© 2014 Wiley Periodicals, Inc.
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CASE REPORT ______________________________________________________________________
Persistent Fifth Aortic Arch with Interrupted Aortic Arch Xiujie Tang, M.D., Lianyi Wang, M.D., Qingyu Wu, M.D., and Xiaoning Tong, M.D. First Hospital of Tsinghua University, Beijing, China ABSTRACT Persistent fifth aortic arch (PFAA) is one of the rarest congenital anomalies. We report a seven-month-old male with PFAA with upper arch interruption and lower arch coarctation diagnosed by echocardiogram and multidetector computed tomography. doi: 10.1111/jocs.12450 (J Card Surg
2015;30:284–287)
Persistent fifth aortic arch (PFAA) is a rare congenital cardiovascular malformation with few cases reported. We report a case of PFAA with upper arch interruption and lower arch coarctation.
PATIENT PROFILE Institutional review board permission was obtained to report the case. This patient was a seven-month-old male and was diagnosed with coarctation of aorta when he was two months old. He suffered from repeated bouts of pneumonia. Physical examination showed a grade 3/6 systolic murmur along the left sternal border at the third intercostal space. Blood pressure was 132/78 mmHg on the right arm, 130/ 75 mmHg on the left arm, 97/67 mmHg on the right leg, and 99/63 mmHg on the left leg. Echocardiogram revealed left ventricular enlargement and severe mitral valve regurgitation, with intact interatrial and interventricular septum. Left ventricular systolic function was decreased with left ventricular ejection fraction (LVEF) of 35%. The ascending aorta separated into two arteries at the level of the aortic arch. Interruption of the true upper arch occurred distal to the branch of the left subclavian artery (Fig. 1). The lower arch connected to the descending aorta with severe stenosis, and was identified as PFAA (Figs. 1 and 2); color Doppler flow image (CDFI) showed reduced and
Conflict of interest: The authors acknowledge no conflict of interest in the submission. Address for correspondence: Lianyi Wang, M.D., Heart Center, First Hospital of Tsinghua University, No. 6, First Street of Jiuxianqiao, Beijing 100016, China. Fax: þ86-10-64374907; e-mail: [email protected]
turbulent flow signals. Multidetector computed tomography was also performed (Fig. 3), showing moderate stenosis of the left primary bronchi, and that the ascending aorta separated into two arteries at the level of the aortic arch. One artery went upward, and interrupted after deriving three branches of the brachiocephalic artery, the other connected to the descending aorta with severe stenosis, with a minimum diameter of 1.3 mm. A sternotomy was performed and hypothermic cardiopulmonary bypass was instituted. The aorta was cannulated between the origin of PFAA and the innominate artery. There was a 10 mm long coarctation segment of the PFAA, with a minimum diameter of 1mm. A large amount of mitral regurgitation was noticed with a dilated mitral annulus. During the operation, the PFAA and the coarctation were resected. After resecting the PFAA, the hole on the lateral wall of the ascending aorta was extended downward to fit the size of the descending aorta. Then a side-to-end anastomosis of the ascending aorta and the descending aorta was performed to repair the anomaly (Fig. 4). Mitral valve annuloplasty was also performed to reduce the regurgitation by adding two 4-0 Prolene pledgeted mattress sutures at the anterolateral and posteromedial commissures, respectively. The postoperative course was uneventful. The echocardiogram performed after surgery showed that the anastomosis was unobstructed and the velocity of blood flow to the descending aorta was normal. The mitral valve regurgitation was mild and much improved compared to the preoperative study. LV systolic function also increased with an EF of 55% (Fig. 5). A nine-month postoperative follow-up echocardiogram showed normal size of the cardiac chambers and normal systolic function with an LVEF of 75%. The maximum pressure gradient across the aortic arch was 25 mmHg. No significant blood pressure discrepancy was identified between the upper extremities and the lower extremities. The patient was doing well. DISCUSSION PFAA is an extremely rare congenital cardiovascular malformation with very few reported cases. It was first reported by van Praagh and van Praagh1 in 1969 as a sizable subway beneath the fourth arch, forming a congenital double-lumen aortic arch. According to Weinberg,2 the PFAA can be classified into three types (Fig. 6). Type A is characterized by a double-lumen aortic arch with both lumen patent. Type B consists of atresia, or interruption of the superior arch, with a patent inferior arch. Type C is described as a systemic-to-pulmonary arterial connection arising proximal to the first brachiocephalic artery. Our case belongs to type B with concomitant inferior arch coarctation. Most of the previously reported cases were associated with major congenital heart disease such as tetralogy of Fallot,3
J CARD SURG 2015;30:284–287
Figure 1. Suprasternal fossa section showing interruption of the upper arch and lower arch (PFAA) with coarctation and connected with DAO. AAO, ascending aorta; DAO, descending aorta; PFAA, persistent fifth aortic arch.
Figure 2. Suprasternal fossa section with color Doppler. AAO, ascending aorta; DAO, descending aorta; PFAA, persistent fifth aortic arch.
Figure 3. 3D reconstruction image of computed tomography. AAO, ascending aorta; DAO, descending aorta; PFAA, persistent fifth aortic arch.
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truncus arteriosus,4 coarctation of aorta,5 and anomalous origin of left pulmonary artery from the ascending aorta.6 In the course of the embryologic development, there are six aortic arches. Not all six, though, are present at the same time, In normal embryology, the fifth arch finally disappears bilaterally. The third arch becomes the common carotid and the proximal internal carotid, the right fourth becomes the proximal part of the right subclavian, and the left fourth becomes the aortic arch proper, and the sixth arches give rise to the pulmonary arteries and ductus. Aortic arch anomalies are associated with abnormal embryologic development. Embryologically, endothelial sprouts arise from the aortic sac and the descending aorta corresponding to the upper and lower end of the fifth arch. This fifth arch is inconstant, being found in only 50% of the human embryos at the time of establishment of the six (pulmonary) arches, from the fourth week of development. Observations show that this is an abnormal arterial branch originating from the distal ascending aorta and opposite to the ostium of the innominate artery7 (Fig. 7). Lambert and colleagues5 reported that only 19% PFAA were associated with IAA. In patients with type A PFAA, it is a systemic-to-systemic connection. If there is no intracardiac shunt or defect associated, no operation is needed and the patient can be asymptomatic. Patients with type B and type C PFAA usually require surgery. Surgical intervention is necessary to repair PFAA with IAA to maintain normal physiologic function. The PFAA associated with IAA can be detected with echocardiography and multidetector computed tomography. Accurate diagnosis of this rare vascular malformation is the key to ensure a good prognosis when operative management is required. In this case, since there is no intracardiac shunt, the key issue affecting the outcome is the repair of the coarctation and the connection of the descending aorta without a residual gradient. If the PFAA is wide enough, it can be anastomosed directly to the descending aorta. From the computed tomography (CT) scan we can see that it is only half size of the ascending aorta; we choose to resect it and anastomose the descending aorta to the wall of the ascending aorta where the PFAA was resected. To restore the connection of the ascending aorta and the descending aorta, direct anastomosis can be used in infants, while a tube graft can be used in older patients. Extensive mobilization of the PFAA, the ductal tissue, and the descending aorta is important in avoiding tension on the suture line. Other important issues to avoid recoarctation include: resection of all ductal tissue, an anastomosis that relieves tubular hypoplasia of the arch, and use of autologous tissue allowing for growth if tube graft connection is needed.
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Figure 4. (A) The persistent fifth aortic arch and the coarctation were resected. (B) The hole on the lateral wall of the ascending aorta was extended downward to fit the size of the descending aorta. (C) A side-to-end anastomosis of the ascending aorta and the descending aorta was performed.
Figure 5. Postoperative apex four-chamber view with color Doppler showing mild mitral regurgitation.
Figure 7. Persistence of left-sided fifth arch with normal arch arising from the fourth branchial arch and patent ductus arising from the distal sixth arch. PDA, patent ductus arteriosus.
Figure 6. 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.
J CARD SURG 2015;30:284–287
TANG, ET AL. PERSISTENT FIFTH AORTIC ARCH
REFERENCES 1. Van Praagh R, Van Praagh S: Persistent fifth arterial arch in man: Congenital double lumen aortic arch. Am J Cardiol 1969;24:279–282. 2. Weinberg PM: Aortic arch anomalies. In Moss and Adams’ Heart disease in Infants, Children, and Adolescents. 5th ed. Williams & Wilkins, Baltimore, 1995, pp. 810–837. 3. Donti A, Soavi N, Sabbatani P, et al: Persistent left fifth aortic arch associated with tetralogy of Fallot. Pediatr Cardiol 1997;18:229–231.
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4. Lim C, Kim WH, Kim SC, et al: Truncus arteriosus with coarctation of persistent fifth aortic arch. Ann Thorac Surg 2002;74:1702–1704. 5. Lambert V, Blaysat G, Sidi D, et al: Double-lumen aortic arch by persistence of fifth aortic arch: A new case associated with coarctation. Pediatr Cardiol 1999;20:167–169. 6. Wang JN, Wu JM, Yang YJ: Double-lumen aortic arch with anomalous left pulmonary artery origin from the main pulmonary artery: Bilateral persistent fifth aortic arch—A case report. Int J Cardiol 1999;69:105–108. 7. Dhanya W, Sejal S, Colin J: A rare association with patent ductus arteriosus. Ann Pediatr Cardiol 2012;5(2):191–193.
Copyright of Journal of Cardiac Surgery is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
284
CASE REPORT ______________________________________________________________________
Persistent Fifth Aortic Arch with Interrupted Aortic Arch Xiujie Tang, M.D., Lianyi Wang, M.D., Qingyu Wu, M.D., and Xiaoning Tong, M.D. First Hospital of Tsinghua University, Beijing, China ABSTRACT Persistent fifth aortic arch (PFAA) is one of the rarest congenital anomalies. We report a seven-month-old male with PFAA with upper arch interruption and lower arch coarctation diagnosed by echocardiogram and multidetector computed tomography. doi: 10.1111/jocs.12450 (J Card Surg
2015;30:284–287)
Persistent fifth aortic arch (PFAA) is a rare congenital cardiovascular malformation with few cases reported. We report a case of PFAA with upper arch interruption and lower arch coarctation.
PATIENT PROFILE Institutional review board permission was obtained to report the case. This patient was a seven-month-old male and was diagnosed with coarctation of aorta when he was two months old. He suffered from repeated bouts of pneumonia. Physical examination showed a grade 3/6 systolic murmur along the left sternal border at the third intercostal space. Blood pressure was 132/78 mmHg on the right arm, 130/ 75 mmHg on the left arm, 97/67 mmHg on the right leg, and 99/63 mmHg on the left leg. Echocardiogram revealed left ventricular enlargement and severe mitral valve regurgitation, with intact interatrial and interventricular septum. Left ventricular systolic function was decreased with left ventricular ejection fraction (LVEF) of 35%. The ascending aorta separated into two arteries at the level of the aortic arch. Interruption of the true upper arch occurred distal to the branch of the left subclavian artery (Fig. 1). The lower arch connected to the descending aorta with severe stenosis, and was identified as PFAA (Figs. 1 and 2); color Doppler flow image (CDFI) showed reduced and
Conflict of interest: The authors acknowledge no conflict of interest in the submission. Address for correspondence: Lianyi Wang, M.D., Heart Center, First Hospital of Tsinghua University, No. 6, First Street of Jiuxianqiao, Beijing 100016, China. Fax: þ86-10-64374907; e-mail: [email protected]
turbulent flow signals. Multidetector computed tomography was also performed (Fig. 3), showing moderate stenosis of the left primary bronchi, and that the ascending aorta separated into two arteries at the level of the aortic arch. One artery went upward, and interrupted after deriving three branches of the brachiocephalic artery, the other connected to the descending aorta with severe stenosis, with a minimum diameter of 1.3 mm. A sternotomy was performed and hypothermic cardiopulmonary bypass was instituted. The aorta was cannulated between the origin of PFAA and the innominate artery. There was a 10 mm long coarctation segment of the PFAA, with a minimum diameter of 1mm. A large amount of mitral regurgitation was noticed with a dilated mitral annulus. During the operation, the PFAA and the coarctation were resected. After resecting the PFAA, the hole on the lateral wall of the ascending aorta was extended downward to fit the size of the descending aorta. Then a side-to-end anastomosis of the ascending aorta and the descending aorta was performed to repair the anomaly (Fig. 4). Mitral valve annuloplasty was also performed to reduce the regurgitation by adding two 4-0 Prolene pledgeted mattress sutures at the anterolateral and posteromedial commissures, respectively. The postoperative course was uneventful. The echocardiogram performed after surgery showed that the anastomosis was unobstructed and the velocity of blood flow to the descending aorta was normal. The mitral valve regurgitation was mild and much improved compared to the preoperative study. LV systolic function also increased with an EF of 55% (Fig. 5). A nine-month postoperative follow-up echocardiogram showed normal size of the cardiac chambers and normal systolic function with an LVEF of 75%. The maximum pressure gradient across the aortic arch was 25 mmHg. No significant blood pressure discrepancy was identified between the upper extremities and the lower extremities. The patient was doing well. DISCUSSION PFAA is an extremely rare congenital cardiovascular malformation with very few reported cases. It was first reported by van Praagh and van Praagh1 in 1969 as a sizable subway beneath the fourth arch, forming a congenital double-lumen aortic arch. According to Weinberg,2 the PFAA can be classified into three types (Fig. 6). Type A is characterized by a double-lumen aortic arch with both lumen patent. Type B consists of atresia, or interruption of the superior arch, with a patent inferior arch. Type C is described as a systemic-to-pulmonary arterial connection arising proximal to the first brachiocephalic artery. Our case belongs to type B with concomitant inferior arch coarctation. Most of the previously reported cases were associated with major congenital heart disease such as tetralogy of Fallot,3
J CARD SURG 2015;30:284–287
Figure 1. Suprasternal fossa section showing interruption of the upper arch and lower arch (PFAA) with coarctation and connected with DAO. AAO, ascending aorta; DAO, descending aorta; PFAA, persistent fifth aortic arch.
Figure 2. Suprasternal fossa section with color Doppler. AAO, ascending aorta; DAO, descending aorta; PFAA, persistent fifth aortic arch.
Figure 3. 3D reconstruction image of computed tomography. AAO, ascending aorta; DAO, descending aorta; PFAA, persistent fifth aortic arch.
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truncus arteriosus,4 coarctation of aorta,5 and anomalous origin of left pulmonary artery from the ascending aorta.6 In the course of the embryologic development, there are six aortic arches. Not all six, though, are present at the same time, In normal embryology, the fifth arch finally disappears bilaterally. The third arch becomes the common carotid and the proximal internal carotid, the right fourth becomes the proximal part of the right subclavian, and the left fourth becomes the aortic arch proper, and the sixth arches give rise to the pulmonary arteries and ductus. Aortic arch anomalies are associated with abnormal embryologic development. Embryologically, endothelial sprouts arise from the aortic sac and the descending aorta corresponding to the upper and lower end of the fifth arch. This fifth arch is inconstant, being found in only 50% of the human embryos at the time of establishment of the six (pulmonary) arches, from the fourth week of development. Observations show that this is an abnormal arterial branch originating from the distal ascending aorta and opposite to the ostium of the innominate artery7 (Fig. 7). Lambert and colleagues5 reported that only 19% PFAA were associated with IAA. In patients with type A PFAA, it is a systemic-to-systemic connection. If there is no intracardiac shunt or defect associated, no operation is needed and the patient can be asymptomatic. Patients with type B and type C PFAA usually require surgery. Surgical intervention is necessary to repair PFAA with IAA to maintain normal physiologic function. The PFAA associated with IAA can be detected with echocardiography and multidetector computed tomography. Accurate diagnosis of this rare vascular malformation is the key to ensure a good prognosis when operative management is required. In this case, since there is no intracardiac shunt, the key issue affecting the outcome is the repair of the coarctation and the connection of the descending aorta without a residual gradient. If the PFAA is wide enough, it can be anastomosed directly to the descending aorta. From the computed tomography (CT) scan we can see that it is only half size of the ascending aorta; we choose to resect it and anastomose the descending aorta to the wall of the ascending aorta where the PFAA was resected. To restore the connection of the ascending aorta and the descending aorta, direct anastomosis can be used in infants, while a tube graft can be used in older patients. Extensive mobilization of the PFAA, the ductal tissue, and the descending aorta is important in avoiding tension on the suture line. Other important issues to avoid recoarctation include: resection of all ductal tissue, an anastomosis that relieves tubular hypoplasia of the arch, and use of autologous tissue allowing for growth if tube graft connection is needed.
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J CARD SURG 2015;30:284–287
Figure 4. (A) The persistent fifth aortic arch and the coarctation were resected. (B) The hole on the lateral wall of the ascending aorta was extended downward to fit the size of the descending aorta. (C) A side-to-end anastomosis of the ascending aorta and the descending aorta was performed.
Figure 5. Postoperative apex four-chamber view with color Doppler showing mild mitral regurgitation.
Figure 7. Persistence of left-sided fifth arch with normal arch arising from the fourth branchial arch and patent ductus arising from the distal sixth arch. PDA, patent ductus arteriosus.
Figure 6. 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.
J CARD SURG 2015;30:284–287
TANG, ET AL. PERSISTENT FIFTH AORTIC ARCH
REFERENCES 1. Van Praagh R, Van Praagh S: Persistent fifth arterial arch in man: Congenital double lumen aortic arch. Am J Cardiol 1969;24:279–282. 2. Weinberg PM: Aortic arch anomalies. In Moss and Adams’ Heart disease in Infants, Children, and Adolescents. 5th ed. Williams & Wilkins, Baltimore, 1995, pp. 810–837. 3. Donti A, Soavi N, Sabbatani P, et al: Persistent left fifth aortic arch associated with tetralogy of Fallot. Pediatr Cardiol 1997;18:229–231.
287
4. Lim C, Kim WH, Kim SC, et al: Truncus arteriosus with coarctation of persistent fifth aortic arch. Ann Thorac Surg 2002;74:1702–1704. 5. Lambert V, Blaysat G, Sidi D, et al: Double-lumen aortic arch by persistence of fifth aortic arch: A new case associated with coarctation. Pediatr Cardiol 1999;20:167–169. 6. Wang JN, Wu JM, Yang YJ: Double-lumen aortic arch with anomalous left pulmonary artery origin from the main pulmonary artery: Bilateral persistent fifth aortic arch—A case report. Int J Cardiol 1999;69:105–108. 7. Dhanya W, Sejal S, Colin J: A rare association with patent ductus arteriosus. Ann Pediatr Cardiol 2012;5(2):191–193.
Copyright of Journal of Cardiac Surgery is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
