallic frame of the prosthetic
valve in the conduit.

73. • Fig. 14.63 (A) Chest radiograph of a child with
pulmonary atresia and a left-sided aortic arch. (B)
Chest radiograph of the same patient following
closure of the VSD and insertion of an external valved
conduit (arrows) from the right ventricle to the main
pulmonary artery. (C) Lateral view of (B), showing the
metallic frame of the prosthetic valve in the conduit.

74. • Fig. 14.64 Left parasternal echocardiogram of
a patient with 'single ventricle'. Both
atrioventricular valves enter the large left
ventricle (LV) from two distinct atria. Outflow
to the aorta is via a restrictive VSD and a small
outflow chamber of right ventricular type
(RV).

75. • Fig. 14.65 Transverse gated spin-echo MRI scan
of a patient with tricuspid atresia. A wedge-
shaped segment of tissue (arrows) lies in the
expected position of the tricuspid valve. rv = right
ventricle; Iv = left ventricle. (Courtesy of the
Trustees of the Bristol MRI Centre.)

76. • Fig. 14.66 Chest radiograph of a patient with
tricuspid atresia. There is pulmonary
oligaemia, a small pulmonary artery, and a
prominent rounded left ventricular curve to
the left heart border.

77. • Fig. 14.67 Subcostal echocardiogram of a
patient with cor triatriatum. A prominent
membrane runs across the left atrium
(arrows). M = mitral valve; LV = left ventricle.

78. • Fig. 14.68 Digital angiogram of a follow-through
pulmonary artery injection in a patient with
partial anomalous pulmonary venous drainage. A
large vein (arrows) is seen draining from the right
lung to the inferior vena cava below the
diaphragm.

79. • Fig. 14.69 Chest radiograph of the patient
shown in Fig. 14.68. The anomalous vein
(scimitar sign) is seen in the right lower zone
(arrows).

80. • Fig. 14.70 (A) Normal pulmonary venous drainage to
the left atrium. (B) TAPVC of the supracardiac type
draining to a left-sided ascending vein and then to the
left brachiocephalic vein. (C) TAPVC of the infracardiac
type showing obstructed drainage to the inferior vena
cava. (D) TAPVC of the cardiac type draining to the
coronary sinus.

81. • Fig. 14.70 (A) Normal pulmonary venous drainage to
the left atrium. (B) TAPVC of the supracardiac type
draining to a left-sided ascending vein and then to the
left brachiocephalic vein. (C) TAPVC of the infracardiac
type showing obstructed drainage to the inferior vena
cava. (D) TAPVC of the cardiac type draining to the
coronary sinus.

82. • Fig. 14.71 Coronal suprasternal
echocardiogram showing TAPVC of the
supracardiac type draining as shown in Fig.
14.7013. IV = brachiocephalic vein or
innominate vein; SVC = superior vena cava; AO
= aorta. (Courtesy Dr R. Martin.)

83. • Fig. 14.72 Follow-through pulmonary arteriogram of a child with
obstructed TAPVC of the intracardiac type draining past an
obstruction at the diaphragmatic level (arrows) to a dilated vein
connecting to the inferior vena cava.

84. • Fig. 14.73 Chest radiograph of a child with TAPVC of
the cardiac type draining to the coronary sinus. There
is marked cardiomegaly and pulmonary plethora but
the upper mediastinum is not wide because the
drainage is directly to the heart.

85. • Fig. 14.74 Chest radiograph of a child with
obstructed TAPVC of the infracardiac type. The
heart borders are obscured by diffuse interstitial
oedema but there is no significant cardiomegaly.

86. • Fig. 14.75 Anatomy of
truncus arteriosus. RV
= right ventricle; LV =
left ventricle; T =
common truncus
arteriosus; PA =
pulmonary artery; Ac,
= aorta. (Reproduced
from Jordan & Scott
1989, with
permission.)

87. • Fig. 14.76 Modified subcostal echocardiogram
in truncus arteriosus. CT = common truncus;
AO = aorta; PA = pulmonary artery. The truncal
valve is arrowed.

88. • Fig. 14.77 Correction of
common truncus artenosus
using the Rastelli procedure. RV
= right ventricle; LV = left
ventricle; Ao = aorta; HAC =
homograft aortic conduit; PA =
pulmonary artery. (Reproduced
from Jordan & Scott 1 989, with
permission.)

89. • Fig. 14.78 Chest radiograph of a child with
severe Einstein's anomaly. There is marked
globular cardiomegaly and pulmonary
oligaemia.

90. • Fig. 14.79 Subcostal echocardiogram in a
child with Ebstein's anomaly. The view shows
the marked right atrial enlargement (ra) and
the prominent tricuspid valve. In spite of the
displacement of the valve, the right ventricle
(rv) is still larger than the left ventricle (lv).

91. • Fig. 14.80 Parasternal short-axis
echocardiogram showing a sinus of Valsalva
aneurysm. (a) RV = right ventricle; RA = right
atrium; LA = left atrium.

92. • Fig. 14.81 Double oblique spin-echo MRI of a Taussig-Bing
anomaly to demonstrate the intracardiac anatomy. There is
a double-outlet right ventricle (RV) with the VSD (arrows)
lying in a subpulmonary position. The aorta (Ao) is distant
from the left ventricle (LV) and 'anatomical repair' by VSD
closure is not possible. PA = pulmonary artery.

93. • Fig. 14.82 (A) Normal left aortic arch branching. (B)
Left aortic arch with an anomalous origin of the right
subclavian artery. (C) Right aortic arch with , mirror
image' branching. This is the commonest type
associated with cyanotic congenital heart disease. (D)
Right aortic arch with an anomalous origin of the left
subclavian artery arising from a posterior diverticulum.
This is the commonest type of right aortic arch to occur
as an isolated abnormality.

94. • Fig. 14.83 (A) Coronal gated spin-echo MRI scan from a child with a
double aortic arch. The ascending aorta (aa) bifurcates at its upper end
(arrows). Iv = left ventricle; pa = pulmonary artery; svc = superior vena
cava; ra = right atrium. (B) A more posterior coronal section from the same
study. The large right and small left arches are shown in cross-section
(arrows) with brachiocephalic arteries arising from them. (C) A yet more
posterior coronal section of the same study, showing confluence of the
two arches to form the descending aorta. The findings were confirmed at
surgery with no angiography being necessary. (Courtesy of the Trustees of
the Bristol MRI Centre.)

95. • Fig. 14.83 (A) Coronal gated spin-echo MRI scan from a child with a
double aortic arch. The ascending aorta (aa) bifurcates at its upper end
(arrows). Iv = left ventricle; pa = pulmonary artery; svc = superior vena
cava; ra = right atrium. (B) A more posterior coronal section from the same
study. The large right and small left arches are shown in cross-section
(arrows) with brachiocephalic arteries arising from them. (C) A yet more
posterior coronal section of the same study, showing confluence of the
two arches to form the descending aorta. The findings were confirmed at
surgery with no angiography being necessary. (Courtesy of the Trustees of
the Bristol MRI Centre.)

96. • Fig. 14.84 (A) Selective right coronary arteriogram in the L
- LAO projection in a child with an aneurysmal fistula to
the right ventricle. (B) Angiogram, from the same patient,
in the same projection immediately after embolisation with
a detachable balloon (arrow). (C) Angiogram in the same
projection taken 1 year later. The fistula remains closed, the
right coronary artery has decreased in size, and the distal
myocardial branches are now seen (arrows). (Courtesy of
Dr G. Hartnell.)

97. • Fig. 14.84 (A) Selective right coronary arteriogram in the L
- LAO projection in a child with an aneurysmal fistula to
the right ventricle. (B) Angiogram, from the same patient,
in the same projection immediately after embolisation with
a detachable balloon (arrow). (C) Angiogram in the same
projection taken 1 year later. The fistula remains closed, the
right coronary artery has decreased in size, and the distal
myocardial branches are now seen (arrows). (Courtesy of
Dr G. Hartnell.)

98. • Fig. 14.85 Bilateral superior
vena cava. A venous catheter
(arrow) has been used for an
angiogram in the left superior
vena cava which drains to the
coronary sinus. There is a large
intercommunicating vein
between the left and right venae
cavae.

99. • Fig. 14.86 Selective coronary angiograms
showing Kawasaki's disease of the left coronary
artery. There is a proximal fusiform aneurysm of
the left anterior descending coronary artery. (A)
Right anterior oblique projection. (B) Left anterior
oblique projection.

100. • Fig. 14.87 Chest radiograph of a child with a
severe dilated cardiomyopathy. There is
marked cardiomegaly and left heart failure.

101. • Fig. 14.88 Transverse echocardiogram of a 20-
week-old fetus showing the 'four-chamber
view'. rv = right ventricle; Iv = left ventricle; s =
spine. The descending aorta is arrowed.

102. • Fig. 14.89 Fetal echocardiogram in a fetus with a
left ventricular cardiomyopathy due to critical
aortic stenosis. The left ventricle (lv) is much
larger than the right ventricle (rv), was visibly less
contractile, and showed endocardial
fibroelastosis (arrows) as an echogenic
endocardium.

103. • Fig. 14.90 M-mode echocardiogram across the normal aortic valve of a 20-
week-old fetus. ma = maternal abdomen. pl = placenta; of = amniotic fluid;
rv = right ventricle; av = aortic valve; la = left atrium. Depth and time
markers are shown, indicating that the heart rate is 150 beats/min and the
aortic root diameter is 4 mm.

104. • Fig. 14.91 a Normal cardiac contour and normal pulmonary artery size.
Normal bronchial anatomy is superimposed. b Normal heart size and
pulmonary vessels with a small and irregular aortic knuckle and rib
notching. Established coarctation in an older child or adult. c Cardiomegaly
and large pulmonary vessels. Left-to-right shunt, particularly ASD or VSD.
Also consider PDA and partially or totally anomalous pulmonary venous
connection of the cardiac type (draining directly to the heart). A left-to-
right shunt alone rarely gives massive cardiomegaly. d Cardiomegaly and
large pulmonary vessels with a very wide upper mediastinum. Totally
anomalous pulmonary venous connection of the supracardiac type. A
large thymus will widen the mediastinum also.

105. • Fig 14.91: e Moderate cardiomegaly and large pulmonary vessels with a small
pulmonary artery segment. Pulmonary arteries must be anatomically abnormal, so
consider D-transposition of the great arteries and truncus arteriosus. The latter is
more likely if the aortic arch is right sided f Large smooth curve to the left heart
border. L-transposition of the great arteries with an abnormal leftward position of
the aorta. The appearance may also be due to other complex malpositions. L-loop
TGA may occasionally have a virtually normal chest X-ray. g Prominent main
pulmonary artery with normal or small pulmonary vessels. Pulmonary valve
stenosis. The left pulmonary artery also may be dilated. The dilatation is not
prominent in subpulmonary stenosis and is not invariably present in valvar
stenosis. h Upturned cardiac apex, right-sided aortic arch, and small pulmonary
vessels. This is almost diagnostic of tetralogy of Fallot, but can be seen in
pulmonary atresia with a VSD and in a few cases of double outlet right ventricle
similar haemodynamics).

106. • Fig 14.91: I Small pulmonary artery and pulmonary vessels with a large rounded
left heart border. Tricuspid atresia. The condition is variable, and there can be
normal or occasionally increased vascularity depending on the haemodynamics of
the VSD and pulmonary valve. Sometimes pulmonary stenosis can give this
appearance. j Very large heart with normal or small pulmonary vessels. Ebstein's
anomaly, dilated cardiomyopathy (including anomalous coronary origin from the
pulmonary artery), and pericardial effusion. There may be associated left heart
failure with cardiomyopathy. k Cardiomegaly with large pulmonary vessels and
pulmonary oedema. Left to right shunt with failure, left heart obstruction (aortic
stenosis or coarctation), severe mitral regurgitation (alone or with other
conditions) and cardiomyopathy. I Small heart with pulmonary oedema.
Obstruction before the heart. Totally anomalous pulmonary venous connection of
the obstructed intracardiac type, cor triatriatum or congenital mitral stenosis.
Pulmonary conditions must be distinguished.