Double Aortic Arch With Ascending Aortic Aneurysm and Aortic Valve Regurgitation Yiwu Liang, MD, Qiwen Zhou, MD, and Ziying Chen, MD Department of Cardiac Surgery, The Second Hospital of Hebei Medical University, Shijiazhuang, China, and Department of Cardiac Surgery, Beijing Anzhen Hospital of Capital Medical University, Beijing, China
Double aortic arch is a rare congenital great vessel anomaly. We report a 21-year-old man who was diagnosed with a double aortic arch combining with ascending aortic aneurysm and aortic valve regurgitation. Computerized tomographic angiography and echocardiogram confirmed the diagnosis before operation. The patient underwent aortic root replacement and artificial vessel construction. At 3 years’ follow-up, the patient continues to have a good surgical recovery, although long-term results need observing. A review of the literature shows no double aortic arch combination with ascending aortic aneurysm and aortic regurgitation has been reported so far. (Ann Thorac Surg 2014;97:e43–5) Ó 2014 by The Society of Thoracic Surgeons
newly reconstructed ascending aorta through another artificial vessel (Intergard conduit 12 mm, DataScope). Postoperative computer tomography scans were performed (Figs 3, 4).
Comment Double aortic arch is a rare congenital heart malformation. The first post-mortem report of DAA was in 1737 by Hommell [1]. It also belongs to one kind of congenital vascular rings of the aortic arch, which account for 1% of cases of congenital heart disease [2]. There are three types of DAA: DAA with a right dominant arch accounts for approximately 70% of surgical cases, those with a left dominant arch accounts for approximately 25%, and a balanced arch represents about 5%. Clinically, the major symptoms of DAA are caused by vascular compression of the airway, esophagus, or both, and whether there are presentations of associated anomalies. Respiratory and gastrointestinal problems such as expiratory wheeze, tachypnea, feeding difficulty, emesis, and choking are often present at early ages in patients, even in the neonate, whereas symptomatic vascular rings manifesting in adults are rare, and most reports emphasize dysphagia as the predominant symptom [3]. Our case is an asymptomatic patient.
D
ouble aortic arch (DAA) is the most common form of the vascular rings, a class of congenital anomalies of the aortic arch system, in which the trachea and esophagus are completely encircled by connected segments of the aortic arch and its branches. Its common defining feature is that both the left and right aortic arches are present. This is a case report of a DAA with balanced aortic arches, presenting without symptoms.
An asymptomatic 21-year-old man with a heart murmur who was thought to have congenital heart disease underwent computed tomographic angiography and echocardiography, which showed that the ascending aorta was divided into an almost balanced type of right and left aortic arches. Each arch gave origin to the common carotid and subclavian artery, and then joined together posterior toward the right side of the chest to form a right descending aorta (Figs 1, 2). The aortic root was aneurysmal with a dilatation of 57 mm in diameter and associated with severe aortic valve regurgitation. During the operation, the aortic root was replaced. The aortic valve was replaced by a mechanical valve (St. Jude A25; St. Jude Medical, St. Paul, MN), and the ascending aorta was replaced by an artificial vessel (Intergard conduit 28 mm, DataScope Corp, Redmond, WA) and reconnected with the right arch. The left arch was reconnected with the Accepted for publication Sept 30, 2013. Address correspondence to Dr Chen, Department of Cardiac Surgery, The Second Hospital of Hebei Medical University, Shijiazhuang 050000, China; e-mail: [email protected].
Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc
Fig 1. An anterior perspective preoperation image (three-dimensionally reconstructed computer tomography scan of the heart and great vessels) shows the ascending aorta divided into almost balanced right and left aortic arches. Left and right subclavian and carotid arteries arose from each corresponding side of arch. The aortic root is enlarged and aneurysmal, associated with severe aortic valve regurgitation. (AO ¼ aorta; LAA ¼ left aortic arch; LCC ¼ left common carotid artery; LSA ¼ left subclavian artery; PA ¼ pulmonary artery; RAA ¼ right aortic arch; RCC ¼ right common carotid artery; RSA ¼ right subclavian artery.) 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.09.111
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Fig 2. A posterior perspective preoperation image (three-dimensionally reconstructed computer tomography scan of heart and great vessels) shows that each arch gave origin to the common carotid and subclavian artery, and joined together posterior toward the right side of the chest to form a right descending aorta. (DA ¼ descending aorta; LAA ¼ left aortic arch; LCC ¼ left common carotid artery; LSA ¼ left subclavian artery; RAA ¼ right aortic arch; RCC ¼ right common carotid artery; RSA ¼ right subclavian artery.)
Double aortic arch is usually found as an isolated malformation. Associated cardiac anomalies occur between 7% and 17% [4, 5]. Ventricular septal defects rank first in those associated cardiac anomalies; other anomalies include atrial septal defects, patent ductus arteriosus, tetralogy of Fallot, and transposition of the great arteries. Most cases of vascular rings have a normal ascending aorta and good function of the aortic valve. Our literature search did not show any case of double aortic arch associated with ascending aortic aneurysm and severe aortic valve regurgitation. It is accidental for the nonsymptomatic patient to be diagnosed with such anomalies just because of a heart murmur. Although chest roentgenograph can provide useful clues for diagnosis, the most convenient and important diagnostic technique for patients with DAA is echocardiography; the diagnosis even can be obtained during the in utero period in major heart centers by echocardiography. It is also a useful and noninvasive method for identifying associated cardiac anomalies, although the major problem of echocardiography resides in its limited ability to image the atretic aortic arch and ligamentum arteriosum. Cardiac catheterization with angiography can provide a diagnosis of DAA, but it is neither routinely required nor clinically feasible for smaller patients with severe cardiopulmonary diseases. Instead, computerized tomographic and magnetic resonance imaging can be performed safely and accurately. They also can provide the arterial branching pattern, the locations of airway and esophageal
Ann Thorac Surg 2014;97:e43–5
Fig 3. An anterior perspective postoperation image (three-dimensionally reconstructed computer tomography scan of heart and great vessels) shows the aortic root was replaced. The aortic valve was replaced by a mechanical valve; the ascending aorta was replaced by an artificial conduit vessel; the newly reconstructed ascending aortic conduit was reconnected with the right arch. The left arch was reconnected with the newly reconstructed ascending aorta through another new artificial conduit vessel. The inner space of the vascular ring was then enlarged. (LAA ¼ left aortic arch; LCC ¼ left common carotid artery; LSA ¼ left subclavian artery; NCAV ¼ newly constructed artificial vessel; NRAA ¼ newly reconstructed ascending aorta; RAA ¼ right aortic arch; RCC ¼ right common carotid artery; RSA ¼ right subclavian artery.)
obstructions, and associated cardiac anatomy. Meanwhile, they both are of great value for operative planning. Surgical care is necessary in any patient with symptoms of airway and esophageal compression and associated cardiac anomalies. It has been a standard of care for more than 60 years. The first open surgical correction was performed by Gross in 1945 [6]. The main purpose of surgery is to eliminate compression and associated cardiac anomalies caused by a vascular ring. Surgical mortality of isolated DAA is very low, but in those patients who exhibit other cardiac anomalies, mortality depends on coexisting conditions [7]. Our patient was asymptomatic; his major problems were ascending aortic aneurysm and severe aortic valve regurgitation, not vascular ring compression. The aortic root (ascending aorta and aortic valve) was replaced (similar to a Bentall procedure). The new reconstructed ascending aorta was connected with the right arch. The left arch was then reconnected with the newly reconstructed ascending aorta through a relatively small constructed artificial vessel, after which the inner space of the vascular ring was enlarged to avoid a possible compression, which possibly would appear in the future. Although 3 years’ surgical follow-up shows
Ann Thorac Surg 2014;97:e43–5
CASE REPORT LIANG ET AL DOUBLE AORTIC ARCH
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excellent result with no complications, long-term followup is warranted. The authors thank Mr Sujeeth Harischandra Suvarna, Senior Consultant Cardiothoracic Surgeon of the KMC-Manipal Hospitals, India, and Mr Jazeel Nimrry, Senior Consultant Cardiothoracic Surgeon, from Sri Lanka, who provided professional advice and language assistance.
References
Fig 4. A posterior perspective postoperation image (three-dimensionally reconstructed computer tomography scan of heart and great vessels) shows reconstruction of the arch vessels. (DA ¼ descending aorta; LAA ¼ left aortic arch; LSA ¼ left subclavian artery; NCAV ¼ newly constructed artificial vessel; RAA ¼ right aortic arch; RCC ¼ right common carotid artery; RSA ¼ right subclavian artery.)
1. Hommell L. Observationes anatomicae de arcu aortae bifido du dueto thoracico duplica, et de carstidum atque subclaviarum. Hildomas 1737;21:161. 2. Nadas AS, Fyler DC. Pediatric cardiology. Philadelphia: WB Saunders; 1972, p. 749. 3. Kouchoukos NT, Blackstone EH, Doty DB, Hanley FL, Karp RB. Kirklin/Barratt-Boyes cardiac surgery. Third Edition. Philadelphia, PA: Churchill Livingstone; 2003:1420. 4. Backer CL, Mavroudis C, Rigsby CL, Holinger LD. Trends in vascular ring surgery. J Thorac Cardiovasc Surg 2005;129: 1339–47. 5. Alsenaidi K, Gurofsky R, Karamlou T, Williams WG, McCrindle BW. Management and outcomes of double aortic arch in 81 patients. Pediatrics 2006;118:e1336–41. 6. Gross RE. Surgical relief for tracheal obstruction from a vascular ring. N Engl J Med 1945;233:586. 7. Ruzmetov M, Vijay P, Rodefeld MD, Turrentine MW, Brown JW. Follow-up of surgical correction of aortic arch anomalies causing tracheoesophageal compression: a 38-year single institution experience. J Pediatr Surg 2009;44:1328–32.
Double aortic arch is a rare congenital great vessel anomaly. We report a 21-year-old man who was diagnosed with a double aortic arch combining with ascending aortic aneurysm and aortic valve regurgitation. Computerized tomographic angiography and echocardiogram confirmed the diagnosis before operation. The patient underwent aortic root replacement and artificial vessel construction. At 3 years’ follow-up, the patient continues to have a good surgical recovery, although long-term results need observing. A review of the literature shows no double aortic arch combination with ascending aortic aneurysm and aortic regurgitation has been reported so far. (Ann Thorac Surg 2014;97:e43–5) Ó 2014 by The Society of Thoracic Surgeons
newly reconstructed ascending aorta through another artificial vessel (Intergard conduit 12 mm, DataScope). Postoperative computer tomography scans were performed (Figs 3, 4).
Comment Double aortic arch is a rare congenital heart malformation. The first post-mortem report of DAA was in 1737 by Hommell [1]. It also belongs to one kind of congenital vascular rings of the aortic arch, which account for 1% of cases of congenital heart disease [2]. There are three types of DAA: DAA with a right dominant arch accounts for approximately 70% of surgical cases, those with a left dominant arch accounts for approximately 25%, and a balanced arch represents about 5%. Clinically, the major symptoms of DAA are caused by vascular compression of the airway, esophagus, or both, and whether there are presentations of associated anomalies. Respiratory and gastrointestinal problems such as expiratory wheeze, tachypnea, feeding difficulty, emesis, and choking are often present at early ages in patients, even in the neonate, whereas symptomatic vascular rings manifesting in adults are rare, and most reports emphasize dysphagia as the predominant symptom [3]. Our case is an asymptomatic patient.
D
ouble aortic arch (DAA) is the most common form of the vascular rings, a class of congenital anomalies of the aortic arch system, in which the trachea and esophagus are completely encircled by connected segments of the aortic arch and its branches. Its common defining feature is that both the left and right aortic arches are present. This is a case report of a DAA with balanced aortic arches, presenting without symptoms.
An asymptomatic 21-year-old man with a heart murmur who was thought to have congenital heart disease underwent computed tomographic angiography and echocardiography, which showed that the ascending aorta was divided into an almost balanced type of right and left aortic arches. Each arch gave origin to the common carotid and subclavian artery, and then joined together posterior toward the right side of the chest to form a right descending aorta (Figs 1, 2). The aortic root was aneurysmal with a dilatation of 57 mm in diameter and associated with severe aortic valve regurgitation. During the operation, the aortic root was replaced. The aortic valve was replaced by a mechanical valve (St. Jude A25; St. Jude Medical, St. Paul, MN), and the ascending aorta was replaced by an artificial vessel (Intergard conduit 28 mm, DataScope Corp, Redmond, WA) and reconnected with the right arch. The left arch was reconnected with the Accepted for publication Sept 30, 2013. Address correspondence to Dr Chen, Department of Cardiac Surgery, The Second Hospital of Hebei Medical University, Shijiazhuang 050000, China; e-mail: [email protected].
Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc
Fig 1. An anterior perspective preoperation image (three-dimensionally reconstructed computer tomography scan of the heart and great vessels) shows the ascending aorta divided into almost balanced right and left aortic arches. Left and right subclavian and carotid arteries arose from each corresponding side of arch. The aortic root is enlarged and aneurysmal, associated with severe aortic valve regurgitation. (AO ¼ aorta; LAA ¼ left aortic arch; LCC ¼ left common carotid artery; LSA ¼ left subclavian artery; PA ¼ pulmonary artery; RAA ¼ right aortic arch; RCC ¼ right common carotid artery; RSA ¼ right subclavian artery.) 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.09.111
e44
CASE REPORT LIANG ET AL DOUBLE AORTIC ARCH
Fig 2. A posterior perspective preoperation image (three-dimensionally reconstructed computer tomography scan of heart and great vessels) shows that each arch gave origin to the common carotid and subclavian artery, and joined together posterior toward the right side of the chest to form a right descending aorta. (DA ¼ descending aorta; LAA ¼ left aortic arch; LCC ¼ left common carotid artery; LSA ¼ left subclavian artery; RAA ¼ right aortic arch; RCC ¼ right common carotid artery; RSA ¼ right subclavian artery.)
Double aortic arch is usually found as an isolated malformation. Associated cardiac anomalies occur between 7% and 17% [4, 5]. Ventricular septal defects rank first in those associated cardiac anomalies; other anomalies include atrial septal defects, patent ductus arteriosus, tetralogy of Fallot, and transposition of the great arteries. Most cases of vascular rings have a normal ascending aorta and good function of the aortic valve. Our literature search did not show any case of double aortic arch associated with ascending aortic aneurysm and severe aortic valve regurgitation. It is accidental for the nonsymptomatic patient to be diagnosed with such anomalies just because of a heart murmur. Although chest roentgenograph can provide useful clues for diagnosis, the most convenient and important diagnostic technique for patients with DAA is echocardiography; the diagnosis even can be obtained during the in utero period in major heart centers by echocardiography. It is also a useful and noninvasive method for identifying associated cardiac anomalies, although the major problem of echocardiography resides in its limited ability to image the atretic aortic arch and ligamentum arteriosum. Cardiac catheterization with angiography can provide a diagnosis of DAA, but it is neither routinely required nor clinically feasible for smaller patients with severe cardiopulmonary diseases. Instead, computerized tomographic and magnetic resonance imaging can be performed safely and accurately. They also can provide the arterial branching pattern, the locations of airway and esophageal
Ann Thorac Surg 2014;97:e43–5
Fig 3. An anterior perspective postoperation image (three-dimensionally reconstructed computer tomography scan of heart and great vessels) shows the aortic root was replaced. The aortic valve was replaced by a mechanical valve; the ascending aorta was replaced by an artificial conduit vessel; the newly reconstructed ascending aortic conduit was reconnected with the right arch. The left arch was reconnected with the newly reconstructed ascending aorta through another new artificial conduit vessel. The inner space of the vascular ring was then enlarged. (LAA ¼ left aortic arch; LCC ¼ left common carotid artery; LSA ¼ left subclavian artery; NCAV ¼ newly constructed artificial vessel; NRAA ¼ newly reconstructed ascending aorta; RAA ¼ right aortic arch; RCC ¼ right common carotid artery; RSA ¼ right subclavian artery.)
obstructions, and associated cardiac anatomy. Meanwhile, they both are of great value for operative planning. Surgical care is necessary in any patient with symptoms of airway and esophageal compression and associated cardiac anomalies. It has been a standard of care for more than 60 years. The first open surgical correction was performed by Gross in 1945 [6]. The main purpose of surgery is to eliminate compression and associated cardiac anomalies caused by a vascular ring. Surgical mortality of isolated DAA is very low, but in those patients who exhibit other cardiac anomalies, mortality depends on coexisting conditions [7]. Our patient was asymptomatic; his major problems were ascending aortic aneurysm and severe aortic valve regurgitation, not vascular ring compression. The aortic root (ascending aorta and aortic valve) was replaced (similar to a Bentall procedure). The new reconstructed ascending aorta was connected with the right arch. The left arch was then reconnected with the newly reconstructed ascending aorta through a relatively small constructed artificial vessel, after which the inner space of the vascular ring was enlarged to avoid a possible compression, which possibly would appear in the future. Although 3 years’ surgical follow-up shows
Ann Thorac Surg 2014;97:e43–5
CASE REPORT LIANG ET AL DOUBLE AORTIC ARCH
e45
excellent result with no complications, long-term followup is warranted. The authors thank Mr Sujeeth Harischandra Suvarna, Senior Consultant Cardiothoracic Surgeon of the KMC-Manipal Hospitals, India, and Mr Jazeel Nimrry, Senior Consultant Cardiothoracic Surgeon, from Sri Lanka, who provided professional advice and language assistance.
References
Fig 4. A posterior perspective postoperation image (three-dimensionally reconstructed computer tomography scan of heart and great vessels) shows reconstruction of the arch vessels. (DA ¼ descending aorta; LAA ¼ left aortic arch; LSA ¼ left subclavian artery; NCAV ¼ newly constructed artificial vessel; RAA ¼ right aortic arch; RCC ¼ right common carotid artery; RSA ¼ right subclavian artery.)
1. Hommell L. Observationes anatomicae de arcu aortae bifido du dueto thoracico duplica, et de carstidum atque subclaviarum. Hildomas 1737;21:161. 2. Nadas AS, Fyler DC. Pediatric cardiology. Philadelphia: WB Saunders; 1972, p. 749. 3. Kouchoukos NT, Blackstone EH, Doty DB, Hanley FL, Karp RB. Kirklin/Barratt-Boyes cardiac surgery. Third Edition. Philadelphia, PA: Churchill Livingstone; 2003:1420. 4. Backer CL, Mavroudis C, Rigsby CL, Holinger LD. Trends in vascular ring surgery. J Thorac Cardiovasc Surg 2005;129: 1339–47. 5. Alsenaidi K, Gurofsky R, Karamlou T, Williams WG, McCrindle BW. Management and outcomes of double aortic arch in 81 patients. Pediatrics 2006;118:e1336–41. 6. Gross RE. Surgical relief for tracheal obstruction from a vascular ring. N Engl J Med 1945;233:586. 7. Ruzmetov M, Vijay P, Rodefeld MD, Turrentine MW, Brown JW. Follow-up of surgical correction of aortic arch anomalies causing tracheoesophageal compression: a 38-year single institution experience. J Pediatr Surg 2009;44:1328–32.
