Ann Thorac Surg 2007;84:1016-1019
© 2007 The Society of Thoracic Surgeons
Case Reports
Surgical Treatment of Persistent Fifth Aortic Arch Associated with Interrupted Aortic Arch
Yong-Hong Zhao, MDa,*,
Zhao-Kang Su, MDb,
Jin-Feng Liu, MDb,
Ding-Fang Cao, MDb,
Wen-Xiang Ding, MDb
a Department of Thoracic Cardiovascular Surgery, The Shanghai Sixth People’s Hospital Affiliated to Shanghai Jiao Tong University, Shanghai, China
b Department of Thoracic and Cardiovascular Surgery, Shanghai Children’s Medical Center, Medical College of Shanghai Jiao Tong University, Shanghai, China
Accepted for publication April 2, 2007.
* Address correspondence to Dr Zhao, Department of Thoracic Cardiovascular Surgery, The Shanghai Sixth People’s Hospital Affiliated to Shanghai Jiao Tong University, 600 Yishan Road, Shanghai, 200233, China (Email: zyh5211987020{at}163.com).
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Abstract
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This study describes two cases of the rare congenital anomaly, persistent fifth aortic arch. Both cases involve boys (1 at 9 years of age and another at 7 months of age). To detect persistent fifth aortic arch with interrupted aortic arch, the following methods were used: echocardiogram, angiocardiography, and magnetic resonance imaging. In both cases the blood pressure between the upper and lower limbs differed. To relieve the obstruction of blood flow, each case was surgically repaired using patching or conduit interposition. Postoperative courses were uneventful. Two-year to 5-year follow-up examinations were positive showing that the anastomosis was unobstructed and the velocity of blood flow to the descending aorta was normal.
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Introduction
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Persistent fifth aortic arch (PFAA) is an extremely rare congenital cardiovascular malformation with sporadic cases reported [1–10]. It may occur in isolation or in association with various anomalies such as patent arterial duct, interrupted aortic arch (IAA), and pulmonary atresia with tetralogy of Fallot. Embryonic observations show this is an abnormal arterial branch originating from the distal ascending aorta and opposite to the ostium of the innominate artery [11]. We report here two cases of this congenital anomaly. Surgical treatments were successful and the patients were found to be stable as suggested by longitudinal follow-up studies.
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Case Reports
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Patient 1
Patient 1 was a 9-year-old boy who was diagnosed with PFAA during a routine physical examination when high-system artery pressure was detected. He was further examined in our hospital during which time a grade 3/6 pansystolic murmur was noted along the left sternal border at the third intercostal space. Femoral artery pulses were found slightly palpable. Blood pressure was 160/90 mm Hg on the right arm, 165/90 mm Hg on the left arm, 90/75 mm Hg on the right leg, and 90/76 mm Hg on the left leg. An echocardiogram revealed left PFAA with IAA; the interruption occurred at the level of the aorta distal to the left subclavian artery. Angiocardiography and magnetic resonance imaging were also performed later (Figs 1, 2).
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Fig 1. Aortogram showing interruption of the aortic arch in patient 1. Note that the fifth aortic arch connects the ascending aorta (AAO) with the descending aorta (DAO). (PFAA = persistent fifth aortic arch.)
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Fig 2. Magnetic resonance image demonstrating the repaired fifth aortic arch in patient 1. (AAO = ascending aorta; DAO = descending aorta; PFAA = persistent fifth aortic arch.)
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The corrective procedure conducted was a median sternotomy supported by normothermia cardiopulmonary bypass. The first arterial cannula was inserted into the ascending aorta. The second arterial cannula was connected to the arterial tubing by a "Y" adapter and was inserted into the descending aorta. Venous return cannulas were inserted into the right atrium. The PFAA diameter was only 0.8 cm. After the first bypass, two curved, side-biting clamps were placed at the two joints of the fifth aortic arch, one connected to the ascending aorta and the other to the descending aorta. A longitudinal incision was made on the surface of the fifth aortic arch, and a Gore-Tex patch (W. L. Gore and Assoc, Flagstaff, AZ) with a running suture of 5-0 Prolene (Ethicon, Somerville, NJ) was used to enlarge the fifth aortic arch. When the bypass was discontinued, blood pressure measured 90/50 mm Hg on the left arm and 90/45 mm Hg on the left leg. Postoperatively the patient remained calm and his blood pressure was stabilized with sedative and sodium nitroprusside. This patient was discharged 13 days after the operation without complications. Several follow-ups examination during the following 5 years revealed no further symptoms of the patients. Magnetic resonance imaging was done at the time of the follow-up study, illustrating that the enlarged fifth aortic arch is smooth and unobstructed (Fig 3).
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Fig 3. Aortogram showing interruption of the aortic arch in patient 1. Note that the fifth aortic arch connects the ascending aorta (AAO) with the descending aorta (DAO). (PFAA = persistent fifth aortic arch.)
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Patient 2
Patient 2 was a 7-month-old boy who had a diagnosis similar to the first patient. Initially the corrective measure performed was a left thoracotomy to repair the aortic arch using a Gore-Tex conduit (W. L. Gore & Assoc) without cardiopulmonary bypass. However, mobilizing the ascending aorta proved very difficult; thus a median sternotomy was conducted to improve exposure of the aorta. During these procedures we noticed that the fifth aortic arch was only 0.4-cm wide; thus we inserted an 8-mm Gore-Tex conduit (W. L. Gore & Assoc) between the ascending and descending aortas. The postoperative course was uneventful. The blood pressure of the lower extremities rose to 90/50 mm Hg, which was similar to the pressure of the upper extremities. This patient was discharged 9 days postoperatively without complaints. During a postoperative observation 2 years later, an echocardiogram displayed that the anastomosis was unobstructed.
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Comment
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These case reports describe 2 male patients diagnosed with the rare congenital anomaly, PFAA. Interestingly these patients have an even more atypical variation of PFAA involving IAA. In a review of PFAA literature by Lambert and colleagues [4], 26 cases associated with miscellaneous heart defects from patent ductus arteriosus to complex cardiopathy were detailed. Twenty (76%) of these cases had systemic-to-systemic connections by the fifth arch. Only five cases (19%) were associated with IAA. In our hospital, only two cases were detected, but this number may be underestimated because PFAA cases can be difficult to detect.
Persistent fifth aortic arch was first described by Van Praagh [11] in 1969. The PFAA can be either right-sided or left-sided and is classified into 3 types (see Fig 4). Type A is characterized by a double-lumen aortic arch with both lumina patent. Anatomically the type A aortic arch is subdivided into superior and inferior channels. The superior channel gives rise to the brachiocephalic arteries and is considered to be composed distally of the left fourth aortic arch. The inferior channel originates proximally, opposite the ostium of the innominate artery and rejoins the superior channel distally, proximal to the ligamentum arteriosum. This inferior channel represents a left PFAA. Type B consists of atresia, or interruption of the superior arch, with a patent inferior arch. The atresia is localized to the superior channel representing interruption of the fourth arch, and the inferior channel is considered to be a left PFAA. Finally, type C is described as a systemic-to-pulmonary arterial connection arising proximal to the first brachiocephalic artery [5, 12]. In this type, PFAA is associated with markedly reduced pulmonary blood flow secondary to severe right-sided outflow tract stenosis or atresia. The coexistence of right ventricular outflow tract obstruction and PFAA may be developmentally significant. Type C has often been described in association with pulmonary atresia and ventricular septal defect [9], the anomalous systemic-to-pulmonary connection was originally misdiagnosed as being a patent arterial duct on the contralateral side to the definitive or descending aorta. However, an arterial duct on that side must always be connected to the subclavian artery and PFAA arises from the ascending aorta and connects to the pulmonary artery through the embryonic sixth arch.
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Fig 4. Diagrams of the types of persistent fifth aortic arch with their corresponding arch patterns. (A) Double-lumen aortic arch with both lumina patent. (B) Atresia or interruption of the superior arch with patent inferior arch. (C) Systemic-to-pulmonary arterial connection atresia and absent arterial duct.
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Both cases detailed in this report can be classified as type B PFAA concerning a systemic-to-systemic connection that is usually hemodynamically beneficial to the associated anomalies, such as coarctation or IAA [8]. It is interesting to note that the potential hemodynamic consequences of a PFAA seem to be favorable. As some cases have been associated with coarctation or IAA, the existence of an alternative systemic arch in the systemic-to-systemic connection would be advantageous. For example, in PFAA with a pulmonary connection, all reported cases have been associated with pulmonary atresia. In this situation, the fifth arch acts as a vital systemic-to-pulmonary shunt in the absence of an arterial duct.
Surgical intervention is necessary to repair PFAA with IAA to maintain normal physiologic function. Two techniques have been used in our cases: (1) patching aortoplasty and (2) interposition of Gore-Tex tube grafts. The median sternotomy was an optional alternative that greatly improved the surgical procedures by further exposing the operative field. These procedures produced ideal results without any complications. The PFAA associated with IAA can be detected with echocardiography and angiography. However, magnetic resonance imaging is more useful for confirming this rare vascular malformation.
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Acknowledgments
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We would like to thank Dr Zaw-Sing Su, PhD, a retired scientist from Stanford Research Institute (SRI International) of Menlo Park, CA, for his careful reading and review of the manuscript.
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References
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- Lee MH, Chiu CC, Dai ZK, et al. Type III persistent fifth aortic arch in association with transposition of great arteries and pulmonary atresia—an unreported case Int J Cardiol 2007;18:e114-e115.
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- Lambert V, Blaysat G, Sidi D, et al. Double-lumen aortic arch: a new case associated with coarctation Pediatr Cardiol 1999;20:167-169.[Medline]
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- Donti A, Soavi N, Sabbatani P, et al. Persistent left fifth aortic arch associated with tetralogy of fallot Pediatr Cardiol 1997;18:229-231.[Medline]
- Lim C, Kim WH, Kim SC, et al. Truncus arteriosus with coarctation of persistent fifth aortic arch Ann Thorac Surg 2003;74:1702-1704.
- Van Praagh R, Van Praagh S. Persistent fifth arterial arch in man: congenital double lumen aortic arch Am J Cardiol 1969;24:279-282.[Medline]
- Weinberg PM. Aortic arch anomaliesIn: Emmanouilides GC, Riemenschneider TA, Allen HD, et al. editors. Heart disease in infants, children, and adolescent. 5th ed.. Baltimore, MD: Williams & Wilkins; 1995. pp. 810-837.
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Abstract
Introduction
