Arch variants that form a vascular ring, such as double aortic arch, can result in respiratory distress due to tracheal compression. Arch anomalies can be associated with symptoms, such as dysphagia lusoria in the setting of left aortic arch with aberrant right subclavian artery. Aortic arch variants and anomalies will be reviewed in the context of a theoretical double aortic arch system. The purpose of this article is to review cross-sectional imaging techniques used in the evaluation of the aortic arch, describe the embryology and anatomy of the aortic arch system, discuss aortic arch variants and anomalies, and review other malformations of the aortic arch, including interrupted aortic arch, hypoplastic aortic arch, and aortic coarctation. Because aortic arch and associated cardiac anomalies represent a neonatal surgical emergency, our therapeutic plan consists of treatment for biventricular failure and PGE(1) infusion to maintain ductal patency, and surgical correction as soon as feasible.Ĭongenital variants and anomalies of the aortic arch are important to recognize as they may be associated with vascular rings, congenital heart disease, and chromosomal abnormalities, and can have important implications for prognosis and management. The three basic techniques were (1) direct aortic to aortic anastomosis bridging the gap, (2) "turndown" or "turnup" of one of the arch vessels to the aorta across the gap, or (3) bypass of the interruption with graft material. A variety of individualized techniques were used to repair the defects. There were 12 Type A (30%), 26 Type B (67%) and one Type C (3%). Two of these (4.6%) had no associated intracardiac lesions, and both survived bypass grafting without complications. Since the first patient with this congenital anomaly was treated at our institution in 1965, 39 patients have undergone surgical treatment. Type C, which is the most uncommon type, is interrupted between the innominate and left common carotid arteries. Type B, which is the most common form, is interrupted between the left common carotid and the left subclavian arteries. There are three types of IAA: Type A is interrupted distal to the left subclavian artery. Because the median age at death in untreated cases is 10 days, this condition usually occurs as a complicated neonatal surgical emergency. More than 97% of the cases also have associated cardiac anomalies complicating their treatment. Interrupted aortic arch (IAA) is an uncommon congenital anomaly representing approximately 1% of congenital heart disease. The treatment method should be chosen based on the patient's clinical symptoms rather than the severity of the lesion. While the CoA patient underwent stent implantation because of uncontrollable hypertension, the blood flow recovered smoothly and the blood pressures at both ends of the stenosis returned to normal after surgery.Īdult type A IAA and CoA have difference and similarity, and type A IAA is associated with CoA to a certain extent. ![]() The IAA patient refused surgical treatment and blood pressure could be controlled with drugs. Computed tomography angiography showed that the aortic arch and descending aorta were discontinuous or significantly narrowed with extensive collateral flow. The two cases of patients presented with hypertension for many years. This paper reports two cases (interrupted aortic arch and severe aortic coarctation) to analyze the difference and similarity between them. Adult type A IAA is considered as an extreme form of CoA, which is complete discontinuity of aortic function and lumen caused by degenerative arterial coarctation. Although the main difference between them is whether arterial lumen exhibits continuity or not, the clinical manifestations are similar and connection exists between them. Aortic coarctation (CoA) is usually confused with interrupted aortic arch (IAA), especially adult type A interrupted aortic arch, due to their similar anatomical location.
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