Aortic Arch Anomalies1Development of Aortic Arch and great vessels234567891011121314151617181920212223Anatomical Categories Abnormalities of branching Normal L Aortic Arch & Variants Abnormal L Aortic Arch Abnormalities of arch position 3. R Aortic Arch4. Cervical Aortic Arch Superpneumarary arches 5. Double Aortic Arch6. Persistent Fifth AA7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies24Clinical Classification Vascular rings Non ring vascular compression of trachea, bronchi, oesophagus Non compressive arch malformation Duct dependent arch anomalies25Clinical 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 food26Sidedness 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 Importance of sidedness of Ao arch BT shunt on side of In A Repair of oesophageal atresia side opp arch27Anatomical Categories Abnormalities of branching Normal L Aortic Arch & Variants Abnormal L Aortic Arch Abnormalities of arch position 3. R Aortic Arch4. Cervical Aortic Arch Superpneumarary arches 5. Double Aortic Arch6. Persistent Fifth AA7. Interrupted Aortic Arch 8. Anomalous origin of PA branches and other AA anomalies281. Normal L Aortic Arch & Variants Variants 1. Common brachiocephalic trunk Present in 10% of L arches No consequences291. Normal L Aortic Arch & VariantsVariants 2. Separate origin of L vertebral a. from aortic arch (normal from L subclavian) Size 12, 3 that of TOF 8% of DTGA, 16% of TGA+VSD+PS have RAA433.1 RAA with Mirror Image Branching Almost always ass. with congenital intracardiac disease Conotruncal anomalies TOF, TA, TGA, DORV, LTGA, PA with RV aorta Other lesions VSD, PA with IVS Ductus is commonly L sided - attached to L innom. A. no vascular ring443.1 RAA with Mirror Image Branching Diagnosis Usually no retro-oesophageal compression/ vascular ring Echo/Angio Distinctive branching pattern CxR/ Ba oesophagography R indentation of trachea/oesophagus Treatment RAA only - No Rx needed45 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)3.1 RAA with Mirror Image Branching46 vascular ring+ Many asymptomatic, in most no other heart defect3.2 RAA with Retro-oesophageal diverticulum (Of Kommerell)47 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 MRI3.2 RAA with Retro-oesophageal diverticulum (Of Kommerell)483.2 RAA with Retro-oesophageal diverticulum (Of Kommerell) Rx Symptomatic Sx division of ligamentum (L thoracotomy/ Median sternotomy) If resp symps/ dysphagia resection of entire diverticulum (R thoracotomy)49 Loss of L 6th ductal arch and persistence of R 6th No vascular ring Smaller posterior indentation of Oesophagus Rx not needed (no ring) except for ass anomalies3.3 R AA with Retro-oesophageal L SCA50 Diagnosis CxR, Ba Study Echo branching pattern + L desc Ao Angio difficult to DD from Normal L AA go by branching pattern MRI Rx when symptomatic need division3.4 R AA with L Desc Ao & L ductus51 Vascular ring+ Very rare Site of arch dissolution L branch of aortic sac (Exception to the general rule 1st arch vessel contain a carotid a. contralateral to Ao A.)3.5 R AA with Retro-oesophageal Innom A.52 Diagnosis Single carotid A. arising from prox. Aorta DD interrupted AA, isolated L carotid/Innominate A. Differentiating feature normal size AA Rx Division of the ring if symptomatic if still symptomatic detachment of Inn a and reimplantation in to AA3.5 R AA with Retro-oesophageal Innom A.533.6 RAA with isolation of contralateral arch vessels Uncommon Vessel arises exclusively from PA via ductus arteriosus without connection to aorta 3 different forms CHD + in 50% of cases 2/3 have TOF Most common isolation isolated SCA541. Isolation of L SCA Dissolution L 4th arch & L distal dorsal Ao3.6 RAA with isolation of contralateral arch vessels552. Isolation of L CCADissolution L 4th arch & L horn of aortic sac with 6th arch connecting to 3rd arch3.6 RAA with isolation of contralateral arch vessels563. Isolation of L Innom. A Dissolution L horn of aortic sac and distal L dorsal aorta3.6 RAA with isolation of contralateral arch vessels57 Clinical F. Low pulse volume/ BP in affected artery When subclavian and vertebral A are involved subclavian steal syndrome Cer