Congenital aortic diseases by MSCT

1. Aorta BY M.GIBREEL, FEBR Cardiac Imaging Associate consultant NHI&AHC Mohamed Elzoghby, MSc. Tanta university

2. Topics  Anatomical considerations.  Cross-sectional imaging modalities in assessment of arch anomalies.  Development of aortic arch.  SAM,BAV,SVAS,IAA,CoA & other aortic arch anomalies.

3. Anatomical points The aortic annulus lies at an angle of 30° ventrally or anteriorly (to the horizontal plane) It is also directed at an angle of at least 45° to the right (to the median plane).

7. Cross-sectional Imaging  Computed tomography (CT) and magnetic resonance imaging (MRI) provide anatomical assessment by specific oblique reconstructed 2D images and 3D images.  MRI provides also functional assessment of obstructive anomalies by phase- contrast images.

8. 2D oblique sagittal2D Sagittal movie 3D movie Cross-sectional Imaging CT images

9. Cross-sectional Imaging MR images 3D whole heart Contrast MRA T1 Black Blood Phase-Contrast

10. Development of aortic arch

13. Development of aortic arch 1st, 2nd, 5th arches  regress 3rd arch  common & internal carotid A. 4th arch  adult arch (left), subclavian A (right) 6th arch  pulmonary arteries and ductus arteriosus 7th intersegmental A.  subclavian A. Dorsal aortae  descending thoracic aorta

14. Anatomical anomalies Hypothetical Edward’s double arch model of arch sidedness and branching pattern

16. PDA

17. CoA

18. Interrupted Aortic Arch

19. Aberrant Right SCA

20. Double Aortic Arch

21. Right-sided Aortic Arch

22. PDA

23. CoA

24. Interrupted Aortic Arch

25. Aberrant Right SCA

26. Double Aortic Arch

27. Right-sided Aortic Arch

28. Arch variants / anomalies Anatomical Position/shap Right sided arch Cervical arch Circumflex arch Supernumerary Branching Obstructive Double arch PDA (PSAA) Persistent 5th arch Bovine arch Aberrant subclavian A. Isolated subcalvian A. Gothic Interrupted arch Hypoplastic arch Co-arctation of aorta

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

30.  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.

31. BAV

32. 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.

47. Right aortic arch

48. Right aortic arch  Regression of the left 4th dorsal aortic arch segment and persistence of right 4th dorsal aorta arch segment.  Passing above right bronchus and to the right of trachea.  Usually asymptomatic and discovered accidentally but commonly suspected with TOF , Truncus and situs inversus totalis.  There are three main subtypes:  I: with mirror image branching  II: with aberrant left subclavian A.  III: with isolated subclavian A.

55. Isolated SCA  Caused by involution of dorsal and middle parts of 4th arch.  It is connected to either vertebral or pulmonary artery.  May be asymptomatic or presented by upper limb ischemic symptoms.

56. Isolated SCA

58. Cervical aortic arch

59. Cervical aortic arch  Persistence of 2nd or 3rd pharyngeal arch or failure of proper caudal migration of 4th arch.  Usually presented as a pulsatile neck mass.

61. Circumflex aortic arch

62. Circumflex aortic arch  Arch crosses the midline before continuing as descending aorta above Carina level.  complete vascular ring is developed if ductus arteriosus is present on the opposite side

68. Double aortic arch  Persistent of 4th arches (complete on one side and complete or partial on the other side) forming complete vascular ring.  Most frequent cause of symptomatic vascular ring.  4 vessel sign in axial CT…  Dominant arch Is the higher in level & slightly larger in diameter.(frequently the right arch).

69. Double aortic arch  One arch may be atretic and should be differentiated from arch with aberrant subclavian A. by compression signs by non- visualized atertic band.  There are 3 types:  Left dominant  Right dominant  Balanced.

73. Double aortic arch

75. Persistent 6th aortic arch

76. Persistent 6th aortic arch (PDA)  Common anomaly either isolated or associated with other anomaly.  May be large enough to cause significant systemic to pulmonary shunting and congestive heart failure.

79. Persistent 5th aortic arch  Persistent of patent 5th arch leads to systemic to systemic (double lumen arch) or systemic to pulmonary connection.  May be misdiagnosed as dissecting flap.

81. PFAA

83. Bovine aortic arch

84. Bovine aortic arch  Common normal variant of branching pattern.  Right brachiocephalic and left common carotid arteries arise by common trunk.

86. Aberrant subclavian artery

87. Aberrant subclavian artery  Caused by involution of middle segment of contralateral 4th arch between common carotid and subclavian arteries and persistence of dorsal segment.  Dilated proximal part is called Diverticulum of Kommerell and misdiagnosed as double aortic arch

88. Aberrant subclavian artery  Aberrant subcalvian artery passes behind oesophagus.  Complete vascular ring is developed Only ligamentum arteriosus is connecting it to the epsilateral pulmonary artery.

94. Coarctation of aorta

95. Coarctation of aorta  Obstructive narrowing involving the aortic isthmus.  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.  Hemodynamically significant if blood pressure gradient is more than 20 mmHg between the upper and lower extremity.

96. Coarctation of aorta  CTA is able to evaluate site and degree of narrowing, collateral vessels, post- operative evaluation following stent placement.

97. Coarctation of aorta  CTA is able to evaluate site and degree of narrowing, collateral vessels, post- operative evaluation following stent placement.

98. Coarctation of aorta  Phase contrast MRA can assess pressure gradient across coarctation.

99. 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.

107. Pseudo-coarctation of aorta  Pseudo-coarctation is a hemodynamically insignificant anomaly in form of Elongated /buckled distal arch & Px descending aorta with narrowing of the isthmus but without significant obstruction.  Differentiated from true coarctation by:  no pressure gradient across it  absence of collateral vessels,,no inferior rib notching  Increased distance between the origins of CCA &SCA.  absence of left ventricular hypertrophy or ascending aortic aneurysm.  Kinking > turbulence > dilatation and aneurysm formation.  Gold standard is the cath >>> no pressure gradient.

109.  End-to-end anastomosis  Subclavian flap procedure  Patch aortoplasty

111. Hypoplastic aortic arch

112. Hypoplastic aortic arch  Aortic arch hypoplasia is defined in terms of relativity to the external diameter of ascending aorta:  Proximal transverse arch is < 60 % of diameter of ascending aorta  Distal transverse aortic arch is < 50% of diameter of ascending aorta  Isthmus is < 40 % of diameter of ascending aorta

113. Hypoplastic aortic arch

115. 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).

116. CT Role:  Description should include:  level of interruption.  Interruption gap = distance between aortic arch and descending thoracic aorta  branching pattern  other associated anomalies

117. Interrupted aortic arch

120. Critical Type A IAA (small PDA)

121. IAA Type B

122. IAA Type B

124. Rare & miscellaneous conditions

127. CoA + aortic tortuosity syndrome

128. Aortic tortuosity syndrome +CoA

129. Corkscrew aorta

131. Huge ascending aorta aneurysm in 12 years female child

134. Thank U GIPRIANO@YAHOO.COM https://www.facebook.com/mohamed.g.ali.319 https://www.linkedin.com/in/mohamed-gibreel-0660539a/ https://www.youtube.com/channel/UC8UV9MmJijH1-__5OmXqtbQ/featured?view_as=subscriber

Congenital aortic diseases by MSCT

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