Pediatric Radiology
Pediatr. Radiol. 8, 4 8 - 5 0 (1979)
9 by Springer-Verlag 1979
Right Aortic Arch with Aberrant Left Innominate Artery I. J. Garti and M. M. Aygen D e p a r t m e n t of Radiology and Toor Heart Institute, Beilinson Medical Center, Petah-Tiqva, and Tel-Aviv University, Sackler School of Medicine, Israel
Abstract. A 9-year-old girl with type D right aortic arch anomaly, has been presented. The diagnosis was based upon the presence of signs and symptoms of a vascular ring and the early visualization of a retroesophageal aberrant left innominate artery, by means of countercurrent right brachial angiography. Divison of the ligamentum arteriosum at operation relieved the symptoms. Key words: Vascular ring - Aortic arch anomalies Aortic diverticulum - Tracheoesophageal compression
Right aortic arch (RAA) occurs when a segment of the left arch of the primitive double aortic arch (Fig. 1 a) undergoes complete absorption at an early stage of embryologic development [1]. The interruption of the left arch may occur in 4 different zones, resulting in type A, B, C or D R A A anomalies [2]. Type D R A A occurs when the interruption is in the anterior part of the left arch proximal to the origin of the common carotid artery (Fig. 1 b). The left common carotid and subclavian arteries together with the distal portion of the left arch are transformed into a left innominate artery which is then the last branch of the right aortic arch and crosses from right to left behind the esophagus (Fig. 1 c). This anomaly
C
a
b
Fig. 1. a D i a g r a m showing the development of type D right aortic arch from the hypothetical double aortic arch of Edwards. b The zone D of the left arch is interrupted, c The interrupted arch is transformed to an aberrant left innominate artery. A A - ascending aorta, D A descending aorta, L S A - left subclavian artery, L C C A - left c o m m o n carotid artery, Ida - left ductus arteriosus
0301-0449/79/0008/0048/$01.00
I. J. Garti and M. M. Aygen: Type D Right Aortic Arch Anomaly
49 innominate artery was found. The ligamentum arteriosum was divided in order to interrupt the vascular ring.
Discussion
Fig. 2. Following countercurrent right brachial artery injection, the right subclavian artery, the distal parts of the two arches and a right sided descending aorta were visualized. The right common carotid artery is faintly filled from the right aortic arch (small arrow). From the distal part of the left arch an aberrant left innominate artery is filled (large arrow)
produces a vascular-ring and causes signs of tracheoesophageal compression. The vascular ring is composed of a right arch on the right side, a left innominate artery at the back, and the pulmonary artery in the front. The ligamentum arteriosum completes the ring on the left side by connecting the left innominate artery to the left pulmonary artery. We were able to find only two cases with angiographic description of this anomaly in the literature [3, 4].
Case Report A 9-year-old girl suffered from recurrent respiratory infections and episodes of dysphagia. Clinical studies did not reveal any evidence of heart disease. The barium filled esophagus showed a right-sided impression in the frontal plane and a deep posterior indentation in the lateral plane. Countercurrent right brachial angiography showed immediate filling of the distal parts of the two arches from the right subclavian artery. In the subsequent films the left innominate and right common carotid arteries were filled from their respective arches in that order. The descending aorta was right sided (Fig. 2). At operation R A A and retro-esophageal left
Type D R A A anomaly is rare. We have not been able to find in the literature any case in which the diagnosis has been documented at operation. In this anomaly as in type A and B RAA, the main portion of the left arch is transformed into a left innominate artery. The location of this artery in relation to the other branches of the right arch varies according to the site of interruption of the left arch during an early stage of embryologic development. In type A the interruption of the left arch is distal to the site of insertion of the Iigamentum arteriosum and is commonly called a right aortic arch with mirror image branching. In type B the interruption of the left arch is proximal to the ligamentum arteriosum [2]. In both anomalies, the left innominate artery is the first branch and the right subclavian artery, the last branch of the RAA. Consequently during countercurrent right brachial angiography, the right subclavian artery is the first and the left innominate artery the last vessel to be visualized. In type D RAA, the left innominate artery is the last branch of the right aortic arch and during countercurrent brachial angiography it fills immediately after the right subclavian artery. All three types of right aortic arch anomalies discussed above produce a right-sided impression on the barium-filled esophagus in the frontal view, but only types B and D cause a posterior indentation in the lateral view. The posterior indentation seen in type D is due to the posterior part of the right and left aortic arches and aberrant left innominate artery. In type B the posterior indentation is caused by the diverticulum, which represents the remnant of the left arch distal to the zone of interruption. In both of these anomalies, the ligamentum arteriosum completes the vascular ring, which causes symptoms and signs of tracheo-esophageal compression. In type A R A A there is no vascular ring and signs of tracheo-esophageal compression are therefore not to be expected; furthermore, in 98% of the cases this anomaly is associated with a cyanotic congenital heart disease, usually Tetralogy of Fallot or persistent Truncus Arteriosus. Such an association has not been found in types B and D RAA. In conclusion, it is suggested that the presence of tracheo-esophageaI compression, demonstration of right and posterior indentation in the bariumfilled esophagus and early visualization of a retro-esophageal aberrant innominate artery by countercurrent right brachial angiography indicates the diagnosis of type D RAA.
50
References 1. Edwards, J. E.: Anomalies of derivatives of aortic arch system. Med. Clin. North Am. 32, 925 (1948) 2. Garti, I. J., Aygen, M. M., Vidne, B., Levy, M. J.: Right aortic arch with mirror-image branching causing vascular ring. A new classification of the right aortic arch patterns. Br. J. Radiol. 46, 115 (1973) 3. Grollman, J.H., Bedynek, J. L., Henderson, H. S., Hall, R. J.: Right aortic arch with an aberrant retroesophageal innominate artery: angiographic diagnosis. Radiology 90, 782 (1968)
1. J. Garti and M. M. Aygen: Type D Right Aortic Arch Anomaly 4. Watkins, E., Hering, A.C.: Compression of the trachea and esophagus by anomalous great vessels. Diagnosis and treatment, 1761-1961. Med. Clin. North Am. 41, 821 (1961) Date of final acceptance: February 24, 1978 I. J. Garti Department of Radiology Beilinson Medical Center Petah-Tiqva Israel
Pediatr. Radiol. 8, 4 8 - 5 0 (1979)
9 by Springer-Verlag 1979
Right Aortic Arch with Aberrant Left Innominate Artery I. J. Garti and M. M. Aygen D e p a r t m e n t of Radiology and Toor Heart Institute, Beilinson Medical Center, Petah-Tiqva, and Tel-Aviv University, Sackler School of Medicine, Israel
Abstract. A 9-year-old girl with type D right aortic arch anomaly, has been presented. The diagnosis was based upon the presence of signs and symptoms of a vascular ring and the early visualization of a retroesophageal aberrant left innominate artery, by means of countercurrent right brachial angiography. Divison of the ligamentum arteriosum at operation relieved the symptoms. Key words: Vascular ring - Aortic arch anomalies Aortic diverticulum - Tracheoesophageal compression
Right aortic arch (RAA) occurs when a segment of the left arch of the primitive double aortic arch (Fig. 1 a) undergoes complete absorption at an early stage of embryologic development [1]. The interruption of the left arch may occur in 4 different zones, resulting in type A, B, C or D R A A anomalies [2]. Type D R A A occurs when the interruption is in the anterior part of the left arch proximal to the origin of the common carotid artery (Fig. 1 b). The left common carotid and subclavian arteries together with the distal portion of the left arch are transformed into a left innominate artery which is then the last branch of the right aortic arch and crosses from right to left behind the esophagus (Fig. 1 c). This anomaly
C
a
b
Fig. 1. a D i a g r a m showing the development of type D right aortic arch from the hypothetical double aortic arch of Edwards. b The zone D of the left arch is interrupted, c The interrupted arch is transformed to an aberrant left innominate artery. A A - ascending aorta, D A descending aorta, L S A - left subclavian artery, L C C A - left c o m m o n carotid artery, Ida - left ductus arteriosus
0301-0449/79/0008/0048/$01.00
I. J. Garti and M. M. Aygen: Type D Right Aortic Arch Anomaly
49 innominate artery was found. The ligamentum arteriosum was divided in order to interrupt the vascular ring.
Discussion
Fig. 2. Following countercurrent right brachial artery injection, the right subclavian artery, the distal parts of the two arches and a right sided descending aorta were visualized. The right common carotid artery is faintly filled from the right aortic arch (small arrow). From the distal part of the left arch an aberrant left innominate artery is filled (large arrow)
produces a vascular-ring and causes signs of tracheoesophageal compression. The vascular ring is composed of a right arch on the right side, a left innominate artery at the back, and the pulmonary artery in the front. The ligamentum arteriosum completes the ring on the left side by connecting the left innominate artery to the left pulmonary artery. We were able to find only two cases with angiographic description of this anomaly in the literature [3, 4].
Case Report A 9-year-old girl suffered from recurrent respiratory infections and episodes of dysphagia. Clinical studies did not reveal any evidence of heart disease. The barium filled esophagus showed a right-sided impression in the frontal plane and a deep posterior indentation in the lateral plane. Countercurrent right brachial angiography showed immediate filling of the distal parts of the two arches from the right subclavian artery. In the subsequent films the left innominate and right common carotid arteries were filled from their respective arches in that order. The descending aorta was right sided (Fig. 2). At operation R A A and retro-esophageal left
Type D R A A anomaly is rare. We have not been able to find in the literature any case in which the diagnosis has been documented at operation. In this anomaly as in type A and B RAA, the main portion of the left arch is transformed into a left innominate artery. The location of this artery in relation to the other branches of the right arch varies according to the site of interruption of the left arch during an early stage of embryologic development. In type A the interruption of the left arch is distal to the site of insertion of the Iigamentum arteriosum and is commonly called a right aortic arch with mirror image branching. In type B the interruption of the left arch is proximal to the ligamentum arteriosum [2]. In both anomalies, the left innominate artery is the first branch and the right subclavian artery, the last branch of the RAA. Consequently during countercurrent right brachial angiography, the right subclavian artery is the first and the left innominate artery the last vessel to be visualized. In type D RAA, the left innominate artery is the last branch of the right aortic arch and during countercurrent brachial angiography it fills immediately after the right subclavian artery. All three types of right aortic arch anomalies discussed above produce a right-sided impression on the barium-filled esophagus in the frontal view, but only types B and D cause a posterior indentation in the lateral view. The posterior indentation seen in type D is due to the posterior part of the right and left aortic arches and aberrant left innominate artery. In type B the posterior indentation is caused by the diverticulum, which represents the remnant of the left arch distal to the zone of interruption. In both of these anomalies, the ligamentum arteriosum completes the vascular ring, which causes symptoms and signs of tracheo-esophageal compression. In type A R A A there is no vascular ring and signs of tracheo-esophageal compression are therefore not to be expected; furthermore, in 98% of the cases this anomaly is associated with a cyanotic congenital heart disease, usually Tetralogy of Fallot or persistent Truncus Arteriosus. Such an association has not been found in types B and D RAA. In conclusion, it is suggested that the presence of tracheo-esophageaI compression, demonstration of right and posterior indentation in the bariumfilled esophagus and early visualization of a retro-esophageal aberrant innominate artery by countercurrent right brachial angiography indicates the diagnosis of type D RAA.
50
References 1. Edwards, J. E.: Anomalies of derivatives of aortic arch system. Med. Clin. North Am. 32, 925 (1948) 2. Garti, I. J., Aygen, M. M., Vidne, B., Levy, M. J.: Right aortic arch with mirror-image branching causing vascular ring. A new classification of the right aortic arch patterns. Br. J. Radiol. 46, 115 (1973) 3. Grollman, J.H., Bedynek, J. L., Henderson, H. S., Hall, R. J.: Right aortic arch with an aberrant retroesophageal innominate artery: angiographic diagnosis. Radiology 90, 782 (1968)
1. J. Garti and M. M. Aygen: Type D Right Aortic Arch Anomaly 4. Watkins, E., Hering, A.C.: Compression of the trachea and esophagus by anomalous great vessels. Diagnosis and treatment, 1761-1961. Med. Clin. North Am. 41, 821 (1961) Date of final acceptance: February 24, 1978 I. J. Garti Department of Radiology Beilinson Medical Center Petah-Tiqva Israel
