Recurrent Coarctation of the Aorta

H. C. Stansel, Jr., M.D., and Robson Newbold, M.D.

ABSTRACT Recurrent coarctation of the aorta after an ap arently satisfac- tory initial resection is now being recognized. This comprication appears especially likely to occur in infants but has been described in adolescents as well. It seems clear that there is tissue distributed along the proximal descending aorta which under certain conditions is capable of producing recurrent coarcta- tion. A review of the literature reveals the very serious nature of this lesion, and suggestions are made regarding a technique to prevent this complication.

ecurrent coarctation of the aorta after an apparently successful repair is an infrequent but well-documented entity. Failure R to sustain normal pressure in the distal aorta and satisfactory

femoral and pedal pulses may be related to an inadequate repair at the initial operation, failure of growth of the anastomosis when performed in a small child, or actual restenosis in the region of the original suture line. In infants the recurrence of stenosis after an ;apparent technically perfect repair may develop quickly. D’Abreu and Parsons in 1956 [Z] and Mulder and Linde in 1959 [5] have each reported examples in which restenosis occurred after a classic end-to-end anastomosis was performed following resection of a clearly defined narrowed aortic seg- ment close to the ductus arteriosus. In both the infants femoral artery pulsation was noted to be excellent at first, but within only a few weeks the pulses were lost in the legs and a tightly coristricted coarctation was demonstrated by aortography. It is pertinent that Mulder and Linde suggested a relationship between the level of aortic incision and that of the duct on one hand and the development of recurrent stenosis on the other. They suggested that tissue with an inherent capability of constriction might have been left adjacent to the suture line and caused the restenosis.

We recently have had experience with 2 infants in whom tech- nically successful initial coarctation repairs were performed with return of excellent peripheral pulses, only to be followed in a short time by a recurrence of coarctation as manifested by loss of peripheral pulses and, in 1 patient, a recurrence of congestive failure. In 1 of these infants a reoperation was performed that confirmed the recurrent coarctation,

From the Department of Surgery, Yale University School of Medicine and Yale-New Haven

Accepted for publication July 29, 1970. Address reprint requests to Dr. Stansel, Yale University School of Medicine, 333 Cedar St..

Hospital, New Haven, Conn.

New Haven, Conn. 06510.

380 THE ANNALS OF THORACIC SURGERY

CASE REPORT: Recurrent Coarctation of the Aorta

and a successful reexcision was achieved. We would like to report these 2 patients as examples of restenosis of coarctation and to suggest how this lesion theoretically can be prevented.

PATIENT 1 K. A. C. was a premature twin who was admitted to the Yale-New Haven

Hospital in December, 1968, two weeks after birth, because of congestive heart failure. She weighed 1,610 gm., her pulse was 160, and respirations were 72 per minute. Respiratory distress was noted, and auscultation revealed systolic mur- murs-grade 2 of 6 at the apex and grade 2 of 6 in the left upper lateral chest. There were no lung rales. The liver was felt 4 cm. below the right costal margin. An electrocardiogram showed right axis deviation and right ventricular hyper- trophy, and a chest roentgenogram demonstrated increased pulmonary vascular markings and a large right atrium. The cardiac failure was treated with digoxin and diuretics, but response was not satisfactory. Cardiac catheterization was done one month later and demonstrated a left-to-right shunt at the atrial level, mod- erate dilatation of the left atrium and left ventricle, and a coarctation of the descending aorta.

Three months after the initial examination an operation was performed for the coarctation. A membranous type of stenosis of the descending aorta just beyond the subclavian artery was resected. The repair was accomplished with 6-0 silk mattress sutures interrupted posteriorly and running anteriorly. The anastomosis was performed approximately 5 mm. distal to the subclavian artery. Recovery was difficult, but lower extremity pulses were excellent. The patient was readmitted two and one-half months later because of congestive failure and pneumonia, and at this visit the lower extremity pulses were not palpable. Conse- quently, in June, 1969, a resection of the restenosis of the descending aorta was carried out. This anastomosis was made at the level of the left subclavian artery to give a wider lumen. When the patient was discharged 19 days later her condi- tion was satisfactory and she had excellent femoral pulses.

PATIENT 2 E. K. was 13 days old when admitted to the Yale-New Haven Hospital on

May 10, 1964, because of heart failure that was unresponsive to digoxin. He had tachypnea, his heart was enlarged, and there was a grade 2 systolic murmur along the left sternal border with radiation to the back. Femoral pulses were faint, and brachial pulses were bounding. The electrocardiogram showed a prolonged P-R interval, right axis deviation, and right ventricular hypertro hy. Cardiac cathe-

tension and a short coarctation of the aorta just distal to the subclavian artery. Five days after admission a wedge resection of the coarctation was done. The

medial aortic wall was left intact, and aortoplasty was carried out. The baby did well and was discharged on the fifteenth ostoperative day with excellent

were barely palpable; nine months after operation no femoral pulses were palpable. However, the child showed no evidence of heart failure, and he was developing normally. Return of palpable but very weak femoral pulses was noted two years later. At the present time he is doing well but does have occa- sional leg pains on vigorous exercise.

Blood pressure in the arms, which had risen to 200 mm. H g after the opera- tion, has now dropped down to the level of 130 mm. Hg. The atient's femoral pulses are faintly palpable, and repeat catheterization is planne l! in 1971. Aorta- plasty as used in this patient was probably a poor operation, because although it provided an adequate lumen, it left all the abnormal tissue along the medial wall.

terization was done the day after admission and demonstrate 1 pulmonary hyper-

femoral pulses. At a clinic visit two months a P ter operation the femoral pulses

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COMMENT

Restenosis of coarctation repair apparently is not a rare occurrence. Khoury and Hawes [4] have described 9 of 25 infants in whom tech- nically successful coarctation repairs were followed by the development of restenosis. Also, restenosis is apparently not limited to the infant age group; it has been described in adults. Cerilli and Lauridsen [l] have described 6 patients aged 5 to 24 years in whom reoperation for recurrent coarctation was necessary. Parson and Astley [6] have also reported immediate and delayed restenosis in 10 patients, 5 of whom were under 1 year and 5 over 8 years of age. They speculated that the restenosis could be related to perivascular infla:mmation, incomplete surgical relief of the obstruction, or clotting at the suture line. Carlos Ibarra-Perez and his colleagues [3] have described. 20 patients, or 8.5% of their total experience, in whom significant reooarctation developed. The serious nature of this complication is borne out by the death of 4 of these 20 patients during and following repeat resection.

Khoury and Hawes performed histological examination of the restenosed segment in their patients. Similar findings were present in all; the tissue was characterized by thickened, edematous intima and a hypertrophied media, and suture granuloma was scattered through- out. They commented that in all their patients the ductus arteriosus had been excised with a coarcted segment and that therefore residual ductal tissue could not be involved in the process of restenosis. They then raised two questions: (1) Can a normal intima and media pro- liferate because of surgical trauma to the aortic wall? (2) If the entire segment of coarctation is resected, is any abnormal tissue retained which has the capability of proliferating and producing secondary stenosis?

As noted by these authors, there would appear to be an ab- normality of the aortic wall not limited to the narrowed coarcted segment. Indeed, it would appear, based upon their studies, that there is thickened and atypical tissue extending for a short distance both cephalad and caudad to the primary coarctation site. The thickened intima and media extend for a distance away from the medial spur and taper down until they blend with normal tissue. This observation and the findings of Mulder and Linde suggest that restenosis is due to retained abnormal intimal and medial tissue which has the potential of proliferating and producing narrowing at the a:nastomotic site. The extent of this abnormal tissue may well vary from one patient to another. Operative trauma and suture granuloma may also play a part in stimu- lating the restenosing process.

The above observations, plus the fact that at operation one fre- quently finds significant thickening of the medial aspect of the proximal

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CASE REPORT: Recurrent Coarctation of the Aorta

EXTENT OF COARCTATION RESECTION REPAIR

FIG. 1. aorta proximal to the coarcted site is removed.

The extent of resection recommended. The entire descending thoracic

descending thoracic aorta after completely resecting all coarcted tissue, have led us to approach the operative repair of coarctation in the fashion described below.

After division of the patent ductus arteriosus or ligamentum arte- riosum, the entire descending thoracic aorta from the origin of the left subclavian artery to a point just distal to the site of coarctation is excised, taking care to remove all the aorta containing thickened tissue along the medial aspect (Fig. 1). A longitudinal incision is then made in the subclavian artery for a distance of 3 to 4 mm. to provide a wide lumen for anastomosis (Fig. 2). The distal aortic lumen is then brought up to the proximal lumen, and a typical end-to-end anastomosis is performed. In infants and young children the posterior row is sutured with interrupted 6-0 mattress sutures and the anterior row with con- tinuous 6-0 mattress sutures. In the infant there is always sufficient elasticity to achieve this radical resection and yet perform the anasto- mosis without undue tension.

FIG. 2. The incision of the proximal subclavian artery in the anastomosis.

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In older patients, in whom such a radical resection may result in inability to oppose the ends without inordinate tension, we have prac- ticed making a limited excision, testing the tension by approximating the aortic ends, and then serially excising more tissue while repeatedly testing the tension until all the abnormal aorta is removed. In this way the anastomosis can be made without undue tension. In practice, at least in the pediatric age group, it is practica1:ly always possible to excise the entire descending thoracic aorta from the subclavian artery to a point just distal to the coarctation. It is our belief that this segment of the descending thoracic aorta has in certain patients the capability of producing restenosis. Therefore, one should excise this entire segment when possible in order to prevent the complication of recoarctation.

REFERENCES

1. Cerilli, J., and Lauridsen, P. Re-operation for coarctation of the aorta. Actu Chir. Scund. 129:391, 1965.

2. D’Abreu, A. L., and Parsons, C. Surgical treatment in children with coarcta- tion of the aorta. Brit. Med. J. 2:390, 1956.

3. Ibarra-Perez, C., Castaneda, A. R., Varco, R. L., and Lillehei, C. W. Recoarc- tation of the aorta. Amer. J. Curdiol. 23:778, 1969.

4. Khoury, G. H., and Hawes, C. R. Recurrent coarctalion of the aorta in in- fancy and childhood. ]. Pediut. 72:801, 1968.

5. Mulder, D. G., and Linde, L. M. Recurrent coarctation of the aorta in in- fancy. Amer. Surg. 25:908, 1959.

6. Parson, C. G., and Astley, R. Recurrence of aortic coarctation after operation in childhood. Brit. Med. J. 1:573, 1966.

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