High-quality echocardiography can reveal types of interrupted aortic arch and associated cardiac lesions. Very rarely there have been cases of patients surviving longer with significant collaterals supplying the lower half of the body. Management of the interruption requires timely diagnosis, use of prostaglandin E infusion immediately after birth to ensure end-organ perfusion, and eventual surgical repair of the interruption. Unrecognized and untreated cases have a median age of death at 4–10 days of life. Perfusion distal to the interruption is dependent on the patency of the ductus arteriosus. 2019 Mar 12 73(9 Supplement 1):2516.The interrupted aortic arch is very rare and occurs in about 1% of the infants with critical congenital heart disease. Journal of the American College of Cardiology. A RARE CASE OF INTERRUPTED AORTIC ARCH AS A CAUSE OF REFRACTORY HYPERTENSION IN AN 18-YEAR-OLD. Murphy A, Yudi M, Calafiore P, Johns J, Matalanis G, Horrigan M. The international journal of cardiovascular imaging. Isolated interrupted aortic arch: a case report and review of the literature. Available from: Īkdemir R, Ozhan H, Erbilen E, Yazici M, Gündüz H, Uyan C. Congenital variants and anomalies of the aortic arch. Interrupted aortic arch: spectrum of MRI findings. (Written consent was taken from the parent of the child for publication)ĭillman JR, Yarram SG, D'Amico AR, Hernandez RJ. In conclusion, CT or MR angiography plays an important role in diagnosis and guiding proper surgical management in cases of Interrupted Aortic Arch. It can be done in one stage or multistage sugery to repair the interruption and related cardiac septal defect. However, it can present late as in our case due to some unique collaterals which maintain the flow to the descending aorta or presenting with refractory hypertension in adolescent The main treatment for IAA is maintaining the flow through PDA with prostaglandin and planning the reconstruction of aortic continuity to enable appropriate blood flow. IAA is related to mortality rate of more than 90% if left untreated after 1 year of age. MR angiography is especially advantageous in children due to abscence of radiation but one major disadvantage is its long time for acquisition compared to CT angiography. Diagnostic assessment by CT or MRI can demonstrate the morphological features and potential complex associated findings because of its multiplanar capabilities. Therefore, CT or MR angiography plays an important diagnostic role. However, it has its limitations as it depends on the observer capabilities and to define the exact site of interruption might be challenging. Echocardiography plays the first and important role in screening diagnosis of aortic arch anomalies which is free of radiation, particularly advantageous for paediatric population. Prior to the surgical planning, imaging plays a crucial role to know the exact types of IAA and associated cardiac anomalies. About 50% of IAA is associated with a chromosome 22q11.2 deletion (associated with DiGeorge syndrome) which is more particularly with type B. The most common anomaly is patent ductus arteriosus (PDA) which accounts for about 97% of the cases. Interrupted aortic arch is associated with intracardiac anomalies like ventricular septal defect (VSD) and patent ductus arteriosus (PDA). Type A is the second most common anomaly and is thought to result from abnormal regression of the left fourth arch segment after the left subclavian artery has ascended to its normal position. Subtype3: Isolated subcalvian artery from ductus arteriosus According to Celoria-Patton classification, IAA classified as ġ.Type A: Second most common distal to the subcalvian arterial originĢ.Type B: Most common (>50%), between the left CCA origin and subclavian arterial origin.ģ.Type C: Rare.Interruption occurs proximal to left CCA origin However, significantly decreased flow during the embryological process within the aortic arch might play an important role. It can either be complete or connected by a fibrous band. Interrupted Aortic Arch (IAA) is an uncommon congenital vascular anomaly (2-3 per million live births) where there is a separation between the ascending and descending aorta.
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