Surgical repair of right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum Five cases of right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum, with or without Kommerell's diverticulum, are presented. Either right or left thoracotomy with division of the aberrant retroesophageal left subclavian artery can be accomplished with good results. However, a right thoracotomy is recommended in the presence of a large Kommerell diverticulum because such a diverticulum should be excised. The development of a subclavian steal syndrome later in life may be prevented by reimplantation qf the divided left subclavian artery into the aorta or the left common carotid artery. r. Y. lung, M.D., Carl H. Almond, M.D., Salim B. Saab, M.D., and Zudhi Lababidi, M.D., Columbia, Mo. The diagnosis and management of the anomalies of the aortic arch and its branches have been well estab- lished. Some of these cases are asymptomatic-either diagnosed at autopsy or as an incidental finding during the angiographic studies for other cardiac defects. However, vascular rings can cause symptoms by con- striction or compression of the tracheaor esophagus. In patients with the latter symptom, accurate diagnosis is essential for planning the approach of a correct opera- tive treatment. The literature is abundant on these sub- jects. However, specific attention to the anomaly of right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum with or without Korn- merell's diverticulum is sporadic, and surgical ap- proaches are not defined clearly. It is the purpose of this communication to report our experience with five such patients and to consider the surgical approach in their management. Case presentations From 1971 through May, 1977, five patients with right aortic arch and aberrant left subclavian artery From the Section of Thoracic and Cardiovascular Surgery, Univer- sity of Missouri Medical Center, Columbia, Mo. Received for publication June I, 1977. Accepted for publication July 15, 1977. Address for reprints: Carl H. Almond, M. D., Professor and Chief, Section of Thoracic and Cardiovascular Surgery, University of Missouri Medical Center, Columbia, Mo. 65201. 0022-5223/78/0275-0237$00.70/0 © 1978 The C. V. Mosby Co. were seen at the University of Missouri Medical Cen- ter. Four of them underwent operative treatment. There were four male patients and one female patient, whose ages ranged from 10 weeks to 14 years. Two patients required two separate operations each to relieve symp- toms completely. Brief case presentations follow. CAS E I. A 10-week-old infant had cyanosis, wheezing, and repeated choking spells. Cardiac catheterization was per- formed. Tetralogy of Fallot, right aortic arch, and aberrant left subclavian artery were diagnosed. Barium swallow dis- closed an indentation at the retroesophageal area. Left thoracotomy was performed because of the patient's respi- ratory distress. At operation, a retroesophageal aberrant left subclavian artery with left ligamentum arteriosum forming a constricting vascular ring was found. The ligamentum ar- teriosum was divided, and the aberrant subclavian artery was suspended to the paravertebral fascia. After operation, respi- ratory distress was markedly improved, but the patient con- tinued to have choking spells and was unable to swallow solid food at the age of 2 years. Barium swallow was repeated and marked retroesophageal indentation was demonstrated again. Right thoracotomy with division of the aberrant left subcla- vian artery was carried out. The postoperative course was uneventful. He was followed for 2 years. No arm weakness or dysphagia was noted. When he was 4 years of age, correction of tetralogy of Fallot was carried out. He died suddenly after onset of ventricular fibrillation 24 hours after operation. CASE 2. A l6-month-old boy presented with stridor, wheezing, feeding difficulty, and heart murmurs. Barium swallow demonstrated a retroesophageal concavity (Fig. I). Cardiac catheterization disclosed a small ventricular septal defect with left-to-right shunt and a Qp/Qs of 1.21. Right aortic arch with aberrant left subclavian artery arising from a 237
Surgical repair of right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum
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Surgical repair of right aortic arch with aberrant left subclavian artery and left ligamentum arteriosumSurgical repair of right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum
Five cases of right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum, with or without Kommerell's diverticulum, are presented. Either right or left thoracotomy with division of the aberrant retroesophageal left subclavian artery can be accomplished with good results. However, a right thoracotomy is recommended in the presence of a large Kommerell diverticulum because such a diverticulum should be excised. The development of a subclavian steal syndrome later in life may be prevented by reimplantation qf the divided left subclavian artery into the aorta or the left common carotid artery.
r. Y. lung, M.D., Carl H. Almond, M.D., Salim B. Saab, M.D., and Zudhi Lababidi, M.D.,
Columbia, Mo.
The diagnosis and management of the anomalies of the aortic arch and its branches have been well estab- lished. Some of these cases are asymptomatic-either diagnosed at autopsy or as an incidental finding during the angiographic studies for other cardiac defects. However, vascular rings can cause symptoms by con- striction or compression of the trachea or esophagus. In patients with the latter symptom, accurate diagnosis is essential for planning the approach of a correct opera- tive treatment. The literature is abundant on these sub- jects. However, specific attention to the anomaly of right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum with or without Korn- merell's diverticulum is sporadic, and surgical ap- proaches are not defined clearly. It is the purpose of this communication to report our experience with five such patients and to consider the surgical approach in their management.
Case presentations
From 1971 through May, 1977, five patients with right aortic arch and aberrant left subclavian artery
From the Section of Thoracic and Cardiovascular Surgery, Univer- sity of Missouri Medical Center, Columbia, Mo.
Received for publication June I, 1977.
Accepted for publication July 15, 1977.
Address for reprints: Carl H. Almond, M. D., Professor and Chief, Section of Thoracic and Cardiovascular Surgery, University of Missouri Medical Center, Columbia, Mo. 65201.
0022-5223/78/0275-0237$00.70/0 © 1978 The C. V. Mosby Co.
were seen at the University of Missouri Medical Cen- ter. Four of them underwent operative treatment. There were four male patients and one female patient, whose ages ranged from 10 weeks to 14 years. Two patients required two separate operations each to relieve symp- toms completely. Brief case presentations follow.
CAS E I. A 10-week-old infant had cyanosis, wheezing, and repeated choking spells. Cardiac catheterization was per- formed. Tetralogy of Fallot, right aortic arch, and aberrant left subclavian artery were diagnosed. Barium swallow dis- closed an indentation at the retroesophageal area. Left thoracotomy was performed because of the patient's respi- ratory distress. At operation, a retroesophageal aberrant left subclavian artery with left ligamentum arteriosum forming a constricting vascular ring was found. The ligamentum ar- teriosum was divided, and the aberrant subclavian artery was suspended to the paravertebral fascia. After operation, respi- ratory distress was markedly improved, but the patient con- tinued to have choking spells and was unable to swallow solid food at the age of 2 years. Barium swallow was repeated and marked retroesophageal indentation was demonstrated again. Right thoracotomy with division of the aberrant left subcla- vian artery was carried out. The postoperative course was uneventful. He was followed for 2 years. No arm weakness or dysphagia was noted. When he was 4 years of age, correction of tetralogy of Fallot was carried out. He died suddenly after onset of ventricular fibrillation 24 hours after operation.
CASE 2. A l6-month-old boy presented with stridor, wheezing, feeding difficulty, and heart murmurs. Barium swallow demonstrated a retroesophageal concavity (Fig. I). Cardiac catheterization disclosed a small ventricular septal defect with left-to-right shunt and a Qp/Qs of 1.21. Right aortic arch with aberrant left subclavian artery arising from a
237
Thoracic and Cardiovascular
Surgery
Fig. 1. Esophagogram from Case 2 before division of ligamentum arteriosum. A, Anteroposterior view. B. Lateral view.
small Kommerell diverticulum was delineated also. Because of severe respiratory distress, a left thoracotomy with division of ligamentum arteriosum was performed. After operation, respiratory distress was alleviated, but as the child grew, swallowing difficulty became more evident. Marked ret- roesophageal concavity was shown again by repeated barium swallow (Fig. 2). When he was 7 years of age, right thoracotomy with division of the retroesophageal aberrant subclavian artery at its origin was performed. Complete relief from dysphagia was achieved.
CAS E 3. An 8-month-old infant was evaluated for wheez- ing, repeated respiratory tract infection, slow feeding, and choking spells. A Grade 2/6 systolic murmur with fixed Sz splitting was heard. A barium swallow displayed ret- roesophageal indentation. Cardiac catheterization demon- strated an atrial septal defect, right aortic arch, and aberrant left subclavian artery arising from a large Kommerell's diver- ticulum. Stenosis of the left subclavian artery at its origin was well demonstrated (Fig. 3). Through a right thoracotomy, division and suture of the left subclavian artery, division of ligamentum arteriosum, and excision of Kommerell's diver- ticulum were carried out. Later follow-up revealed an excel- lent result. Closure of atrial septal defect is planned in the near future.
CAS E 4. An ll-year-old girl had dysphagia related to swal- lowing solid food. She has been eating strained food since her infancy. A severe choking spell after eating prompted her seeking medical advice. Barium swallow revealed a large retroesophageal concavity (Fig. 4). Aortogram clearly de-
fined a right aortic arch and an aberrant left subclavian artery arising from the aorta with a large Kommerell diverticulum (Fig. 5). A right thoracotomy was carried out and the ret- roesophageal aberrant left subclavian artery was divided. The large Kommerell diverticulum was excised. The ligamentum arteriosum could not be identified from the right side. Postoperative barium swallow, however, demonstrated complete resolution of the esophageal compression (Fig. 6).
CASE 5. A 17-year-old boy had tetralogy of Fallot cor- rected elsewhere. He was referred to the University of Missouri Medical Center because of dysphagia with solid food and congestive heart failure. A residual ventricular sep- tal defect with a left-to-right shunt and a Qp/Qs of 2.51, as well as a right aortic arch with aberrant left subclavian artery, were demonstrated. Esophagogram revealed marked indenta- tion. Patch closure of the recurrent ventricular septal defect was carried out. He had an uneventful postoperative course. While awaiting for right thoracotomy and division of the aberrant left subclavian artery, a brain abscess developed and he died at a local hospital.
A summary of these patients' profiles and operative findings and results is presented in Table I.
Comment
The anatomic pattern of a complete vascular ring formed by right aortic arch, aberrant retroesophageal left subclavian artery, left ligamentum arteriosum, and
Volume 75
Number 2
February. 1978 Repair of right aortic arch 2 3 9
Fig. 2. Esophagogram from Case 2 before second operation. Esophageal compression persistsdespite previous division of ligamentum arteriosum. A. Anteroposterior view. B. Lateral view.
pulmonary artery falls under Class IIIB of Stewart, Kincaid , and Edward's' classification of aortic arch malformations. This is a relativel y rare variety, with only a few cases having been reported in several large series of vascular ring. 2- 8 The constricting vascular ring is believed to be responsible for respiratory and swallowing disturbance in all of our patients. In addi- tion, we believe a large Komrnerell " diverticulum in Cases 3 and 4 caused a significant additional compres- sion of the esophagus.I? Actually, this diverticulum, rather than the vascular ring, was the major cause of esophageal compression in Case 4. This was depicted by the anteroposterior projection of the esophagogram and was verified during the operat ion (Fig. 4) . In Case 3, a marked coarctation at the junction of the Kom- merell diverticulum and the aberrant retroesophageal left subclavian artery was demonstrated (Fig. 3). This may represent an intermediate anatomic pattern be- tween Classes IIIB and IIIC (right aortic arch with iso- lation of left subclavian artery). As indicated by Stewart , Kincaid, and Edwards I in their monograph , the right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum resulted from an inde- pendent error in the embryologic development of the aortic arch system. Thus its presentation with other cardiac anomalies is coincidental. However, though the number is small and does not allow specific conclu- sions, four of the five patients in our series had associ-
Fig. 3. Aortogram of Case 3. A large Kommerell diver- ticulum from where the aberrantleft subclavian artery arises. Marked coarctation is presentat its junction. This mayrepre- sent an intermediate anatomic pattern between Stewart's Classes IllS and mc.
ated congenital cardiac defects-two with tetralogy of Fallot, one with a small ventricul ar septal defect, and one with an atrial septal defect. We believe that the surgical repair of these associated defects should be done at a different date.
The most striking symptom calling for urgent surgi- cal intervention in infancy is respiratory distress, manifested by stridor, wheezing, and respiratory tract
240 lung et al. The Journal of
Thoracic and Cardiovascular
Surgery
Fig. 4. Esophagogram from Case 4. A. Anteroposterior view (spot). B, Lateral view. The compression of the esophagus was due to a large Kommerell diverticulum. In anteroposterior view the length of compression corresponds to the diameter of the Kommerell diverticulum.
Fig. 5. Aortogram from Case 4, illustrating the aberrant left subclavian artery arising from a large Kommerell diver- ticulum.
infection (Cases I, 2, and 3). In older children dys- phagia becomes a dominant symptom (Case 1,2,3,4, and 5). Endoscopy was not included in the investiga- tion , but an esophagogram was found to be a most useful diagnostic aid. Aortography was essential for delineation of the anatomic pattern. The differential diagnosis includes Class IlIA (right aortic arch with mirror-image branching) and a double aortic arch with
an atretic segment between left lower carotid and left subclavian artery. Meticulous dissection and identify- ing the anatomy accurately are essential prerequisites for successful surgical management.
Left thoracotomy with division of the ligamentum arteriosum has been employed with success by some authors." 6 Our experience indicates that this approach possibly could relieve respiratory distress in infancy, but relief of dysphagia was quite insufficient, as dem- onstrated by Cases I and 2. Esophagograms obtained before and after division of the ligamentum arteriosum in Case 2 (Fig. I and 2) clearl y demonstrated the failure in alleviating the esophageal compression . Both of these patients required a second operation for relief of dysphagia . Similar experience has been reported by Hallman and Cooley.4 Retrospectively, had division of the liagmentum arteriosum as well as division of the first portion of the aberrant left subclavian artery been carried out at the first operation, as described by other authors,": 7. 8 the second operation could have been avoided.
In four operated patients, we approached the lesion through a posterolateral right thoracotomy via the fourth intercostal space. Division and suturing of the aberrant left subclavian artery at its origin and excision
Volume 75 Number 2 February. 1978
Repair of right aortic arch 2 4 1
Fig. 6. Postoperative esophagogram from Case 4 showing complete relief of esophageal compression after division of aberrant left subclavian artery and excision of Kommerell 's diverticulum through a right thoracotomy. A. Anteroposterior view. B. Lateral view.
of Kommerell's diverticulum constituted the major part of the operative procedure . The ligamentum arteriosum was divided if it was identified . Accessibility to the aortic arch and descending aorta and exposure of Kommerell's diverticulum and the origin of the aber- rant left subclavian artery are excellent. We believe the right-sided approach should be chosen when Kom- merell's diverticulum is responsible for major esophageal compression and its excision is mandato- ry.9. 10 Resection of this aortic diverticulum is advisa- ble also, because aneurysmal formation from Kom- rnerell's diverticulum in the later life has been described." Arguments against the "right" approach have sound clinical and theoretical bases. Theoretical- Iy, interruption of major pulsatile flow to the left aber- rant subclavian artery could subject the patient to the danger of ischemic gangrene, intermittent claudication of the left upper extremity, or the development of sub- clavian steal syndrome.'! In the clinical setting these complications were observed after Blalock-Taussig anastomosis .13
To our knowledge, no cases of severe ischemia or definite subclavian steal syndrome have been reported
after division of the subclavian artery at its origin . No such complication was observed in our patients during the relatively short periods of observation. Long-term follow-up seems necessary for further evaluation. Nikaidoh, Riker, and Idriss" pointed out two of their 11 patients followed 20 years after division of the subcla- vian artery at its origin had clinical symptoms sugges- tive of subclavian steal. Another patient described by Shuford, Sybers, and Schlant," a young man with right aortic arch with isolation of left subclavian artery (Class IIIC) , was free from symptoms until his late teens . Subclavian steal syndrome became apparent in his early 20 's . Hemodynamically, this anomaly is equivalent to Class IIIB patients treated with division of the subclavian artery alone. Thus subclavian artery steal syndrome could make its appearance later in life. This potential hazard may be prevented by reanas- tomosis of the divided left subclavian artery to the left common carotid artery or aorta. This can be accom- plished by a left thoracotomy which also would allow division of the ligamentum arteriosum and the first part of the aberrant subclavian. 4 . 1. 8
However, exposure and resection of Kommerell's
The Journal of
Table I. Patients' profiles and operative results
Case Sex Onset of symptoms Associated anomaly Operative findings Operation Result
M 10 wks. Stridor, Tetralogy of Fallot Right aortic arch, right First operation: Divi- Respiratory distress wheezing, descending aorta, sion of ligamentum improved but cyanosis left ligamentum ar- arteriosum alone dysphagia per-
teriosum, left retro- through left thor- sisted esophageal sub- acotomy clavian artery
Second operation: Di- Complete relief of vision of aberrant dysphagia left subclavian ar- tery through right thoracotomy
2 M 16 mo. Stridor Small VSD Right aortic arch, right First operation: Divi- Respiratory distress descending aorta, sion of ligamentum improved but aberrant retro- arteriosum through dysphagia per- esophageal left sub- left thoracotomy sisted clavian artery, left ligamentum arterio- Second operation: sum, small Kom- Division of aberrant merell diverticulum left subclavian ar-
tery; excision of Komrnerell's diver- ticulum through right thoracotomy
3 M 8 mo. Wheezing, re- ASD Right aortic arch, right Division of aberrant Complete relief of peated res- descending aorta, left subclavian ar- dysphagia piratory in- large Kommerell di- tery through right fection, verticulum, aberrant thoracotomy choking retroesophageal left spell subclavian artery
with coarctation at its origin, left liga- mentum arteriosum
4 F 2 yr. Dysphagia on None Right aortic arch, Division of aberrant Complete relief of ingesting right descending left subclavian ar- dysphagia solid food aorta; aberrant left tery through right
subclavian artery, thoracotomy large Kommerell diverticulum
5 M 17 yr. Cyanosis, Tetralogy of Fallot None None Died of brain ab- dysphagia scess while
awaiting opera- tion
Legend: VSD, Ventricular septal defect. ASD, Atrial septal defect.
diverticulum causing esophageal obstruction may be difficult through this approach. A right thoracotomy would be the procedure of choice in such a situation and may be combined with a separate cervical incision for establishing a left subclavian to common carotid anastomosis.
Conclusion
1. Division of the ligamentum arteriosum alone may be sufficient to alleviate tracheal constriction, but it is insufficient to relieve esophageal compression.
2. Right thoracotomy with division of the aberrant subclavian artery is the approach of choice if Kom-
mereU's diverticulum is responsible for esophageal compression, and its excision is mandatory.
3. Left thoracotomy with division of the aberrant subclavian artery and ligamentum arteriosum and re- establishment of direct flow to the left subclavian artery are preferred if no large Kommerell diverticulum is present.
REFERENCES
Stewart.T. R., Kincaid, O. W., and Edwards, J. E.: An Atlas of Vascular Rings and Related Malformations of Aortic Arch System, Springfield, III., 1964, Charles C Thomas, Publisher.
Volume 75 Number 2 February, 1978
2 Wychalis, A. R., Kincaid, O. W., and Danielsen, G. K.: Congenital Vascular Ring: Surgical Considerations and Results of Operation, Mayo Clin. Proc. 46: 182, 1971.
3 Nikaidoh, H., Riker, W. L., and Idriss, F. S.: Surgical Management of "Vascular Rings," Arch. Surg. 105: 327, 1972.
4 Hallman, G. L., and Cooley, D. A.: Congenital Aortic Vascular Rings: Surgical Considerations, Arch. Surg. 88: 666, 1964.
5 Lincoln, J. C. R., Deverall, P. B., Stark, J., Aberdeen, E., and Waterston, D. 1.: Vascular Anomalies Compress- ing the Esophagus and Trachea, Thorax 24: 295, 1969.
6 Tucker, B. L., Meyer, B. W., Lindesmith, G. G., Stiles, Q. R., and Jones, J. c.: Congenital Aortic Vascular Ring, Arch. Surg. 99: 521, 1969.
7 Gross, R. E., and Neuhauser, E. B. D.: Compression of the Trachea or Esophagus by Vascular Anomalies: Surgi- cal Therapy of Forty Cases, Pediatrics 7: 69, 1951.
8 Mahoney, B. E., and Manning, J. A.: Congenital Ab- normalities of the Aortic Arch, Surgery 55: I, 1964.
9 Kommerell, B.: Verlagerung des Osophagus durch eine
Repair of right aortic arch 2 4 3
Abnorm Verlaufende Arteria Subclavia Dextra (Arteria Lusoria), Fortsch Geb. Rontgenstr. 54: 590, 1936.
10 Shannon, J. M.: Aberrant Right Subclavian Artery With Kommerell's Diverticulum, J. THORAC. CARDIOVASC. SURG. 41: 408, 1961.
II Campbell, C. F.: Repair of an Aneurysm of an Aberrant Retroesophageal Right Subclavian Artery Arising From Kommerell's Diverticulum, J. THORAe. CARDIOVASe. SURG. 62: 330, 1971.
12 Reirich, M., Holling, H. E., Roberts, B., and Toole, J. F.: Reversal of Blood Flow Through Vertebral Artery and its Effect on Cerebral Circulation, N. Eng!. J. Med. 265: 878, 1961.
Five cases of right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum, with or without Kommerell's diverticulum, are presented. Either right or left thoracotomy with division of the aberrant retroesophageal left subclavian artery can be accomplished with good results. However, a right thoracotomy is recommended in the presence of a large Kommerell diverticulum because such a diverticulum should be excised. The development of a subclavian steal syndrome later in life may be prevented by reimplantation qf the divided left subclavian artery into the aorta or the left common carotid artery.
r. Y. lung, M.D., Carl H. Almond, M.D., Salim B. Saab, M.D., and Zudhi Lababidi, M.D.,
Columbia, Mo.
The diagnosis and management of the anomalies of the aortic arch and its branches have been well estab- lished. Some of these cases are asymptomatic-either diagnosed at autopsy or as an incidental finding during the angiographic studies for other cardiac defects. However, vascular rings can cause symptoms by con- striction or compression of the trachea or esophagus. In patients with the latter symptom, accurate diagnosis is essential for planning the approach of a correct opera- tive treatment. The literature is abundant on these sub- jects. However, specific attention to the anomaly of right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum with or without Korn- merell's diverticulum is sporadic, and surgical ap- proaches are not defined clearly. It is the purpose of this communication to report our experience with five such patients and to consider the surgical approach in their management.
Case presentations
From 1971 through May, 1977, five patients with right aortic arch and aberrant left subclavian artery
From the Section of Thoracic and Cardiovascular Surgery, Univer- sity of Missouri Medical Center, Columbia, Mo.
Received for publication June I, 1977.
Accepted for publication July 15, 1977.
Address for reprints: Carl H. Almond, M. D., Professor and Chief, Section of Thoracic and Cardiovascular Surgery, University of Missouri Medical Center, Columbia, Mo. 65201.
0022-5223/78/0275-0237$00.70/0 © 1978 The C. V. Mosby Co.
were seen at the University of Missouri Medical Cen- ter. Four of them underwent operative treatment. There were four male patients and one female patient, whose ages ranged from 10 weeks to 14 years. Two patients required two separate operations each to relieve symp- toms completely. Brief case presentations follow.
CAS E I. A 10-week-old infant had cyanosis, wheezing, and repeated choking spells. Cardiac catheterization was per- formed. Tetralogy of Fallot, right aortic arch, and aberrant left subclavian artery were diagnosed. Barium swallow dis- closed an indentation at the retroesophageal area. Left thoracotomy was performed because of the patient's respi- ratory distress. At operation, a retroesophageal aberrant left subclavian artery with left ligamentum arteriosum forming a constricting vascular ring was found. The ligamentum ar- teriosum was divided, and the aberrant subclavian artery was suspended to the paravertebral fascia. After operation, respi- ratory distress was markedly improved, but the patient con- tinued to have choking spells and was unable to swallow solid food at the age of 2 years. Barium swallow was repeated and marked retroesophageal indentation was demonstrated again. Right thoracotomy with division of the aberrant left subcla- vian artery was carried out. The postoperative course was uneventful. He was followed for 2 years. No arm weakness or dysphagia was noted. When he was 4 years of age, correction of tetralogy of Fallot was carried out. He died suddenly after onset of ventricular fibrillation 24 hours after operation.
CASE 2. A l6-month-old boy presented with stridor, wheezing, feeding difficulty, and heart murmurs. Barium swallow demonstrated a retroesophageal concavity (Fig. I). Cardiac catheterization disclosed a small ventricular septal defect with left-to-right shunt and a Qp/Qs of 1.21. Right aortic arch with aberrant left subclavian artery arising from a
237
Thoracic and Cardiovascular
Surgery
Fig. 1. Esophagogram from Case 2 before division of ligamentum arteriosum. A, Anteroposterior view. B. Lateral view.
small Kommerell diverticulum was delineated also. Because of severe respiratory distress, a left thoracotomy with division of ligamentum arteriosum was performed. After operation, respiratory distress was alleviated, but as the child grew, swallowing difficulty became more evident. Marked ret- roesophageal concavity was shown again by repeated barium swallow (Fig. 2). When he was 7 years of age, right thoracotomy with division of the retroesophageal aberrant subclavian artery at its origin was performed. Complete relief from dysphagia was achieved.
CAS E 3. An 8-month-old infant was evaluated for wheez- ing, repeated respiratory tract infection, slow feeding, and choking spells. A Grade 2/6 systolic murmur with fixed Sz splitting was heard. A barium swallow displayed ret- roesophageal indentation. Cardiac catheterization demon- strated an atrial septal defect, right aortic arch, and aberrant left subclavian artery arising from a large Kommerell's diver- ticulum. Stenosis of the left subclavian artery at its origin was well demonstrated (Fig. 3). Through a right thoracotomy, division and suture of the left subclavian artery, division of ligamentum arteriosum, and excision of Kommerell's diver- ticulum were carried out. Later follow-up revealed an excel- lent result. Closure of atrial septal defect is planned in the near future.
CAS E 4. An ll-year-old girl had dysphagia related to swal- lowing solid food. She has been eating strained food since her infancy. A severe choking spell after eating prompted her seeking medical advice. Barium swallow revealed a large retroesophageal concavity (Fig. 4). Aortogram clearly de-
fined a right aortic arch and an aberrant left subclavian artery arising from the aorta with a large Kommerell diverticulum (Fig. 5). A right thoracotomy was carried out and the ret- roesophageal aberrant left subclavian artery was divided. The large Kommerell diverticulum was excised. The ligamentum arteriosum could not be identified from the right side. Postoperative barium swallow, however, demonstrated complete resolution of the esophageal compression (Fig. 6).
CASE 5. A 17-year-old boy had tetralogy of Fallot cor- rected elsewhere. He was referred to the University of Missouri Medical Center because of dysphagia with solid food and congestive heart failure. A residual ventricular sep- tal defect with a left-to-right shunt and a Qp/Qs of 2.51, as well as a right aortic arch with aberrant left subclavian artery, were demonstrated. Esophagogram revealed marked indenta- tion. Patch closure of the recurrent ventricular septal defect was carried out. He had an uneventful postoperative course. While awaiting for right thoracotomy and division of the aberrant left subclavian artery, a brain abscess developed and he died at a local hospital.
A summary of these patients' profiles and operative findings and results is presented in Table I.
Comment
The anatomic pattern of a complete vascular ring formed by right aortic arch, aberrant retroesophageal left subclavian artery, left ligamentum arteriosum, and
Volume 75
Number 2
February. 1978 Repair of right aortic arch 2 3 9
Fig. 2. Esophagogram from Case 2 before second operation. Esophageal compression persistsdespite previous division of ligamentum arteriosum. A. Anteroposterior view. B. Lateral view.
pulmonary artery falls under Class IIIB of Stewart, Kincaid , and Edward's' classification of aortic arch malformations. This is a relativel y rare variety, with only a few cases having been reported in several large series of vascular ring. 2- 8 The constricting vascular ring is believed to be responsible for respiratory and swallowing disturbance in all of our patients. In addi- tion, we believe a large Komrnerell " diverticulum in Cases 3 and 4 caused a significant additional compres- sion of the esophagus.I? Actually, this diverticulum, rather than the vascular ring, was the major cause of esophageal compression in Case 4. This was depicted by the anteroposterior projection of the esophagogram and was verified during the operat ion (Fig. 4) . In Case 3, a marked coarctation at the junction of the Kom- merell diverticulum and the aberrant retroesophageal left subclavian artery was demonstrated (Fig. 3). This may represent an intermediate anatomic pattern be- tween Classes IIIB and IIIC (right aortic arch with iso- lation of left subclavian artery). As indicated by Stewart , Kincaid, and Edwards I in their monograph , the right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum resulted from an inde- pendent error in the embryologic development of the aortic arch system. Thus its presentation with other cardiac anomalies is coincidental. However, though the number is small and does not allow specific conclu- sions, four of the five patients in our series had associ-
Fig. 3. Aortogram of Case 3. A large Kommerell diver- ticulum from where the aberrantleft subclavian artery arises. Marked coarctation is presentat its junction. This mayrepre- sent an intermediate anatomic pattern between Stewart's Classes IllS and mc.
ated congenital cardiac defects-two with tetralogy of Fallot, one with a small ventricul ar septal defect, and one with an atrial septal defect. We believe that the surgical repair of these associated defects should be done at a different date.
The most striking symptom calling for urgent surgi- cal intervention in infancy is respiratory distress, manifested by stridor, wheezing, and respiratory tract
240 lung et al. The Journal of
Thoracic and Cardiovascular
Surgery
Fig. 4. Esophagogram from Case 4. A. Anteroposterior view (spot). B, Lateral view. The compression of the esophagus was due to a large Kommerell diverticulum. In anteroposterior view the length of compression corresponds to the diameter of the Kommerell diverticulum.
Fig. 5. Aortogram from Case 4, illustrating the aberrant left subclavian artery arising from a large Kommerell diver- ticulum.
infection (Cases I, 2, and 3). In older children dys- phagia becomes a dominant symptom (Case 1,2,3,4, and 5). Endoscopy was not included in the investiga- tion , but an esophagogram was found to be a most useful diagnostic aid. Aortography was essential for delineation of the anatomic pattern. The differential diagnosis includes Class IlIA (right aortic arch with mirror-image branching) and a double aortic arch with
an atretic segment between left lower carotid and left subclavian artery. Meticulous dissection and identify- ing the anatomy accurately are essential prerequisites for successful surgical management.
Left thoracotomy with division of the ligamentum arteriosum has been employed with success by some authors." 6 Our experience indicates that this approach possibly could relieve respiratory distress in infancy, but relief of dysphagia was quite insufficient, as dem- onstrated by Cases I and 2. Esophagograms obtained before and after division of the ligamentum arteriosum in Case 2 (Fig. I and 2) clearl y demonstrated the failure in alleviating the esophageal compression . Both of these patients required a second operation for relief of dysphagia . Similar experience has been reported by Hallman and Cooley.4 Retrospectively, had division of the liagmentum arteriosum as well as division of the first portion of the aberrant left subclavian artery been carried out at the first operation, as described by other authors,": 7. 8 the second operation could have been avoided.
In four operated patients, we approached the lesion through a posterolateral right thoracotomy via the fourth intercostal space. Division and suturing of the aberrant left subclavian artery at its origin and excision
Volume 75 Number 2 February. 1978
Repair of right aortic arch 2 4 1
Fig. 6. Postoperative esophagogram from Case 4 showing complete relief of esophageal compression after division of aberrant left subclavian artery and excision of Kommerell 's diverticulum through a right thoracotomy. A. Anteroposterior view. B. Lateral view.
of Kommerell's diverticulum constituted the major part of the operative procedure . The ligamentum arteriosum was divided if it was identified . Accessibility to the aortic arch and descending aorta and exposure of Kommerell's diverticulum and the origin of the aber- rant left subclavian artery are excellent. We believe the right-sided approach should be chosen when Kom- merell's diverticulum is responsible for major esophageal compression and its excision is mandato- ry.9. 10 Resection of this aortic diverticulum is advisa- ble also, because aneurysmal formation from Kom- rnerell's diverticulum in the later life has been described." Arguments against the "right" approach have sound clinical and theoretical bases. Theoretical- Iy, interruption of major pulsatile flow to the left aber- rant subclavian artery could subject the patient to the danger of ischemic gangrene, intermittent claudication of the left upper extremity, or the development of sub- clavian steal syndrome.'! In the clinical setting these complications were observed after Blalock-Taussig anastomosis .13
To our knowledge, no cases of severe ischemia or definite subclavian steal syndrome have been reported
after division of the subclavian artery at its origin . No such complication was observed in our patients during the relatively short periods of observation. Long-term follow-up seems necessary for further evaluation. Nikaidoh, Riker, and Idriss" pointed out two of their 11 patients followed 20 years after division of the subcla- vian artery at its origin had clinical symptoms sugges- tive of subclavian steal. Another patient described by Shuford, Sybers, and Schlant," a young man with right aortic arch with isolation of left subclavian artery (Class IIIC) , was free from symptoms until his late teens . Subclavian steal syndrome became apparent in his early 20 's . Hemodynamically, this anomaly is equivalent to Class IIIB patients treated with division of the subclavian artery alone. Thus subclavian artery steal syndrome could make its appearance later in life. This potential hazard may be prevented by reanas- tomosis of the divided left subclavian artery to the left common carotid artery or aorta. This can be accom- plished by a left thoracotomy which also would allow division of the ligamentum arteriosum and the first part of the aberrant subclavian. 4 . 1. 8
However, exposure and resection of Kommerell's
The Journal of
Table I. Patients' profiles and operative results
Case Sex Onset of symptoms Associated anomaly Operative findings Operation Result
M 10 wks. Stridor, Tetralogy of Fallot Right aortic arch, right First operation: Divi- Respiratory distress wheezing, descending aorta, sion of ligamentum improved but cyanosis left ligamentum ar- arteriosum alone dysphagia per-
teriosum, left retro- through left thor- sisted esophageal sub- acotomy clavian artery
Second operation: Di- Complete relief of vision of aberrant dysphagia left subclavian ar- tery through right thoracotomy
2 M 16 mo. Stridor Small VSD Right aortic arch, right First operation: Divi- Respiratory distress descending aorta, sion of ligamentum improved but aberrant retro- arteriosum through dysphagia per- esophageal left sub- left thoracotomy sisted clavian artery, left ligamentum arterio- Second operation: sum, small Kom- Division of aberrant merell diverticulum left subclavian ar-
tery; excision of Komrnerell's diver- ticulum through right thoracotomy
3 M 8 mo. Wheezing, re- ASD Right aortic arch, right Division of aberrant Complete relief of peated res- descending aorta, left subclavian ar- dysphagia piratory in- large Kommerell di- tery through right fection, verticulum, aberrant thoracotomy choking retroesophageal left spell subclavian artery
with coarctation at its origin, left liga- mentum arteriosum
4 F 2 yr. Dysphagia on None Right aortic arch, Division of aberrant Complete relief of ingesting right descending left subclavian ar- dysphagia solid food aorta; aberrant left tery through right
subclavian artery, thoracotomy large Kommerell diverticulum
5 M 17 yr. Cyanosis, Tetralogy of Fallot None None Died of brain ab- dysphagia scess while
awaiting opera- tion
Legend: VSD, Ventricular septal defect. ASD, Atrial septal defect.
diverticulum causing esophageal obstruction may be difficult through this approach. A right thoracotomy would be the procedure of choice in such a situation and may be combined with a separate cervical incision for establishing a left subclavian to common carotid anastomosis.
Conclusion
1. Division of the ligamentum arteriosum alone may be sufficient to alleviate tracheal constriction, but it is insufficient to relieve esophageal compression.
2. Right thoracotomy with division of the aberrant subclavian artery is the approach of choice if Kom-
mereU's diverticulum is responsible for esophageal compression, and its excision is mandatory.
3. Left thoracotomy with division of the aberrant subclavian artery and ligamentum arteriosum and re- establishment of direct flow to the left subclavian artery are preferred if no large Kommerell diverticulum is present.
REFERENCES
Stewart.T. R., Kincaid, O. W., and Edwards, J. E.: An Atlas of Vascular Rings and Related Malformations of Aortic Arch System, Springfield, III., 1964, Charles C Thomas, Publisher.
Volume 75 Number 2 February, 1978
2 Wychalis, A. R., Kincaid, O. W., and Danielsen, G. K.: Congenital Vascular Ring: Surgical Considerations and Results of Operation, Mayo Clin. Proc. 46: 182, 1971.
3 Nikaidoh, H., Riker, W. L., and Idriss, F. S.: Surgical Management of "Vascular Rings," Arch. Surg. 105: 327, 1972.
4 Hallman, G. L., and Cooley, D. A.: Congenital Aortic Vascular Rings: Surgical Considerations, Arch. Surg. 88: 666, 1964.
5 Lincoln, J. C. R., Deverall, P. B., Stark, J., Aberdeen, E., and Waterston, D. 1.: Vascular Anomalies Compress- ing the Esophagus and Trachea, Thorax 24: 295, 1969.
6 Tucker, B. L., Meyer, B. W., Lindesmith, G. G., Stiles, Q. R., and Jones, J. c.: Congenital Aortic Vascular Ring, Arch. Surg. 99: 521, 1969.
7 Gross, R. E., and Neuhauser, E. B. D.: Compression of the Trachea or Esophagus by Vascular Anomalies: Surgi- cal Therapy of Forty Cases, Pediatrics 7: 69, 1951.
8 Mahoney, B. E., and Manning, J. A.: Congenital Ab- normalities of the Aortic Arch, Surgery 55: I, 1964.
9 Kommerell, B.: Verlagerung des Osophagus durch eine
Repair of right aortic arch 2 4 3
Abnorm Verlaufende Arteria Subclavia Dextra (Arteria Lusoria), Fortsch Geb. Rontgenstr. 54: 590, 1936.
10 Shannon, J. M.: Aberrant Right Subclavian Artery With Kommerell's Diverticulum, J. THORAC. CARDIOVASC. SURG. 41: 408, 1961.
II Campbell, C. F.: Repair of an Aneurysm of an Aberrant Retroesophageal Right Subclavian Artery Arising From Kommerell's Diverticulum, J. THORAe. CARDIOVASe. SURG. 62: 330, 1971.
12 Reirich, M., Holling, H. E., Roberts, B., and Toole, J. F.: Reversal of Blood Flow Through Vertebral Artery and its Effect on Cerebral Circulation, N. Eng!. J. Med. 265: 878, 1961.
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