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Tandem coarctation of the thoracic aorta
J THoRAc CARDIOVASC SURG 82:592-594, 1981
Tandem coarctation of the thoracic aorta An unusual congenital anomaly A coarctation involving the aortic isthmus, with hypoplasia of the upper aortic segment, was satisfactorily repaired in a 15-year-o/d girl. The technique used was subclavian flap arterioplasty followed by reimplantation of the distal subclavian artery into the left carotid artery. Surprisingly weak femoral pulses were noted postoperatively and prompted a control angiogram. This showed an intraluminal diaphragm 2 em below the site of the previous repair, with a 100 mm Hg gradient. At reoperation, a centrally perforated fibrous diaphragm was excised, and the aorta was enlarged with a small Dacron patch. The postoperative course was uneventful.
Ulrik Hvass, M.D., Jean Langlois, M.D.,* Plessis Robinson, France, and P. E. Valere, M.D.,** Paris, France
Tandem coarctation of the aorta is an unusual congenital anomaly. The following report describes our management of this anomaly in a 15-year-old girl.
Case report A 15-year-old Guadeloupean girl was admitted for repair of coarctation of the thoracic aorta. She had been asymptomatic until shortly before admission, when she started experiencing malaise and visual impairment. Physical examination showed a well-developed girl. The arm blood pressure was 170/ 110 mm Hg and femoral pulses were absent. The chest roentgenogram showed no cardiac enlargement. Costal vascular markings were noted. The electrocardiogram revealed slight left ventricular strain. The diagnosis was confirmed by a cineangiocardiogram (Fig. I) with contrast media injected into the ascending aorta. Coarctation of the aortic isthmus was shown, with a fairly long, hypoplastic precoarctation segment. Collateral circulation was well developed. Initial operation. Operation was performed in November, 1980, via a left lateral thoracotomy in the bed of the fourth rib. After controlling the different segments of the aorta, we believed that resection of the coarctation would require an
From Centre Chirurgical Marie Lannelongue, 92350 Plessis Robinson, France, and Hopital Bichat, Paris, France. Received for publication Jan. 7, 1981. Accepted for publication March 24, 1981. Addressfor reprints: Dr. Ulrik Hvass, Centre Chirurgical Marie Lannelongue, 133 Av. de la Resistance, Plessis Robinson 92350, France. *Professorof HeartSurgery, CentreChirurgical MarieLannelongue. **Professorof Cardiology, Hopital Bichat.
592
interposition Dacron graft. The hypoplastic upper aortic segment was fairly long, and the lower segment was anchored by several large intercostal arteries, which would have to be ligated. To avoid using prosthetic material, we chose to use a subclavian flap arterioplasty and reimplant the distal subclavian artery into the left carotid artery, as previously reported in patients with long segment or recurrent coarctations. J, 2 The subclavian artery was large and long and was divided 1.5 ern proximal to the vertebral artery. It then was opened along its lateral margin, the opening being extended across the coarctation as originally described by Waldhausen and Nahrwold. a An intraluminal diaphragm was excised and the flap was sewn with a running 5-0 Prolene suture. After being unclamped, the aorta had a perfect contour with no gradient between the horizontal arch and the aorta just beyond the suture line. An anastomosis of the distal subclavian to left carotid artery then was performed via the same thoracotomy. Postoperative course. The immediate postoperative course was disappointing because of persistent upper extremity hypertension and unexpectedly feeble femoral pulses. Thrombosis, dissection, and an associated abdominal coarctation were discussed as explanatory possibilities. A control angiogram showed a perfectly well enlarged aortic isthmus and a nearly complete intraluminal diaphragm approximately 2 em below the previous repair (Fig. 2). At this site, a 100 mm Hg systolic gradient was recorded. Reoperation. Reoperation was accepted 3 weeks after the first operation. Intraoperative pressure recordings confirmed the absence of a gradient immediately distal to the subclavian flap, but showed a 90 mm Hg gradient a few centimeters further down. The exterior aspect of the aorta was normal except for a discrete indentation at the site of the pressure gradient. Lateral clamping of the aorta allowed a longitudinal opening. A fibrous diaphragm I mm thick was found with a central perforation 3 mm in diameter. The intima was intact on each surface of the diaphragm. The diaphragm was ex-
0022-5223/81/100592+03$00.30/0 © 1981 The C. V. Mosby Co.
Volume 82 Number 4 October, 1981
Tandem coarctation of thoracic aorta
593
tlv
Fig. I. Preoperative angiogram: The first coarctation is obvious, with hypoplasia of the upper aortic segment. The diaphragm of the second coarctation is faintly outlined .
v
Fig. 2. Postoperative control angiogram: The aortic isthmus is correctly enlarged, but there is a nearly complete intralum inal diaphragm . cised and the aorta enlarged with a small Dacron patch (Fig . 3). After the aorta was undamped . a 12 mm Hg systolic gradient was demonstrated between the right radial artery and the aorta ju st distal to the patch . The postoperative course was uneventful. No gradient was detected by cuff and Doppler measurements between the upper and lower limbs. nor between the left and right arms. Histologic stud y of the fragment s excised at both operations was not rewarding . since no aortic wall had been excised .
Discussion
Tandem coarctation obviously is a very unusual anomaly. Reports of atypical coarctations do not mention such a finding. 4- 8 The diagnosis was missed preoperatively, mainly because the first coarctation with segmental hypoplasia was so obvious that there was no thought of looking for another lesion. The second reason for missing the diag-
The Journal of
594
Hvass, Langlois, Valere
Thoracic and Cardiovascular Surgery
v
",
\
subclavian flap could have been tailored a bit longer so as to overlap both lesions. The subclavian-carotid artery anastomosis then would have been easier to accomplish via a cervical approach. A similar case might not be encountered again for a long time, but it is worthwhile to remember that one coarctation can hide another, even at the thoracic level. \
\
\
'--a
"
Appreciation is extended to Dr. Marthe Gautier, Maitre de Recherche, I.N.S.E.R.M., Hopital Bicetre, Paris, for reviewing the histology.
2
3 Fig. 3. Schematic drawing of the two-staged operation, which comprises a subclavian flap arterioplasty with the carotid reimplantation of the distal subclavian segment and a small patch enlargement just below the first repair.
4
5 nosis was the poor opacification of the aorta beyond the first coarctation. However, retrospective analysis of the preoperative cineangiocardiogram showed that a correct diagnosis should have been possible. The type of repair used for the initial operation in this patient deserves several remarks. It demonstrates our reluctance to use prosthetic material in young patients; reported results as well as our own experience show long-term complications such as aneurysms, infection, or thrombosis." As previously mentioned, we already had used the subclavian flap procedure for long segment coarctations and recurrent coarctations. Direct reimplantation of the distal subclavian with the left vertebral artery into the left carotid artery avoids shortterm 10 and long-term ischemia of the left arm as well as a subclavian steal syndrome. II Nevertheless, at reoperation we used a small Dacron patch to enlarge the aorta after resection of the second diaphragm. Perhaps an autologous hypogastric arterial graft would have been a better solution. Had the coarctation been correctly diagnosed before or during the operation, the
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8
9
10
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REFERENCES Hvass U, Binet JP: Long segment coarctation of the thoracic aorta in a young adult. An alternative treatment. Thorac Cardiovasc Surg 28:449-451, 1980 Hvass U, Binet JP: Recurrent coarctation of the thoracic aorta. Subclavian flap arterioplasty with carotid reimplantation of the distal subclavian artery. Ann Thorac Surg (in press) Waldhausen JA, Nahrwold DL: Repair of coarctation of the aorta with a subclavian flap. J THoRAc CARDIOVASC SURG 51:532-533, 1966 Kimoto S: Surgical treatment of coarctation of the aorta with special reference to atypical coarctations. Clin Surg (Japan) 15:5-12, 1960 Morris GC, DeBakey ME, Cooley DA, Crawford ES: Subisthmic aortic stenosis and occlusive disease. Arch Surg 80:87-104, 1960 Glenn F, Keefer EBC, Speer OS, Dotter CT: Coarctation of the lower thoracic and abdominal aorta immediately proximal to the celiac axis. Surg Gynecol Obstet 94:561-569, 1952 Schuster SR, Gross RE: Surgery for coarctation of the aorta. A review of 500 cases. J THORAC CARDIOVASC SURG 43:54-70, 1962 Sautter RD, Myers WO, Smullen WA, Griese CG, Ray JF: Tandem coarctations of the thoracic and abdominal aorta with intervening hypoplastic thoracic aorta. Treatment with unilateral axiliofemoral graft. Ann Thorac Surg 23:582-584, 1977 Bergdahl L, Ljungqvist A: Long-term results after repair of coarctation of the aorta by patch grafting. J THoRAc CARDIOVASC SURG 80: 177-181, 1980 Geiss D, Williams WG, Lindsay WK, Rowe RD: Upper extremity gangrene. A complication of subclavian artery division. Ann Thorac Surg 30:487-489, 1980 Midgley FM, McClenathan JE: Subclavian steal syndrome in the pediatric age group. Ann Thorac Surg 24:252-257, 1977
Tandem coarctation of the thoracic aorta An unusual congenital anomaly A coarctation involving the aortic isthmus, with hypoplasia of the upper aortic segment, was satisfactorily repaired in a 15-year-o/d girl. The technique used was subclavian flap arterioplasty followed by reimplantation of the distal subclavian artery into the left carotid artery. Surprisingly weak femoral pulses were noted postoperatively and prompted a control angiogram. This showed an intraluminal diaphragm 2 em below the site of the previous repair, with a 100 mm Hg gradient. At reoperation, a centrally perforated fibrous diaphragm was excised, and the aorta was enlarged with a small Dacron patch. The postoperative course was uneventful.
Ulrik Hvass, M.D., Jean Langlois, M.D.,* Plessis Robinson, France, and P. E. Valere, M.D.,** Paris, France
Tandem coarctation of the aorta is an unusual congenital anomaly. The following report describes our management of this anomaly in a 15-year-old girl.
Case report A 15-year-old Guadeloupean girl was admitted for repair of coarctation of the thoracic aorta. She had been asymptomatic until shortly before admission, when she started experiencing malaise and visual impairment. Physical examination showed a well-developed girl. The arm blood pressure was 170/ 110 mm Hg and femoral pulses were absent. The chest roentgenogram showed no cardiac enlargement. Costal vascular markings were noted. The electrocardiogram revealed slight left ventricular strain. The diagnosis was confirmed by a cineangiocardiogram (Fig. I) with contrast media injected into the ascending aorta. Coarctation of the aortic isthmus was shown, with a fairly long, hypoplastic precoarctation segment. Collateral circulation was well developed. Initial operation. Operation was performed in November, 1980, via a left lateral thoracotomy in the bed of the fourth rib. After controlling the different segments of the aorta, we believed that resection of the coarctation would require an
From Centre Chirurgical Marie Lannelongue, 92350 Plessis Robinson, France, and Hopital Bichat, Paris, France. Received for publication Jan. 7, 1981. Accepted for publication March 24, 1981. Addressfor reprints: Dr. Ulrik Hvass, Centre Chirurgical Marie Lannelongue, 133 Av. de la Resistance, Plessis Robinson 92350, France. *Professorof HeartSurgery, CentreChirurgical MarieLannelongue. **Professorof Cardiology, Hopital Bichat.
592
interposition Dacron graft. The hypoplastic upper aortic segment was fairly long, and the lower segment was anchored by several large intercostal arteries, which would have to be ligated. To avoid using prosthetic material, we chose to use a subclavian flap arterioplasty and reimplant the distal subclavian artery into the left carotid artery, as previously reported in patients with long segment or recurrent coarctations. J, 2 The subclavian artery was large and long and was divided 1.5 ern proximal to the vertebral artery. It then was opened along its lateral margin, the opening being extended across the coarctation as originally described by Waldhausen and Nahrwold. a An intraluminal diaphragm was excised and the flap was sewn with a running 5-0 Prolene suture. After being unclamped, the aorta had a perfect contour with no gradient between the horizontal arch and the aorta just beyond the suture line. An anastomosis of the distal subclavian to left carotid artery then was performed via the same thoracotomy. Postoperative course. The immediate postoperative course was disappointing because of persistent upper extremity hypertension and unexpectedly feeble femoral pulses. Thrombosis, dissection, and an associated abdominal coarctation were discussed as explanatory possibilities. A control angiogram showed a perfectly well enlarged aortic isthmus and a nearly complete intraluminal diaphragm approximately 2 em below the previous repair (Fig. 2). At this site, a 100 mm Hg systolic gradient was recorded. Reoperation. Reoperation was accepted 3 weeks after the first operation. Intraoperative pressure recordings confirmed the absence of a gradient immediately distal to the subclavian flap, but showed a 90 mm Hg gradient a few centimeters further down. The exterior aspect of the aorta was normal except for a discrete indentation at the site of the pressure gradient. Lateral clamping of the aorta allowed a longitudinal opening. A fibrous diaphragm I mm thick was found with a central perforation 3 mm in diameter. The intima was intact on each surface of the diaphragm. The diaphragm was ex-
0022-5223/81/100592+03$00.30/0 © 1981 The C. V. Mosby Co.
Volume 82 Number 4 October, 1981
Tandem coarctation of thoracic aorta
593
tlv
Fig. I. Preoperative angiogram: The first coarctation is obvious, with hypoplasia of the upper aortic segment. The diaphragm of the second coarctation is faintly outlined .
v
Fig. 2. Postoperative control angiogram: The aortic isthmus is correctly enlarged, but there is a nearly complete intralum inal diaphragm . cised and the aorta enlarged with a small Dacron patch (Fig . 3). After the aorta was undamped . a 12 mm Hg systolic gradient was demonstrated between the right radial artery and the aorta ju st distal to the patch . The postoperative course was uneventful. No gradient was detected by cuff and Doppler measurements between the upper and lower limbs. nor between the left and right arms. Histologic stud y of the fragment s excised at both operations was not rewarding . since no aortic wall had been excised .
Discussion
Tandem coarctation obviously is a very unusual anomaly. Reports of atypical coarctations do not mention such a finding. 4- 8 The diagnosis was missed preoperatively, mainly because the first coarctation with segmental hypoplasia was so obvious that there was no thought of looking for another lesion. The second reason for missing the diag-
The Journal of
594
Hvass, Langlois, Valere
Thoracic and Cardiovascular Surgery
v
",
\
subclavian flap could have been tailored a bit longer so as to overlap both lesions. The subclavian-carotid artery anastomosis then would have been easier to accomplish via a cervical approach. A similar case might not be encountered again for a long time, but it is worthwhile to remember that one coarctation can hide another, even at the thoracic level. \
\
\
'--a
"
Appreciation is extended to Dr. Marthe Gautier, Maitre de Recherche, I.N.S.E.R.M., Hopital Bicetre, Paris, for reviewing the histology.
2
3 Fig. 3. Schematic drawing of the two-staged operation, which comprises a subclavian flap arterioplasty with the carotid reimplantation of the distal subclavian segment and a small patch enlargement just below the first repair.
4
5 nosis was the poor opacification of the aorta beyond the first coarctation. However, retrospective analysis of the preoperative cineangiocardiogram showed that a correct diagnosis should have been possible. The type of repair used for the initial operation in this patient deserves several remarks. It demonstrates our reluctance to use prosthetic material in young patients; reported results as well as our own experience show long-term complications such as aneurysms, infection, or thrombosis." As previously mentioned, we already had used the subclavian flap procedure for long segment coarctations and recurrent coarctations. Direct reimplantation of the distal subclavian with the left vertebral artery into the left carotid artery avoids shortterm 10 and long-term ischemia of the left arm as well as a subclavian steal syndrome. II Nevertheless, at reoperation we used a small Dacron patch to enlarge the aorta after resection of the second diaphragm. Perhaps an autologous hypogastric arterial graft would have been a better solution. Had the coarctation been correctly diagnosed before or during the operation, the
6
7
8
9
10
II
REFERENCES Hvass U, Binet JP: Long segment coarctation of the thoracic aorta in a young adult. An alternative treatment. Thorac Cardiovasc Surg 28:449-451, 1980 Hvass U, Binet JP: Recurrent coarctation of the thoracic aorta. Subclavian flap arterioplasty with carotid reimplantation of the distal subclavian artery. Ann Thorac Surg (in press) Waldhausen JA, Nahrwold DL: Repair of coarctation of the aorta with a subclavian flap. J THoRAc CARDIOVASC SURG 51:532-533, 1966 Kimoto S: Surgical treatment of coarctation of the aorta with special reference to atypical coarctations. Clin Surg (Japan) 15:5-12, 1960 Morris GC, DeBakey ME, Cooley DA, Crawford ES: Subisthmic aortic stenosis and occlusive disease. Arch Surg 80:87-104, 1960 Glenn F, Keefer EBC, Speer OS, Dotter CT: Coarctation of the lower thoracic and abdominal aorta immediately proximal to the celiac axis. Surg Gynecol Obstet 94:561-569, 1952 Schuster SR, Gross RE: Surgery for coarctation of the aorta. A review of 500 cases. J THORAC CARDIOVASC SURG 43:54-70, 1962 Sautter RD, Myers WO, Smullen WA, Griese CG, Ray JF: Tandem coarctations of the thoracic and abdominal aorta with intervening hypoplastic thoracic aorta. Treatment with unilateral axiliofemoral graft. Ann Thorac Surg 23:582-584, 1977 Bergdahl L, Ljungqvist A: Long-term results after repair of coarctation of the aorta by patch grafting. J THoRAc CARDIOVASC SURG 80: 177-181, 1980 Geiss D, Williams WG, Lindsay WK, Rowe RD: Upper extremity gangrene. A complication of subclavian artery division. Ann Thorac Surg 30:487-489, 1980 Midgley FM, McClenathan JE: Subclavian steal syndrome in the pediatric age group. Ann Thorac Surg 24:252-257, 1977