Double Lumen Aortic Arch or Persistence of Fifth Aortic Arch?- Report of a Case with No Associated Cardiac Defects and Literature Review

Abstract

Different vascular abnormalities have been reported under the denomination of "persistence of the fifth aortic arch." Detailed studies on experimental embryology raised the discussion about the existence of the fifth aortic arch as an embryological structure, both in humans and mammals. In 1969 the Van Praaghs described the occurrence of double left aortic arch, denominating such anomaly as persistence of the fifth arch. We describe here a female patient showing the presence of an anomalous vessel in parallel with the aortic arch. The finding was occasional, during a preoperative evaluation for cholecystectomy.

Assessment of persistent fifth aortic arch by echocardiography and computed tomography angiography

“…The diagnoses of these three patients were ultimately correctly defined at the time of surgery. It is noteworthy that Weinberg type A PFAA may be misdiagnosed as double aortic arch (DAA), [ 21 ] Weinberg type B was usually misdiagnosed as IAA or CoA, and Weinberg type C may be diagnosed as APW. The evidence for PFAA diagnosis was the detection of an abnormal vascular connection on imaging.…”

Double-lumen Aortic Arch: Persistence of the Fifth Aortic Arch?

“…Although frequently associated with several cardiac defects, such as coarctation or interruption of the aorta, patent ductus arteriosus, tetralogy of Fallot, atrioventricular septal defect, truncus arteriosus, pulmonary and tricuspid atresia, this anomaly can also be found alone. 3,8 The clinical presentation of double-lumen aortic arch depends on the type of connection and presence of associated anomalies. Type I, as reported here, is the most common form, usually of no clinical significance.…”

“…Types II and III have positive hemodynamic consequences, by providing an alternative systemic arch in case of coarctation or aortic arch interruption (type II), and by acting as a systemic-pulmonary shunt when associated with pulmonary or tricuspid atresia (type III). 3,[6][7][8] In the present case, both arches ran on the same side of the trachea and, unlike the classical double aortic arch, which involves both trachea and esophagus, and does not result in a vascular ring. 6 Thus, it may correspond to a non-classical double arch possibly secondary to the presence of collaterals between the fourth and sixth artery arches, as described by Gupta et.al.…”

“…Differential diagnosis should be made with patent ductus arteriosus and aortopulmonary window, especially in type III. 1,3,6,7 Echocardiography allows the detection of two lumens of the aortic arch running in parallel and helps in the identification of coexisting anomalies. Computed tomography and magnetic resonance imaging help define the anatomy of the aortic arch and its variants, confirm the diagnosis of this anomaly and detect possible coexisting abnormalities such as pulmonary or cardiovascular diseases.…”

“…Double-lumen aortic arch is a rare, usually underdiagnosed congenital anomaly, possibly secondary to the persistence of the fifth embryonic aortic arch or the presence of dorsal collateral arteries that connect the fourth and sixth aortic arch. [1][2][3] The authors report a case of double-lumen aortic arch associated with coarctation of the aorta and ductus arteriosus in a child with congenital malformation characterized by presence of vertebral defects, imperforate anus, heart disease, tracheoesophageal fistula, renal anomalies and limb abnormalities, known as the VACTERL association, which was accurately diagnosed by echocardiography and confirmed by computed tomography.…”