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J Thorac Cardiovasc Surg 1994;108:587-588
© 1994 Mosby, Inc.

LETTERS TO THE EDITOR

Coarctation of aorta with right-sided arch: Surgical correction through right thoracotomy

8010 Frost St.
Suite 501
San Diego, CA 92123

To the Editor:

Coarctation of the aorta with right-sided aortic arch is rare. A search of the surgical literature revealed only six reported cases, none of which were repaired through a right thoracotomy.

My colleagues and I treated a 15-month-old girl in 1989. Catheterization showed a supracristal ventricular septal defect with a right-sided aortic arch and a discrete coarctation of the aorta with a 15 mm Hg gradient at the origin of an aberrant right subclavian artery. Reactive pulmonary hypertension (65/20 mm Hg), mild mitral regurgitation, and a moderately dilated left ventricle were also noted. The pulmonary/systemic flow ratio was 3.2:1. Pulmonary artery banding was done on March 1, 1989. The pulmonary pressures were brought to 30/15 mm Hg, with a simultaneous increase of systemic pressure from 50 to 65 mm Hg; oxygen saturations were maintained at 100%.

The patient returned for reoperation in November 1989. Repeat catheterization showed that the gradient in the coarctation had increased from 15 to 70 mm Hg. In addition, a subaortic peak systolic gradient of 70 mm Hg was identified. Because of these findings of increased severity of coarctation after pulmonary artery banding, we proceeded with coarctation repair. Inasmuch as the descending aorta had not been visible during the left thoracotomy for pulmonary artery banding, we approached the coarctation through a right thoracotomy.

The descending aorta was located behind the esophagus. The aberrant branches were dissected free. After induction of anesthesia, the patient was given intravenous corticosteroids. She was heparinized and a pressure-monitoring cannula was placed in the distal thoracic aorta. Every artery in the area of the coarctation was encircled with silicone rubber loops. The esophagus was separated medially. The anomalous origin of the right subclavian artery was found exactly at the site of the coarctation. This anomalous artery had a significant ostial narrowing. The right common carotid artery was adjacent to this aberrant subclavian artery, immediately below and also at the level of the coarctation. The left subclavian artery was arising on the opposite side, also at the area of the coarctation posteriorly. Pressure measurements showed a distal aortic pressure before clamping of 40 to 50 mm Hg. On test clamping this pressure dropped to about 35 mm Hg. It was technically unfeasible to place any shunt in this area because of the deep location of the proximal aorta and the limited exposure of the descending aorta. Therefore, we elected to do the simplest possible operation to minimize the crossclamp time and the risk of postoperative paraplegia. The anatomy was such that the only runoff vessels after crossclamping were the left common carotid, the coronaries, and the collateral vessels. The aorta was crossclamped distally and then all the proximal vessels, including the left subclavian, right subclavian, and right carotid, as well as the aorta above the coarctation, were included in one clamp. A longitudinal incision was made across the coarctation and extended above and below for about 3 to 5 mm to the normal ascending and descending aorta. The incision was then extended into the origin of the right subclavian artery to provide a new opening with a polytetrafluoroethylene patch. An oval-shaped polytetrafluoroethylene patch was then sewn in place with continuous 6-0 monofilament suture. The total crossclamp time was 21 minutes. No residual postoperative gradient was detectable.

View larger version (162K): [in this window] [in a new window]   Fig. 1. Aortogram showing right-sided aortic coarctation with three brachiocephalic vessels arising at the coarctation site.

The first case of aortic coarctation with right-sided arch was reported in 1963. ¹ A report in 1975 showed only six cases. ^2-4 Among these six reported cases, repair was attempted in five. No details are available on the first two cases, but successful resection with end-to-end anastomosis was reported in the third case. In the last two cases the approach apparently was made through a left thoracotomy. This incision afforded poor exposure in the first case, making end-to-end anastomosis unfeasible. Apparently, a patch repair was done in both cases. One of the patients had severe residual coarctation, and rerepair was not attempted because the patient's condition was considered to pose insurmountable surgical problems.

To our knowledge, the right thoracotomy approach to repair of coarctation has not been reported. In our case, it provided satisfactory although limited exposure to the descending aorta, which was located behind the esophagus. A unique technical challenge in this patient was the fact that three of the four arch branches were arising from the coarctation. To repair the coarctation, we had to crossclamp those three vessels and leave only the left common carotid as the runoff from the arch. A patch angioplasty simplified this operation.

In view of our success, we would like to suggest the right thoracotomy approach for patients with coarctation and right-sided aortic arch who have similar anatomic circumstances. We would also recommend moderate hypothermia and other measures of spinal cord protection to minimize the risk of postoperative paraplegia. Another alternative is the use of cardiopulmonary bypass with hypothermia, which may be the procedure of choice for difficult anatomic situations and poor collateral blood flow.

References

1. Stewart JR, Kincaid OW, Edwards JE. An atlas of vascular rings and related valve malformations of the aortic arch systems. Springfield, Ill. Thomas, 1965.
   
2. Price HL, Schienken RM. Right aortic arch with coarctation of the aorta. Chest 1974;65:110.[Abstract/Free Full Text]
   
3. Felson BF, Palayew MT. The two types of right aortic arch. Radiology 1963;81:745.
   
4. Grossman M, Jacoby WJ Jr. Right aortic arch and coarctation of the aorta. Dis Chest 1968;56:158.
   

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