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Cervical Aortic Arch with Retroesophageal Aortic Obstruction: Report of a Case with Successful Surgical Intervention
CASE REPORT
Cervical Aortic Arch with Retroesophageal Aortic Obstruction: Report of a Case with Successful Surgical Intervention William E. Hellenbrand, M.D., Michael J. Kelley, M.D., Norman S. Talner, M.D., H. C. Stansel, Jr., M.D., a n d Michael A. Berman, M.D.
ABSTRACT The clinical, roentgenographic, hemodynamic, and angiographic features of a patient with a right cervical aortic arch and retroesophageal aortic obstruction associated with a ventricular septal defect are presented. Surgical relief of the aortic obstruction was successfully achieved by placement of a bypass graft between the left common carotid artery and the descending thoracic aorta.
The cervical aortic arch complex consists of the following: (1)cervical position of the apex of the aortic arch with separate origin of the contralatera1 common carotid artery; (2) a retroesophageal descending aorta crossing contralateral to the arch; and (3) anomalous origin of the subclavian artery off the descending aorta [6]. This entity has been reported in association with ipsilateral separate origins of the external and internal carotid artery, stenosis of the contralateral subclavian artery, and a vascular ring [4, 6, 101. Only 4 cases of cervical aortic arch with intracardiac defects have been described [51. This communication presents a patient with right cervical aortic arch complex, a large ventricular septal defect (VSD) complicated by severe narrowing of a tortuous retroesophageal segment of the aorta, and contralateral subclavian artery steal. This is the first report of major aortic obstruction associated with a cervical aortic arch. Relief of this obstruction was successfully achieved by insertion of a bypass conduit. From the Departments of Pediatrics, Diagnostic Radiology, and Surgery, Yale University School of Medicine and Yale-New Haven Hospital, New Haven, CT. Accepted for publication Dec 22, 1977. Address reprint requests to Dr. Hellenbrand, Department of Pediatrics, Yale University School of Medicine, 333 Cedar St, New Haven, CT 06510.
A male infant (birth weight 3.3 kg) was seen initially at 10 weeks of age with signs and symptoms of severe congestive heart failure. (This patient has previously been reported as a case of double aortic arch producing obstruction to left ventricular outflow tract [2].) Systolic blood pressure in the right arm (138 mm Hg) was 30 mm greater than in the left arm and legs. The right brachial pulse was prominent, but the left brachial and femoral pulses were barely palpable. Auscultation of the heart revealed a pansystolic murmur along the left sternal border, consistent with a VSD. An electrocardiogram demonstrated combined ventricular hypertrophy. The chest roentgenogram showed increased pulmonary vascularity, cardiomegaly, and a poorly defined aortic knob (Fig 1). Cardiac catheterization (Table) and angiography (Fig 2) demonstrated a large left-to-right shunt at the ventricular level (Q,/Q, = 2.711.0) with pulmonary artery hypertension and severe obstruction over a long hypoplastic segment of descending aorta. Medical management was unsuccessful in controlling congestive heart failure, and an operation to relieve the aortic obstruction was performed. At operation, no continuity was found between the left common carotid artery and the descending aorta. The descending aorta filled from a tortuous retroesophageal vessel. Pulmonary artery banding was performed to control the excess pulmonary blood flow, but no surgical relief of the aortic obstruction was attempted. The patient was maintained on digitalis and diuretics. Systemic hypertension gradually increased (right arm systolic pressure of 200 mm Hg). Repeat heart catheterization at 2l/2 years of age revealed a peak systolic pressure difference of 60 mm Hg between the ascending and descending aorta. Pulmonary artery pressure was
86 0003-4975/78/0026-0117$01.25@ 1978 by William E. Hellenbrand
87
Case Report: Hellenbrand et al: Cervical Aortic Arch with Aortic Obstruction
Fig 1. Posteroanterior chest roentgenogram at 10 zueeks of age demonstrating increased pulmonary vascularity and cardiornegaly. Note the poorly defined aortic knob and the right superior mediastinal widening.
clavian arteries originated from the proximal portion of a very tortuous, elongated retroesophageal aortic segment. This segment extended from the first to the sixth thoracic vertebral level, where it communicated with the descending thoracic aorta. There was delayed retrograde filling of the left vertebral and left subclavian artery; the latter communicated with the descending aorta at the fifth thoracic vertebral level. A second operation was performed through a left thoracotomy to relieve the aortic obstruction. The aorta was reconstructed with a 12 mm Gore-Tex expanded microporous polytetrafluoroethylene graft sutured end to side between the left common carotid artery and the descending aorta. Postoperative catheterization and angiography at age 4 years 9 months revealed significant hemodynamic improvement (see the Table), patency of the graft from the left common carotid artery to the left descending aorta, and antegrade filling of the left subclavian artery (Fig 4). The VSD was successfully closed, and the pulmonary artery band was removed at age 5 years.
75/32 mm Hg (mean, 51) and pulmonary vascular resistance was elevated at 5 resistance units per square meter of body surface area (see the Table). The ascending aortogram (Fig 3) revealed an elongated ascending aorta with the arch in the right supraclavicular area. The first Comment branch of the ascending aorta was a large left Although cervical aortic arch was described in common carotid artery. The right external and 1913 [7], the embryological error responsible for internal carotid arteries arose separately at the this malformation has not been clearly defined. apex of the arch. The right vertebral and sub- A commonly accepted theory is that there is
Hernodynamic Data Pressures (mm Hg) ~
Pulmonary Pulmonary Artery Left Artery Wedge Ventricle
Patient's Age
Right Ventricle
2 mo 2l/2 yr
10016 160112
100130 75/32a M = 51
43/4yr
120/5
30112a M = 18
Pulmonary Vascular Ascending Descending Resistance Aorta Aorta (RUlm')
100115 A= V= M= A= V= M=
18 16 15 11 10 6
~~
aPressure distal to pulmonary artery band.
RU = resistance units; A = arterial; V = venous; M = mean
QJQ,
160175
100180
7.8 5.0
2.7:l 1.9:l
130160
120/60
2.0
1.8:1
88 The Annals of Thoracic Surgery Vol 26 No 1 July 1978
A
B
Fig 2 . (A)Slight left anterior oblique and ( B ) lateral views of a left ventriculogram demonstrating a normalsized left ventricle (LV), a large infracristal V S D (large white arrows), a slightly dilated ascending aorta (AA), and a tortuous hypoplastic descending aorta (small white arrows). Film coning at this time did not include the neck region, so that the anatomy of the right cervical arch (note the position of the trachea [TI) was not clearly defined. (RV = right ventricle; PT = pulmonary trunk:)
nary stenosis, and VSD [5]. Only 1 of these patients presented in congestive heart failure redefect lated to the associated (double-outlet right ventricle). In the present patient, congestive heart failure was due to the combined effects of aortic obstruction and increased pulmonary blood flow through a large infracristal VSD. Severe obstruction between the cervical arch and the descending aorta has not been de-
persistence of the third rather than the fourth brachial arch [5]. The ipsilateral ductus caroticus remains, leading to separate origins of the external and internal carotid arteries from the cervical arch. The contralateral subclavian artery arises from the descending aorta, since the dorsal aorta persists as the continuation of the seventh intersegmental artery. The clinical significance of this anomaly did not become apparent until 1947, following ligation of a cervical arch mistakenly diagnosed as a carotid aneurysm [l]. To date, 25 cases have been reported, most appearing as either a pulsatile mass in the neck or with symptoms of upper airway obstruction or dysphagia, or both [51. The four intracardiac defects described in association with the cervical arch have included tetralogy of Fallot, pulmonary atresia with VSD, double-outlet right ventricle without pulmo-
Fig 3 . Posteroanterior ascending cineaortogram at 2 1/2 years of age. ( A ) Early arterial phase, with the right heart catheter traversing the ventricular septa1 defect to reach the ascending aorta (AA). There is a large left common carotid artery (LCC), with the aorta continuing into the right supraclavicular area as the right cervical arch (CA). The arch gives rise to a right internal carotid (RIC) and a right external carotid artery (REC). Note the level of the right clavicle (CL). ( B ) Later phase of the aortogram showing the origin of the right subclavian artery (RSA) and right vertebral artery (RVA) from the proximal descending limb of the right cervical arch (CA). The tortuous, hypoplastic descending aortic segment (arrows)joins the descending thoracic aorta (DA) at the level of the sixth thoracic vertebra (T-6). (0Late phase of the aortogram demonstrating retrograde flow in the left vertebral artery (LVA), with filling of the left subclavian artery (LSA), which joins the descending aorta (DA) at the fifth thoracic level, creating a left subclavian steal. ( D ) Diagram summarizing the key angiographic findings (abbreviations as in A-C).
89 Case Report: Hellenbrand et al: Cervical Aortic Arch with Aortic Obstruction
A
B
C
D
90 The Annals of Thoracic Surgery Vol 26 No 1 July 1978
A
B
Fig 4 . Anteroposterior ascending thoracic aortogram performed a t age 4 years 9 months, following placement of a Gore-Tex graft from the left common carotid artery to the descending aorta. ( A ) Early arterial phase shoming similar anatomy as in Figure 2 A , with the right cervical arch (CA) and the left common carotid artery (LCC) arisingfrom the ascending aorta (AA). The patent Gore-Tex graft ( G )is clearly visualized between the left common carotid artery and the descending thoracic aorta (DA). ( B ) Later phase of the aortogram demonstrating the cervical arch (CA), the origin of the right subclavian artery (RSA)from the proximal descendin,? aorta, and primary filling of the left subclavian artery (LSA)frorn the Gore-Tex graftdescending aorta junction.. (C) Late phase of the arteriogram showing venous return from the right internal jugular vein (JV), with opacification of the right atrium (RA).Note displacement o f t h e jugular vein to the right and the impression on the rightside of the trachea (arrow)from the right cervical arch complex.
C
91 Case Report: Hellenbrand et al: Cervical Aortic Arch with Aortic Obstruction
scribed, although several authors have reported small pressure differences [5, 6, 81. In the present case, an extremely tortuous retroesophageal vessel connected the right cervical arch and left descending aorta. There was no apparent discrete obstruction in this vessel. The peak systolic pressure difference of 60 mm Hg resulted from the narrow diameter and long, tortuous course. Origin of the left subclavian artery distal to the obstruction, with filling through the left vertebral artery, produced a left subclavian artery steal. Cervical aortic arch must be differentiated from vascular rings, great vessel aneurysms, and other entities in which the presenting symptom is pulsatile neck mass, upper airway obstruction, or dysphagia [3]. Original and follow-up chest roentgenograms in the present case (see Fig 1) illustrate several radiographic features that may be of help in differentiating cervical arch from the other conditions: 1. Absence of a normal aortic knob. 2. Localized indentation of the upper tracheal air column on the side of the arch. 3. Superior mediastinal widening. 4. Soft tissue mass in the supraclavicular area. 5. Descending aorta contralateral to the superior mediastinal mass. 6 . An oblique posterior impression on the barium column (representing the retroesophageal aortic segment), with absence of an associated anterior tracheal impression.
The initial banding procedure performed in the present patient markedly improved the congestive heart failure by decreasing the volume of blood from the left-to-right shunt. However, a pressure load was created in the right ventricle, in addition to that already present in the left ventricle. Relief of the aortic obstruction was undertaken when the patient was 2% years of age because of marked systemic hypertension in the upper body, pulmonary artery hypertension distal to the band (see the Table), and a left subclavian steal. The elevated systemic pressure in this patient represented an increased afterload on the left ventricle with the possibility that irrevers-
ible subendocardial fibrosis might develop. Moreover, with cerebral hypertension, spontaneous cerebrovascular accidents in the first decade of life have been observed [9]. The elevated pulmonary wedge pressure reflected the increased left ventricular end-diastolic pressure associated with the pressure load on the left ventricle. Pulmonary vascular resistance was elevated and represented a reactive constriction of the vascular bed, with a secondary increase in the pulmonary artery pressure distal to the band. The subclavian artery steal posed the risk of future cerebral insufficiency. Surgical intervention in patients with cervical aortic arch has been reported for relief of subclavian steal, vascular ring, or associated intracardiac defects [5,10]. Since the intracardiac defect had been well palliated in infancy in our patient, the operation was performed to relieve the aortic obstruction. A Gore-Tex graft was interposed between the left common carotid artery and the descending aorta through a left thoracotomy. A direct anastomosis between the ascending and descending aorta was not performed because this would lie directly anterior to the pulmonary artery and complicate future removal of the pulmonary artery band at the time of VSD repair. Postoperative hemodynamics (see the Table) revealed a minimal pressure difference between the ascending and descending aorta, with increased pulse pressure in the distal aortic segment consistent with decreased obstruction. Decreased left ventricular afterload was manifested by a fall in distal pulmonary artery and pulmonary wedge pressures and by normal pulmonary vascular resistance. The normal pulmonary vascular resistance with an unchanged pulmonary-systemic flow ratio also confirmed that the previously elevated resistance represented passive reactive constriction. Angiography revealed patency of the graft from the left common carotid to the descending aorta with antegrade flow into the left subclavian and vertebral arteries (see Fig 4). Hemodynamic and angiographic evaluation of the Gore-Tex graft will be performed at appropriate intervals to assess the need for replacem.ent of the graft as the patient grows.
92 The Annals of Thoracic Surgery Vol 26 No 1 July 1978
References
1. Beavan TED, Fatti L: Ligature of aortic arch in neck. Br J Surg 34:414, 1947 2. Brenner JI, Berman MA, Talner NS, et al: Double aortic arch system producing obstruction to left ventricular outflow. Am J Cardiol 34:854, 1974 3. Lewis C, Rogers L: Cervical aortic knuckle which resembles aneurysm. Lancet 1925, 1953 4. Massumi R, Wiener L, Charif P: The syndrome of cervical aorta. Report of a case and review of the previous cases. Am J Cardiol 11:678, 1963 5. Moncado R, Shannon M, Miller R, et al: The cervical aortic arch. Am J Roentgen01 125:591, 1975
6. Mullins CE, Gillette PC, McNamara DG: The complex of cervical aortic arch. Pediatrics 51:210, 1973 7. Reid DG: Three examples of right aortic arch. J. Anat. Physiol 48:174, 1913 8. Richie R, Delrio C, Mullins CE, et al: Right sided cervical aortic arch. Am Heart J 84:531, 1972 9. Shearer WT, Rutman JY, Weinberg WA, et al: Coarctation of the aorta and cerebrovascular accident: a proposal for early corrective surgery. J Pediatr 77:1004, 1970 10. Shepherd RM, Kerth WJ, Rosenthal JH: Right cervical aortic arch with left descending aorta. Am J Dis Child 118:642, 1969
Cervical Aortic Arch with Retroesophageal Aortic Obstruction: Report of a Case with Successful Surgical Intervention William E. Hellenbrand, M.D., Michael J. Kelley, M.D., Norman S. Talner, M.D., H. C. Stansel, Jr., M.D., a n d Michael A. Berman, M.D.
ABSTRACT The clinical, roentgenographic, hemodynamic, and angiographic features of a patient with a right cervical aortic arch and retroesophageal aortic obstruction associated with a ventricular septal defect are presented. Surgical relief of the aortic obstruction was successfully achieved by placement of a bypass graft between the left common carotid artery and the descending thoracic aorta.
The cervical aortic arch complex consists of the following: (1)cervical position of the apex of the aortic arch with separate origin of the contralatera1 common carotid artery; (2) a retroesophageal descending aorta crossing contralateral to the arch; and (3) anomalous origin of the subclavian artery off the descending aorta [6]. This entity has been reported in association with ipsilateral separate origins of the external and internal carotid artery, stenosis of the contralateral subclavian artery, and a vascular ring [4, 6, 101. Only 4 cases of cervical aortic arch with intracardiac defects have been described [51. This communication presents a patient with right cervical aortic arch complex, a large ventricular septal defect (VSD) complicated by severe narrowing of a tortuous retroesophageal segment of the aorta, and contralateral subclavian artery steal. This is the first report of major aortic obstruction associated with a cervical aortic arch. Relief of this obstruction was successfully achieved by insertion of a bypass conduit. From the Departments of Pediatrics, Diagnostic Radiology, and Surgery, Yale University School of Medicine and Yale-New Haven Hospital, New Haven, CT. Accepted for publication Dec 22, 1977. Address reprint requests to Dr. Hellenbrand, Department of Pediatrics, Yale University School of Medicine, 333 Cedar St, New Haven, CT 06510.
A male infant (birth weight 3.3 kg) was seen initially at 10 weeks of age with signs and symptoms of severe congestive heart failure. (This patient has previously been reported as a case of double aortic arch producing obstruction to left ventricular outflow tract [2].) Systolic blood pressure in the right arm (138 mm Hg) was 30 mm greater than in the left arm and legs. The right brachial pulse was prominent, but the left brachial and femoral pulses were barely palpable. Auscultation of the heart revealed a pansystolic murmur along the left sternal border, consistent with a VSD. An electrocardiogram demonstrated combined ventricular hypertrophy. The chest roentgenogram showed increased pulmonary vascularity, cardiomegaly, and a poorly defined aortic knob (Fig 1). Cardiac catheterization (Table) and angiography (Fig 2) demonstrated a large left-to-right shunt at the ventricular level (Q,/Q, = 2.711.0) with pulmonary artery hypertension and severe obstruction over a long hypoplastic segment of descending aorta. Medical management was unsuccessful in controlling congestive heart failure, and an operation to relieve the aortic obstruction was performed. At operation, no continuity was found between the left common carotid artery and the descending aorta. The descending aorta filled from a tortuous retroesophageal vessel. Pulmonary artery banding was performed to control the excess pulmonary blood flow, but no surgical relief of the aortic obstruction was attempted. The patient was maintained on digitalis and diuretics. Systemic hypertension gradually increased (right arm systolic pressure of 200 mm Hg). Repeat heart catheterization at 2l/2 years of age revealed a peak systolic pressure difference of 60 mm Hg between the ascending and descending aorta. Pulmonary artery pressure was
86 0003-4975/78/0026-0117$01.25@ 1978 by William E. Hellenbrand
87
Case Report: Hellenbrand et al: Cervical Aortic Arch with Aortic Obstruction
Fig 1. Posteroanterior chest roentgenogram at 10 zueeks of age demonstrating increased pulmonary vascularity and cardiornegaly. Note the poorly defined aortic knob and the right superior mediastinal widening.
clavian arteries originated from the proximal portion of a very tortuous, elongated retroesophageal aortic segment. This segment extended from the first to the sixth thoracic vertebral level, where it communicated with the descending thoracic aorta. There was delayed retrograde filling of the left vertebral and left subclavian artery; the latter communicated with the descending aorta at the fifth thoracic vertebral level. A second operation was performed through a left thoracotomy to relieve the aortic obstruction. The aorta was reconstructed with a 12 mm Gore-Tex expanded microporous polytetrafluoroethylene graft sutured end to side between the left common carotid artery and the descending aorta. Postoperative catheterization and angiography at age 4 years 9 months revealed significant hemodynamic improvement (see the Table), patency of the graft from the left common carotid artery to the left descending aorta, and antegrade filling of the left subclavian artery (Fig 4). The VSD was successfully closed, and the pulmonary artery band was removed at age 5 years.
75/32 mm Hg (mean, 51) and pulmonary vascular resistance was elevated at 5 resistance units per square meter of body surface area (see the Table). The ascending aortogram (Fig 3) revealed an elongated ascending aorta with the arch in the right supraclavicular area. The first Comment branch of the ascending aorta was a large left Although cervical aortic arch was described in common carotid artery. The right external and 1913 [7], the embryological error responsible for internal carotid arteries arose separately at the this malformation has not been clearly defined. apex of the arch. The right vertebral and sub- A commonly accepted theory is that there is
Hernodynamic Data Pressures (mm Hg) ~
Pulmonary Pulmonary Artery Left Artery Wedge Ventricle
Patient's Age
Right Ventricle
2 mo 2l/2 yr
10016 160112
100130 75/32a M = 51
43/4yr
120/5
30112a M = 18
Pulmonary Vascular Ascending Descending Resistance Aorta Aorta (RUlm')
100115 A= V= M= A= V= M=
18 16 15 11 10 6
~~
aPressure distal to pulmonary artery band.
RU = resistance units; A = arterial; V = venous; M = mean
QJQ,
160175
100180
7.8 5.0
2.7:l 1.9:l
130160
120/60
2.0
1.8:1
88 The Annals of Thoracic Surgery Vol 26 No 1 July 1978
A
B
Fig 2 . (A)Slight left anterior oblique and ( B ) lateral views of a left ventriculogram demonstrating a normalsized left ventricle (LV), a large infracristal V S D (large white arrows), a slightly dilated ascending aorta (AA), and a tortuous hypoplastic descending aorta (small white arrows). Film coning at this time did not include the neck region, so that the anatomy of the right cervical arch (note the position of the trachea [TI) was not clearly defined. (RV = right ventricle; PT = pulmonary trunk:)
nary stenosis, and VSD [5]. Only 1 of these patients presented in congestive heart failure redefect lated to the associated (double-outlet right ventricle). In the present patient, congestive heart failure was due to the combined effects of aortic obstruction and increased pulmonary blood flow through a large infracristal VSD. Severe obstruction between the cervical arch and the descending aorta has not been de-
persistence of the third rather than the fourth brachial arch [5]. The ipsilateral ductus caroticus remains, leading to separate origins of the external and internal carotid arteries from the cervical arch. The contralateral subclavian artery arises from the descending aorta, since the dorsal aorta persists as the continuation of the seventh intersegmental artery. The clinical significance of this anomaly did not become apparent until 1947, following ligation of a cervical arch mistakenly diagnosed as a carotid aneurysm [l]. To date, 25 cases have been reported, most appearing as either a pulsatile mass in the neck or with symptoms of upper airway obstruction or dysphagia, or both [51. The four intracardiac defects described in association with the cervical arch have included tetralogy of Fallot, pulmonary atresia with VSD, double-outlet right ventricle without pulmo-
Fig 3 . Posteroanterior ascending cineaortogram at 2 1/2 years of age. ( A ) Early arterial phase, with the right heart catheter traversing the ventricular septa1 defect to reach the ascending aorta (AA). There is a large left common carotid artery (LCC), with the aorta continuing into the right supraclavicular area as the right cervical arch (CA). The arch gives rise to a right internal carotid (RIC) and a right external carotid artery (REC). Note the level of the right clavicle (CL). ( B ) Later phase of the aortogram showing the origin of the right subclavian artery (RSA) and right vertebral artery (RVA) from the proximal descending limb of the right cervical arch (CA). The tortuous, hypoplastic descending aortic segment (arrows)joins the descending thoracic aorta (DA) at the level of the sixth thoracic vertebra (T-6). (0Late phase of the aortogram demonstrating retrograde flow in the left vertebral artery (LVA), with filling of the left subclavian artery (LSA), which joins the descending aorta (DA) at the fifth thoracic level, creating a left subclavian steal. ( D ) Diagram summarizing the key angiographic findings (abbreviations as in A-C).
89 Case Report: Hellenbrand et al: Cervical Aortic Arch with Aortic Obstruction
A
B
C
D
90 The Annals of Thoracic Surgery Vol 26 No 1 July 1978
A
B
Fig 4 . Anteroposterior ascending thoracic aortogram performed a t age 4 years 9 months, following placement of a Gore-Tex graft from the left common carotid artery to the descending aorta. ( A ) Early arterial phase shoming similar anatomy as in Figure 2 A , with the right cervical arch (CA) and the left common carotid artery (LCC) arisingfrom the ascending aorta (AA). The patent Gore-Tex graft ( G )is clearly visualized between the left common carotid artery and the descending thoracic aorta (DA). ( B ) Later phase of the aortogram demonstrating the cervical arch (CA), the origin of the right subclavian artery (RSA)from the proximal descendin,? aorta, and primary filling of the left subclavian artery (LSA)frorn the Gore-Tex graftdescending aorta junction.. (C) Late phase of the arteriogram showing venous return from the right internal jugular vein (JV), with opacification of the right atrium (RA).Note displacement o f t h e jugular vein to the right and the impression on the rightside of the trachea (arrow)from the right cervical arch complex.
C
91 Case Report: Hellenbrand et al: Cervical Aortic Arch with Aortic Obstruction
scribed, although several authors have reported small pressure differences [5, 6, 81. In the present case, an extremely tortuous retroesophageal vessel connected the right cervical arch and left descending aorta. There was no apparent discrete obstruction in this vessel. The peak systolic pressure difference of 60 mm Hg resulted from the narrow diameter and long, tortuous course. Origin of the left subclavian artery distal to the obstruction, with filling through the left vertebral artery, produced a left subclavian artery steal. Cervical aortic arch must be differentiated from vascular rings, great vessel aneurysms, and other entities in which the presenting symptom is pulsatile neck mass, upper airway obstruction, or dysphagia [3]. Original and follow-up chest roentgenograms in the present case (see Fig 1) illustrate several radiographic features that may be of help in differentiating cervical arch from the other conditions: 1. Absence of a normal aortic knob. 2. Localized indentation of the upper tracheal air column on the side of the arch. 3. Superior mediastinal widening. 4. Soft tissue mass in the supraclavicular area. 5. Descending aorta contralateral to the superior mediastinal mass. 6 . An oblique posterior impression on the barium column (representing the retroesophageal aortic segment), with absence of an associated anterior tracheal impression.
The initial banding procedure performed in the present patient markedly improved the congestive heart failure by decreasing the volume of blood from the left-to-right shunt. However, a pressure load was created in the right ventricle, in addition to that already present in the left ventricle. Relief of the aortic obstruction was undertaken when the patient was 2% years of age because of marked systemic hypertension in the upper body, pulmonary artery hypertension distal to the band (see the Table), and a left subclavian steal. The elevated systemic pressure in this patient represented an increased afterload on the left ventricle with the possibility that irrevers-
ible subendocardial fibrosis might develop. Moreover, with cerebral hypertension, spontaneous cerebrovascular accidents in the first decade of life have been observed [9]. The elevated pulmonary wedge pressure reflected the increased left ventricular end-diastolic pressure associated with the pressure load on the left ventricle. Pulmonary vascular resistance was elevated and represented a reactive constriction of the vascular bed, with a secondary increase in the pulmonary artery pressure distal to the band. The subclavian artery steal posed the risk of future cerebral insufficiency. Surgical intervention in patients with cervical aortic arch has been reported for relief of subclavian steal, vascular ring, or associated intracardiac defects [5,10]. Since the intracardiac defect had been well palliated in infancy in our patient, the operation was performed to relieve the aortic obstruction. A Gore-Tex graft was interposed between the left common carotid artery and the descending aorta through a left thoracotomy. A direct anastomosis between the ascending and descending aorta was not performed because this would lie directly anterior to the pulmonary artery and complicate future removal of the pulmonary artery band at the time of VSD repair. Postoperative hemodynamics (see the Table) revealed a minimal pressure difference between the ascending and descending aorta, with increased pulse pressure in the distal aortic segment consistent with decreased obstruction. Decreased left ventricular afterload was manifested by a fall in distal pulmonary artery and pulmonary wedge pressures and by normal pulmonary vascular resistance. The normal pulmonary vascular resistance with an unchanged pulmonary-systemic flow ratio also confirmed that the previously elevated resistance represented passive reactive constriction. Angiography revealed patency of the graft from the left common carotid to the descending aorta with antegrade flow into the left subclavian and vertebral arteries (see Fig 4). Hemodynamic and angiographic evaluation of the Gore-Tex graft will be performed at appropriate intervals to assess the need for replacem.ent of the graft as the patient grows.
92 The Annals of Thoracic Surgery Vol 26 No 1 July 1978
References
1. Beavan TED, Fatti L: Ligature of aortic arch in neck. Br J Surg 34:414, 1947 2. Brenner JI, Berman MA, Talner NS, et al: Double aortic arch system producing obstruction to left ventricular outflow. Am J Cardiol 34:854, 1974 3. Lewis C, Rogers L: Cervical aortic knuckle which resembles aneurysm. Lancet 1925, 1953 4. Massumi R, Wiener L, Charif P: The syndrome of cervical aorta. Report of a case and review of the previous cases. Am J Cardiol 11:678, 1963 5. Moncado R, Shannon M, Miller R, et al: The cervical aortic arch. Am J Roentgen01 125:591, 1975
6. Mullins CE, Gillette PC, McNamara DG: The complex of cervical aortic arch. Pediatrics 51:210, 1973 7. Reid DG: Three examples of right aortic arch. J. Anat. Physiol 48:174, 1913 8. Richie R, Delrio C, Mullins CE, et al: Right sided cervical aortic arch. Am Heart J 84:531, 1972 9. Shearer WT, Rutman JY, Weinberg WA, et al: Coarctation of the aorta and cerebrovascular accident: a proposal for early corrective surgery. J Pediatr 77:1004, 1970 10. Shepherd RM, Kerth WJ, Rosenthal JH: Right cervical aortic arch with left descending aorta. Am J Dis Child 118:642, 1969