主动脉弓变异
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Aortic Arch Anomalies
1
Development of Aortic Arch and great vessels
2
3
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5
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23
Anatomical Categories
• Abnormalities of branching
41
3. R Aortic Arch
Major types
1. R AA with mirror image branching 2. R AA with retro-oesophageal L SCA 3. R AA with retro-oesophageal diverticulum
4. R AA with L descending aorta
5. Double Aortic Arch
6. Persistent Fifth AA
7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies
24
Clinical Classification
31
2. Abnormal L Aortic Arch
1. L AA with retroesophageal R SCA 2. L AA with R Desc Ao & R ductus
Biblioteka Baidu
32
2.1 L AA with retroesophageal R SCA
• Most common arch anomaly – 0.5% of general population • Higher incidence in Downs with CHD – 38% • Mostly asymptomatic
1. Normal L Aortic Arch & Variants
2. Abnormal L Aortic Arch
•
Abnormalities of arch position
3. R Aortic Arch 4. Cervical Aortic Arch
•
Superpneumarary arches
36
2.2 L AA with R Desc Ao & R ductus
• Diagnosis
• Suspect when symps of vascular ring + L aortic arch present
– CxR
• L Ao A + R upper desc. Ao (adults)
1. Normal L Aortic Arch & Variants
2.
Abnormal L Aortic Arch
•
Abnormalities of arch position
3. R Aortic Arch 4. Cervical Aortic Arch
•
Superpneumarary arches
• Vascular rings • Non ring vascular compression of trachea, bronchi, oesophagus • Non compressive arch malformation • Duct dependent arch anomalies
25
7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies
40
3. R Aortic Arch
•
Definition
– Single aortic arch that crosses over the R main bronchus passing to the R of the trachea
Clinical features of vascular rings
• • • • • • Stridor – increase with RTI Recurrent pneumonia/ bronchitis Hyperextension of neck (esp. in infants) Reflex apnoea associated with eating Swallowing difficulty Chocking of food
• Distinctive branching pattern
• CxR/ Ba oesophagography
• R indentation of trachea/oesophagus
• Treatment
• RAA only - No Rx needed
45
3.1 RAA with Mirror Image Branching
• Variant – – L ductus to RE diverticulum from R Desc Ao
Vascular ring No arch vv from diverticulum (Rarely true mirror image of normal – L ductus disappear and R 6th arch continue as ductus)
26
Sidedness of Aortic arch
• L & R aortic arch definitions
– Refers to which bronchus is crossed by the arch
• Normal –
– Cross the L main bronchus at T5 – Branching…. general rule – 1st arch vessel contain a carotid a. contralateral to Ao A
2.
Abnormal L Aortic Arch
•
Abnormalities of arch position
3. R Aortic Arch
4. Cervical Aortic Arch
•
Superpneumarary arches
5. Double Aortic Arch
6. Persistent Fifth AA
• • Size 1>2, 3<4 DD – anomalous R SCA
30
Anatomical Categories
• Abnormalities of branching
1. Normal L Aortic Arch & Variants
2. Abnormal L Aortic Arch
• Abnormalities of arch position
34
2.1 L AA with retroesophageal R SCA
– Ba oesophagography
• Small filling defect slanting up and R
– MRI
35
2.2 L AA with R Desc Ao & R ductus
• Branching pattern similar to previous • Rare • Arch – retro-oesophageal • Desc Ao connected to PA by R ductus ring
• Importance of sidedness of Ao arch
• BT shunt – on side of In A • Repair of oesophageal atresia – side opp arch
27
Anatomical Categories
• Abnormalities of branching
– Angiography, MRI
38
2.2 L AA with R Desc Ao & R ductus
• Rx – R thoracotomy & division of ring
39
Anatomical Categories
• Abnormalities of branching
1. Normal L Aortic Arch & Variants
5. Double Aortic Arch
6. Persistent Fifth AA
7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies
28
1. Normal L Aortic Arch & Variants
• Diagnosis
– – – – – Presentation – vascular ring +CxR – R AA ? RE Div of Com Ba Oesophagogram Echo Angio – charact branching pattern, abrupt change in caliber from diverticulum to SCA – MRI
46
3.2 RAA with Retro-oesophageal diverticulum (Of Kommerell)
• vascular ring+ • Many asymptomatic, in most no other heart defect
47
3.2 RAA with Retro-oesophageal diverticulum (Of Kommerell)
• Almost always ass. with congenital intracardiac disease
– Conotruncal anomalies – TOF, TA, TGA, DORV, LTGA, PA with RV aorta – Other lesions – VSD, PA with IVS
37
2.2 L AA with R Desc Ao & R ductus
• Diagnosis
– Ba oesophagography
• Large indentation directed up and L • DD – R Ao A with retro-oesophageal diverticulum
42
3. R Aortic Arch
• • •
13- 34% of TOF have RAA Incidence in Truncus Arteriosus > that of TOF 8% of DTGA, 16% of TGA+VSD+PS have RAA
43
3.1 RAA with Mirror Image Branching
33
2.1 L AA with retroesophageal R SCA
• Diagnosis
– Echo/angio
• • • • Branch sizes of 1=2, 3=4 1st – no bifurcation, goes to R 2nd,3rd – to L, non bifurcating 4th – towards R, disappear behind trachea
• Ductus is commonly L sided - attached to L innom. A. – no vascular ring
44
3.1 RAA with Mirror Image Branching
• Diagnosis
• Usually no retro-oesophageal compression/ vascular ring • Echo/Angio
3. R Aortic Arch
4. Cervical Aortic Arch
•
Superpneumarary arches
5. Double Aortic Arch
6. Persistent Fifth AA
7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies
Variants
1. Common brachiocephalic trunk
• • Present in 10% of L arches No consequences
29
1. Normal L Aortic Arch & Variants
Variants
2. Separate origin of L vertebral a. from aortic arch (normal – from L subclavian)
1
Development of Aortic Arch and great vessels
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
18
19
20
21
22
23
Anatomical Categories
• Abnormalities of branching
41
3. R Aortic Arch
Major types
1. R AA with mirror image branching 2. R AA with retro-oesophageal L SCA 3. R AA with retro-oesophageal diverticulum
4. R AA with L descending aorta
5. Double Aortic Arch
6. Persistent Fifth AA
7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies
24
Clinical Classification
31
2. Abnormal L Aortic Arch
1. L AA with retroesophageal R SCA 2. L AA with R Desc Ao & R ductus
Biblioteka Baidu
32
2.1 L AA with retroesophageal R SCA
• Most common arch anomaly – 0.5% of general population • Higher incidence in Downs with CHD – 38% • Mostly asymptomatic
1. Normal L Aortic Arch & Variants
2. Abnormal L Aortic Arch
•
Abnormalities of arch position
3. R Aortic Arch 4. Cervical Aortic Arch
•
Superpneumarary arches
36
2.2 L AA with R Desc Ao & R ductus
• Diagnosis
• Suspect when symps of vascular ring + L aortic arch present
– CxR
• L Ao A + R upper desc. Ao (adults)
1. Normal L Aortic Arch & Variants
2.
Abnormal L Aortic Arch
•
Abnormalities of arch position
3. R Aortic Arch 4. Cervical Aortic Arch
•
Superpneumarary arches
• Vascular rings • Non ring vascular compression of trachea, bronchi, oesophagus • Non compressive arch malformation • Duct dependent arch anomalies
25
7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies
40
3. R Aortic Arch
•
Definition
– Single aortic arch that crosses over the R main bronchus passing to the R of the trachea
Clinical features of vascular rings
• • • • • • Stridor – increase with RTI Recurrent pneumonia/ bronchitis Hyperextension of neck (esp. in infants) Reflex apnoea associated with eating Swallowing difficulty Chocking of food
• Distinctive branching pattern
• CxR/ Ba oesophagography
• R indentation of trachea/oesophagus
• Treatment
• RAA only - No Rx needed
45
3.1 RAA with Mirror Image Branching
• Variant – – L ductus to RE diverticulum from R Desc Ao
Vascular ring No arch vv from diverticulum (Rarely true mirror image of normal – L ductus disappear and R 6th arch continue as ductus)
26
Sidedness of Aortic arch
• L & R aortic arch definitions
– Refers to which bronchus is crossed by the arch
• Normal –
– Cross the L main bronchus at T5 – Branching…. general rule – 1st arch vessel contain a carotid a. contralateral to Ao A
2.
Abnormal L Aortic Arch
•
Abnormalities of arch position
3. R Aortic Arch
4. Cervical Aortic Arch
•
Superpneumarary arches
5. Double Aortic Arch
6. Persistent Fifth AA
• • Size 1>2, 3<4 DD – anomalous R SCA
30
Anatomical Categories
• Abnormalities of branching
1. Normal L Aortic Arch & Variants
2. Abnormal L Aortic Arch
• Abnormalities of arch position
34
2.1 L AA with retroesophageal R SCA
– Ba oesophagography
• Small filling defect slanting up and R
– MRI
35
2.2 L AA with R Desc Ao & R ductus
• Branching pattern similar to previous • Rare • Arch – retro-oesophageal • Desc Ao connected to PA by R ductus ring
• Importance of sidedness of Ao arch
• BT shunt – on side of In A • Repair of oesophageal atresia – side opp arch
27
Anatomical Categories
• Abnormalities of branching
– Angiography, MRI
38
2.2 L AA with R Desc Ao & R ductus
• Rx – R thoracotomy & division of ring
39
Anatomical Categories
• Abnormalities of branching
1. Normal L Aortic Arch & Variants
5. Double Aortic Arch
6. Persistent Fifth AA
7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies
28
1. Normal L Aortic Arch & Variants
• Diagnosis
– – – – – Presentation – vascular ring +CxR – R AA ? RE Div of Com Ba Oesophagogram Echo Angio – charact branching pattern, abrupt change in caliber from diverticulum to SCA – MRI
46
3.2 RAA with Retro-oesophageal diverticulum (Of Kommerell)
• vascular ring+ • Many asymptomatic, in most no other heart defect
47
3.2 RAA with Retro-oesophageal diverticulum (Of Kommerell)
• Almost always ass. with congenital intracardiac disease
– Conotruncal anomalies – TOF, TA, TGA, DORV, LTGA, PA with RV aorta – Other lesions – VSD, PA with IVS
37
2.2 L AA with R Desc Ao & R ductus
• Diagnosis
– Ba oesophagography
• Large indentation directed up and L • DD – R Ao A with retro-oesophageal diverticulum
42
3. R Aortic Arch
• • •
13- 34% of TOF have RAA Incidence in Truncus Arteriosus > that of TOF 8% of DTGA, 16% of TGA+VSD+PS have RAA
43
3.1 RAA with Mirror Image Branching
33
2.1 L AA with retroesophageal R SCA
• Diagnosis
– Echo/angio
• • • • Branch sizes of 1=2, 3=4 1st – no bifurcation, goes to R 2nd,3rd – to L, non bifurcating 4th – towards R, disappear behind trachea
• Ductus is commonly L sided - attached to L innom. A. – no vascular ring
44
3.1 RAA with Mirror Image Branching
• Diagnosis
• Usually no retro-oesophageal compression/ vascular ring • Echo/Angio
3. R Aortic Arch
4. Cervical Aortic Arch
•
Superpneumarary arches
5. Double Aortic Arch
6. Persistent Fifth AA
7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies
Variants
1. Common brachiocephalic trunk
• • Present in 10% of L arches No consequences
29
1. Normal L Aortic Arch & Variants
Variants
2. Separate origin of L vertebral a. from aortic arch (normal – from L subclavian)