CT in congenital heart diseases

1. Role of CT in Congenital Heart
Disease
By
Dr.M.Gibreel, FEBR, FAHC
Cardiac imaging senior Registrar
AHC & NHI

2.  Segmental approach .
 Shunts ,, ASD, VSD,, PDA.
 Diseases;
 TOF.
 APVD.
 AS & Systemic obstructive lesions.
 Truncus & its DD.
 Vascular rings
 TGA.
 ALCAPA & coronary fistulae.
 Cor triatriatum
 Miscellaneous

3. Anatomical considerations

12. Sagittal plain

13. Segmental Approach
Analysis/ Evaluation of the heart in terms of segments in a step-by-step
fashion based on pattern of recognition of “normal”. Relation of the
segments to each other.
It facilitates communication between physicians from various subspecialties
who are involved in diagnosis and management of CHD.
 The segments are the atria, ventricles, and great arteries
 Visceroatrial situs, the ventricular loop, and the conotruncus.
 The only fixed landmark is: right and left of the patient.,,,,
 Planes of CT .

15. Terminology of Congenital Heart
Disease
 Position.
 Situs.
 Looping.
 Concordance .

16. Cardiac Position = -cardia , relation of
apex to base
 Levocardia (left-sided).
 Dextrocardia (right-sided).
 Mesocardia (centrally located) within the thorax.
 Ectopia cordis (partially or completely outside the thorax).

17. Situs
 Situs = arrangement
 Possibilities :
1)Solitus = usual /normal . LA to the left , RA to the right.
2) Inversus = mirror the solitus . LA to the right , RA to the left.
3) ambiguus= Vague either right or left isomerism Heterotaxy
Syndromes
Hetero = “different”; -taxy = “arrangement”

18. Situs
 Body Segments: Atrial, Tracheo-bronchial, and Abdominal

19. RA
 Appendage (triangular & wide base) + IVC.
 Crista terminalis >> ridge dividing between
the smooth part of the right atrium and the
right atrial appendage

20. RA

21. LA
 Appendage (long & narrow),
trabeculations
 Rhomboid

22. LA

25. Atrial situs

26. Atrial morphology, arrangement and relation to each other
2 morphologically different
atriums, left atrium is to left
side and right atrium to the
right side .
2 morphologically different
atriums, left atrium is to right
side and right atrium to the
left side .
2 morphologically similar
atriums, 2 left atria
2 right atria .

28. Thoraco-
abdominal situs

30. Abdominal Situs

31. Tracheo-bronchial situs

33. Tracheo-bronchial situs

34. Left or right isomerism?Left Right
IVC Interrupted continuous
SVC Bilateral ++ Bilateral
CS LSVC to CS Absent CS
PV Normal to ipslateral TAPVD

35. Ventricular loop / Looping
 Defined by the rotation of the right ventricle (D-loop when the RV seen to the right).
D-loop: RV right-sided relative to LV
L-loop: RV left-sided (levo or L) relative to LV.

36. RV
RV
D-loop L-loop

37. Ventricle The normal ventricle possess 3 components:
 Inlet component: the portion downstream of AV valve annlus = inflow
 Trabecualr component: body of the ventricle distal to the insertion of the
papillary muscle
 Outlet (infundibular)component : portion that supports the arterial valve of the
ventricle= outflow tract or conus.
 Continuity of inlet &outlet.

38.  LV in DORV is accepted to a ventricle though it has no outlet
competent.
 The minimal requirement for chamber to be considered a ventricle
are that it possess an inlet and trabecular competent.
 Chambers which do not have an inlet portion are not ventricles
and are described as rudimentary chambers.
 Rudimentary chambers are 2 types :
 Outlet chambers , if it supplies great vessel
 Trabecular pouches, if no great vessel supplied by it .

40. Ventricular identification
RV
 Pyramidal shape.
 Moderator band.
 Trabeculated septal surface
 TV septal leaflet is more apical i.e. more toward the
apex.
 (TV predilection to septum & reverse to semilunar V.)
 Coarse trabeculations .

42. LV
 Oval shape
 Fine trabeculations
 Smooth septal surface, Papillary muscle.
 (Mitral V predilection to semilunar V i.e. A-M continuity , and the reverse
to the septum).

44. LV

46. LA
RA

49. Version
 Dextroversion= situs solitus + dextrocardia.
 Levoversion = situs inversus + levocardia.

50. Criss cross heart

51. Supero-inferior relation of the ventricles

52. M.Gibreel

53. Criss-Cross heart
 Rare congenital anomaly , 8 per million.
 First described by liv and Rowlatt 1961, the tem
criss-cross introduced by Rob Anderson 1974.
 loss of normal parallel axes of ventricular
inlets with crossing of inflow streams
(twisted AV alignment) ; crossed the AV
connections.

54. Criss-Cross heart
 It is associated with other severe cardiac
anomalies ,, TV & RV hypoplasia , TGA (in 80%) ,
DORV, VSD (rare presented with intact IVS).
 The etiology of criss cross has been not
understood yet . It seems to be due to abnormal
twisting of the apex while the base remains fixed.

55. Criss-Cross heart
 Diagnosed can be made antenatal US or post
natal by echo , as the sonographer / pediatric
cardiologist can not obtain the usual 4
chamber view in one plane with crossed AV
streams.

56. Criss-Cross heart
 Management always directed to the
associated congenital anomalies.

57. RT & LT Inflow

66. AV connections

68. STRADDLING
 A feature of the tensor apparatus (chordae tendineae and papillary
muscles) of an AV valve and indicates anomalous insertion into the
contralateral. ventricle, either along its septum or its free wall

69. Potential AV connections
A
O
PA
RV
LV
RA
Concordant Discordant; cc-TGA CAVC
RV
LV
RA
LA

70. Potential AV connections
Tricuspid atresia DILVMitral atresia
RV
LA
LV
RA
RV
LA
LV
RA
LA
LVRA

71. Great vessels……No isomersum
 Aorta: Arch and neck vessles , giving coronaries
 Pulmonary artery: usually bifurcates

72. Normal
Inversus

76. VA connection,,, VentriculoArterial
Connection
 If Ao from LV and PA from RV: Concordant
 If Ao from RV and PA from LV: Discordant
 3rd type of VA connection: Double outlet, almost always from RV.
 Final type of VA connection: Single outlet (truncus arteriosus).

78. Loop Rule
Assumption used if determination of morphologic ventricles is difficult:
 In presence of a right-sided aortic valve, the RV is to the right of the LV
(D-loop).
 In presence of a left-sided aortic valve, the RV is to the left of the LV (L-
loop).

79. D-TGA L-TGA
Ao PA
RV LV
A
O
PA
LV
R
V
Potential ventriculo-arterial connections

80. Potential ventriculo-arterial connections
DORV
PA
AO
RV

81. Potential ventriculo-arterial connections
Truncus
arteriosus
Ao PA
RV
LV

82. ASD

92. NOT ASD

93. NOT ASD

94. Amplatzer septal occluder
• There must be enough rim of septal tissue around the defect.
• Weight >15 kg.
 Complications
• Early ECG abnormalities , resolve quickly.
• Early or late erosion into aortic root >> unexplained pericardial collection and
tamponade.
• Rare thrombus formation.
• Allergy >> release of nickel from the device.

97. VSD
• Shunting depends on defect size (not location)&
PVR.

99. TYPES

107. Not a VSD

108. PDA

113. PDA closure device

122. WHAT IS TETRLOGY OF FALLOUT IN
CT??
 1- LARGE MALILENED VSD
 2- OVERRIDDING AORT
 3- PULMONARY STENOSIS
 4- RIGHT VENTRICULAR HYPERTROPHY

123. TOF CT FINDINGS

152. APVD T&P

153. TAPVD
 DEF:
No direct communication between the pulmonary veins and the LA. Instead, they
drain anomalously into the systemic venous tributaries or into the RA .
 Types:
1)Supracardiac: 50% of TAPVR patients. The common pulmonary venous sinus
(posterior chamber) drains into the right SVC through the left vertical vein and the left
innominate vein.
2)Cardiac: 20% of TAPVR patients. The pulmonary veins enter the RA separately
through four openings or the common pulmonary venous sinus drains into the
coronary sinus.
3)Infracardiac: 20% of TAPVR patients. The common pulmonary venous sinus drains
to the portal vein, hepatic vein, or IVC. The common pulmonary vein penetrates the
diaphragm through the esophageal hiatus.
4)Mixed type: combination of the other types, 10% of TAPVR patients.

155. Features Common to All Types:
 A large RV with a compressed LV (i.e., relative hypoplasia of the LV) A
RA and a small LA, dilated Pas.
 An interatrial communication is usually present. PFO occurs in 70% of
patients, and secundum ASD occurs in 30%. RESTRICTIVE OR NOT BY
ECHO ,,, CT >> Size otherwise Rashkind procedure.
 A large common chamber .

156. CT role
 Confirm the diagnosis ,,,,, Anatomy .
 Exclude obstruction of the vertical /descending vein.
 Size of ASD is it adequate for mixing or not .
 Describe the course of the vertical vein ,,,, (not intra-parenchymal).

168. PAPVD
 Definition:
One or more (but not all) pulmonary veins drain into the RA or its venous
tributaries such as the SVC, IVC, coronary sinus, and left innominate vein.
The right pulmonary veins are involved twice as often as the left pulmonary
veins.

169. CT role:
 Confirm the diagnosis.
 Full assessment of the pulmonary veins (number, drainage of each
segment , exclude ostial stenosis)
 If u noted four pulmonary veins draining into LA, this doesn't mean normal
venous drainage always ,,,,anomalous segmental drainage may be present .
 Distance of each draining vein to SVC/RA junction.( what above the level
of the RPA /LPA not suitable for repair )

175. Another case

177. Another case (inferior type SVASD)

182. Aortic Stenosis
 Valvular AS is the most frequent(71%) followed by subvalvular stenosis
(23%) and supravalvular stenosis (6%).
 VALVULAR AS,,, BAV.

183.  Supravalvular AS is an annular constriction at the upper margin of the
sinus of Valsalva.
 Subvalvular (subaortic) stenosis >> sub-aortic membrane or tunllel like
LVOT.

184. BAV

185. CT role:
 Confirm the diagnosis & search for other combinations.
 Subaortic membrane ,,its distance from aortic valve . (LVOT VIEWS).
 Aortic valve cuspidity in systole .
 Supravalvular AS in coronal MPR.

195. CoA
 The usual location of COA is juxtaductal, just distal to the left subclavian
artery; less often it is proximal to the origin of the left SCA.
 The most common associated anomaly is bicuspid aortic valve, which
occurs in more than 50% of all patients with COA.
 Intracerebral aneurysm is present in approximately 10% of patients with
COA.

196.  About 20% to 30% of all patients with COA develop CHF by 3 month of
age. If COA is undetected and untreated in a symptomatic infant, early
death may result from CHF and renal shutdown.
 CT role ,,, anatomy ,,, associations (BAV, Arch hypoplasia) & Z-score
correlation ,,,
Level of Coarctation from SCA ,, & its diameters ,area with correlation with
aorta at the diaphragm.

202. CoA + aortic tortuosity syndrome

203. Corkscrew aorta

204. Pseudocoarctation of the Aorta
 Elongated /buckled distal arch & Px descending aorta with narrowing of
the isthmus but without significant obstruction.
 Increased distance between the origins of CCA &SCA.
 The arch is below the level of the clavicle DD : cervical arch.
 Kinking > turbulence > dilatation and aneurysm formation.
 No collaterals (unlike true CoA) , no inferior rib notching.
 Gold standard is the cath >>> no pressure gradient.

206. Interrupted Aortic Arch
 This is an extreme form of COA in which the aortic arch is atretic or a
segment of the arch is absent.
Three types depending on the location of the interruption:
 Type A: The interruption is distal to the left subclavian artery (occurring in
30% of cases).
 Type B: The interruption is between the left carotid and left subclavian
arteries(occurs in 43% of cases). An aberrant right subclavian artery is
common. 50 % have DiGeorge syndrome.
 Type C: rarest The interruption is between the innominate and left
carotid arteries (occurs in 17% of cases).

207.  DiGeorge syndrome
Hypertelorism, short philtrum, downslanting eyes, hypoplasia or absence of
thymus and para-thyroid, hypocalcemia, deficient cell-mediated immunity.
CT Role:
Anatomy of the aorta & associated lesions.
Interruption gap.

210. Critical Type A IAA (small PDA)

211. IAA Type B

212. IAA Type B

214. Huge ascending aorta aneurysm in 12
years female child

217. Truncus Arteriosus

218.  Only a single arterial trunk with a truncal valve leaflets the heart and gives
rise to the pulmonary, systemic, and coronary circulations. A large
perimembranous or subarterial (subtruncal) VSD is present directly below
the truncus .
 The truncal valve may be bicuspid, tricuspid, or quadricuspid, and it is
often incompetent.

219. Types (Collett and Edwards’ classification)

220.  A right aortic arch is present in 30% of patients.
 DiGeorge syndrome with hypocalcemia is present in 33% of patients.
Role of CT
 Confirm the diagnosis , Anatomy & type (with delineation of MPA ,PAs
 Number of sinuses & coronary origins (exclude ostial stenosis) , course &
abnormalities.

221. TA type I

227. Truncus arteriosus Type II

230. Aortopulmonary Window / Aortopulmonary Septal Defect/ Aortopulmonary
fenestration
 a large defect is present between the ascending aorta and the main PA.
 This condition results from failure of the spiral septum to completely
divide the embryonic truncus arteriosus.
 Not common trunk as in Truncus arteriosus.
 CHF and pulmonary hypertension appear in early infancy.
haemodynamics similar to that of a large untreated PDA but not continuous
murmur , it is systolic ejection type.
 This defect never close spontaneously,, ttt >>surgical closure.

232.  A large aorto-pulmonary window is seen, freely
connecting the ascending aorta to the main
pulmonary artery (MPA), measuring about –x- mm in
diameter.

234. POST OPERATIVE APW case
S/P surgical closure of AP window,,, developed infective endocarditis

235. POST OPERATIVE APW case
 S/P surgical closure of AP window,,, developed infective
endocarditis showing:
 Dehiscent closure patch with pseudo-aneurysmal sac formation
and reopening of the AP window.
 Supravalvular pulmonary vegetations with showering to LPA lower
lobe segmental branches that seen near totally occluded (the
lateral and anterior basal) ,, subsequent corresponding patches of
consolidations ,,, likely infected left lower lobar lung infarctions.

236. POST OPERATIVE APW case

237. POST OPERATIVE APW case

238. AORPA
 origin of one PA from the ascending aorta. Usually the RPA.
 Associated defects such as PDA, VSD, and TOF are occasionally present.
 Hemodynamically, one lung receives blood directly from the aorta, as in
PDA , with resulting pressure overload , and the other lung receives the
entire RV output, resulting in volume overload of that lung.
 BVH ,, cardiomegaly & increased pulmonary vascular markings.

241. Vascular Rings
1) Complete vascular ring>>complete circle
2) Incomplete vascular ring >>incomplete circle with direct compression of
the esophagus & trachea.

243. DAA
 4 vessel sign.
 Dominant arch I the higher in lever & slightly larger in diameter.(frequently
the right arch).
Pulmonary sling
 Abnormal origin of the LPA from RPA with pre RMB course behind the
trachea and infront of the esophagus.

247. PULMONARY SLING

250. TGA

251.  In complete TGA, the aorta arises anteriorly from the RV carrying
desaturated blood to the body, and the pulmonary artery (PA) arises
posteriorly from the LV carrying oxygenated blood back to the lungs. In
the classic complete TGA, the aorta is located anteriorly and to the right
(dextro) of the PA. D-transposition(D-TGA).
 When the transposed aorta is located to the left of the PA, it is called L-
transposition (L-TGA).
 Defects that permit mixing of the two circulations (e.g. ASD, VSD,PDA) are
necessary for survival.

254. Atrial switch operation

256. Congenitally Corrected Transposition of the Great
Arteries
 Double discordance.
 Anti-congestive agents / Antiarrhythmic agents.

258. CT assessment of atrial switch
operation complications ; baffle leak,
baffle stenosis,,,,

263. ALCAPA
Anomalous Origin Of The Left Coronary Artery from
The Pulmonary Artery (Bland-White-Garland
syndrome, ALCAPA Syndrome)

264. CT
 ectasia ( especially RCA) and tortuosity of the coronaries.
 Anomalous origin of the LCA better seen in coronal or sagittal MPR
 Dilated LV /thinned out myocardium.
 Contrast streaks or blush seen in MPA base in aortic phase (while rest of
MPA show faint opacification).

268. ARCAPA

269. ARCAPA

270. Coronary arteries fistulas
 1) coronary arteriovenous fistula:
7% of the cases of coronary fistulas .
branching tributary from a coronary artery coursing along a normal anatomic
distribution and eventually entering into the coronary sinus.
 2) coronary fistula /coronary-cameral fistula:
>90% .
Aberrant termination rather than true arteriovenous fistula.
The RCA commonly involved.
The fistula terminates in the right side of the heart (either to the RV or the PA; less
commonly to the RA).
It rarely terminates in the left side of the heart, but if so ,it will enter the LA.

277. Cor Triatriatum
 Rare congenital cardiac anomaly in which ,,, Failure of incorporation of
the embryonic common PV into the LA.
 The LA is divided into two compartments by an abnormal fibromuscular
septum with an opening.
 upper compartment (accessory /Pseudo LA) is a dilated common PV
(=posterior chamber), and the lower compartment is the true LA.
 Hemodynamic abnormalities similar to those of mitrals tenosis in that
both conditions produce pulmonary venous and pulmonary arterial
hypertension.
 DD: Suprarmital ridge (intimately related to mitral valve).

283. PLSVC
 Mainly drains into Coronary sinus>>RA,,, but may drain into LA directly ,,,
CT role:
 Anatomy (drainage ), bridging vein .
 Any anomalous PVs draining into it.
 Exclude atretic coronary sinus and anomalous drainage of coronary
arteries into it .

286. Absent(atretic) LPA or RPA

288. Anomalous LT SCA from MPA

289. PULMONARY VENOUS VARIX

290. SVC VARIX

293. Unroofed CS

294. Thank U
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