Diagnosis and Operative Treatment of Coarctation of the Aorta MARY ALLEN ENGLE, M.D. FRANK GLENN, M.D.
COARCTATION of the aorta is a narrowing of the lumen sufficient to obstruct the normal flow of blood and to produce a systolic pressure gradient across the obstruction. The coarctation is commonly located in the descending arch of the aorta just distal to the left subclavian artery, but it may occur at any area from the transverse arch downward and there may even be multiple areas of narrowing. It is fortunate that most coarctations are located just below the left subclavian artery, for this region affords the best opportunity for development of collateral circulation, as vessels from the subclavian system anastomose with intercostal arteries and deliver blood in retrograde fashion into the descending aorta. The degree of constriction varies from an exaggeration of the aortic narrowing that is normal in the fetus proximal to a patent ductus arteriosus, to almost complete obliteration of the lumen of the aorta. Usually
Fig. 1. Areas of coarctation resected surgically demonstrate variation in size of aperture of the diaphragm at the coarcted site.
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Fig. 2. Elongated coarctation of lower thoracic and abdominal aorta in a 19 year old woman. (From Glenn, F. et al.: Surg. Gynec. & Obst . .9,4: 561, 1952. Reprinted with permission of Surgery, Gynecology and Obstetrics.)
the coarctation is localized and when viewed from outside has an hourglass appearance with poststenotic dilatation. Inside the aorta the narrowing is even more marked, for there is a diaphragm of tissue with a small central or eccentric perforation (Fig. 1). Elongated, diffuse areas of narrowing occur, and especially if located in the low thoracic or abdominal aorta, there is often a reaction in the periaortic tissues suggesting an inflammatory, acquired condition (Fig. 2). Although coarctation is about four times more common in men than in women, those unusual in location or otherwise complicated are more apt to be found in women. Associated malformations that not infrequently coexist with coarctation include aortic valve anomalies, patent ductus arteriosus and ventricular septal defect. DIAGNOSIS
The diagnosis of coarctation is based on the discrepancy between the upper and lower extremities in the pulse and blood pressure. Pulses are
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Fig. 3. Preoperative roentgenogram of chest of 972 year old boy with coarctation of aorta. Note upper displacement of barium in esophagus by the left aortic arch and the lower displacement by the poststenotic dilatation of the aorta below the coarctation. There is no rib notching.
less strong and are delayed, and the blood pressure is lower, in the vessels distal to the coarctation than in those proximal. Often there is hypertension in the arms while the blood pressure in the legs is unobtainable. Frequently too, there are bounding pulsations in the suprasternal notch and in the carotid and radial arteries while the femoral and more distal pulses are impalpable. It should be emphasized, however, that these extreme changes are not essential for the diagnosis of coarctation to be made; the condition can exist in the absence of hypertension and in the presence of easily palpable femoral pulses. This range of variation depends on the degree of narrowing and adequacy of collateral pathways. It is the reversal of the normal pulse and pressure relationship between upper and lower extremities that is diagnostic. A systolic murmur is heard posteriorly over the coarctation site but in adults this may be masked by the murmur heard over the vessels of collateral circulation in the chest wall. Other corollary evidence in the diagnosis of coarctation is chiefly radiologic: dilatation of the ascending arch of the aorta, decreased prominence
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Fig 4.
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Fig. 5. Angiocardiogram of young woman showing coarctation below left subclavian artery and the poststenotic dilatation of aorta. There is ample distance between the dilated subclavian artery and the narrowest point of the coarctation for application of aortic clamps and for resection of the coarcted region. Note tortuous and dilated internal mammary artery.
of the aortic knob, indentation of the descending aorta at the coarcted site with poststenotic dilatation visible below and outlined with barium in the esophagus in frontal (Fig. 3) and left anterior-oblique and lateral projections (Fig. 4). Notching of the inferior margins of the ribs by tortuous intercostal arteries is not ordinarily seen until adolescence or early adult life (Fig. 4). Left ventricular enlargement may be vresent.
Fig. 4. Frontal and lateral views of chest of a 23 year old man with coarctation. Note dilatation of ascending aorta at the right base and on the left, prominence of the aortic knob, below which is an indentation due to coarctation in the descending aorta. and a second prominence due to poststenotic dilatation. There is bilateral scalloping of the inferior margins of the ribs. In the lateral view, barium in esophagus is displaced anteriorly by the postcoarcted, dilated segment of descending aorta. Inner plate of sternum is scalloped.
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Angiocardiography and aortography afford visualization of the location and extent of the coarctation (Fig. 5). -' Typically the diagnosis is made at the time of a routine physical examination of a healthy, husky individual. Symptoms from the coarctation are most often encountered in two age groups, infancy and young adult life. The baby may fail to thrive and even manifest symptoms and signs of heart failure. After the benign period of the childhood years, new symptoms may appear: headache, epistaxes, claudication and easy fatigue. One boy was only 17 years old when he suffered a cerebrovascular accident. He recovered and was operated upon successfully. Deaths attributable to coarctation similarly occur in these two age periods: in infancy from heart failure, usually in association with additional cardiac anomalies, and in early to mid-adult life because of hypertensive cardiovascular disease with heart failure or cerebral hemorrhage, or because of sudden rupture of the aorta, or bacterial endarteritis or endocarditis. INDICATIONS FOR OPERATION
Indications for surgical repair of the coarctation depend on the age of the patient and severity of the coarctation. Although resection of the coarctation has been safely performed as early as ten weeks of age, we believe that operation on symptomatic young babies should be deferred unless they fail to respond to medical management. Children generally tolerate this anomaly well and operation can be postponed until the aorta has reached nearly adult proportions but before the development of degenerative changes in the vessel. This age for elective surgery in children is around 9 to 13 years. There is no need to postpone surgical treatment when the diagnosis is first made in the teen-ager or young adult. The risk of the operation in this group is now sufficiently low and the long-term results of surgery sufficiently good that we believe operation should be recommended on the basis of the diagnosis alone. When the diagnosis is made for the first time in an older patient, over the age of 40 to 45, he is apt to have a slight coarctation with little collateral circulation-a situation that may not interfere with the patient's longevity and one where the operation carries a somewhat higher risk because of the possibility of spinal cord ischemia at operation. Other older adults may have more evidence of significant narrowing of the aorta but, again, operation carries a higher risk because of sclerotic changes in the walls of the aorta, aneurysmal dilatation of the aorta or intercostal arteries. Furthermore, relief of hypertension is apt to be less complete in the older patient after operative treatment. Decision about operation in the older adult, therefore, is more difficult and depends on the assessment in that individual of the relative risks of operation or observation. The youngest patient we have operated upon was a 272 year old boy with excessive hypertension of 180/110 mm. Hg,
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Fig. 6. Operative view of area of coarctation and a still-patent ductus arteriosus. Rubber tapes have been placed about the coarctation above and below the ductus.
and the oldest patient was a 49 year old man who had recently become symptomatic. Both have done well. THE OPERATION
The operation consists of a left thoracotomy through the third interspace or the bed of the third rib to expose the distal arch of the aorta, the coarctation, the ligamentum arteriosum, and the descending thoracic aorta down to the diaphragm (Fig. 6). The aorta in the vicinity of the coarctation is dissected free, the ligament()us or occasionally patent ductus is divided, and the adjacent intercostals are secured. Those nearest the coarcted segment are usually divided. The area of coarctation is resected, removing the diaphragm and as much of the narrowed aorta above and below as possible while still permitting approximation of the divided ends of the proximal and distal aorta. These are anastomosed by everting sutures to' re-establish continuity without obstruction. Long
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segments of coarcted aorta, aneurysmal formation and atherosclerotic changes may require the use of a homograft or a plastic prosthesis to bridge a gap too great for direct approximation of the ends of the vessels. OPERATIVE MORTALITY
Since Crafoord and Gross individually reported on correction of coarctation of the aorta in 1945, several thousand patients have been treated surgically by a number of widely distributed surgeons. The results have been excellent. The morbidity and the complications have been low, and the mortality rate has ranged around 5 per cent among experienced teams. Gross has reported the largest series of cases, 500 patients with a mortality rate of 4 per cent. Our experience is much less. In the first 70 consecutive patients there were three deaths. One death in a child of 11 occurred on the operating table due to cardiac arrest as the clamps were released from the aorta. The second was in a young man with an expanding aneurysmal dilatation of the aorta distal to the coarctation. This was resected and continuity restored with a graft, but a few days after operation the suture cut through the diseased aortic wall and exsanguination occurred. The third patient, a young man with coexistent patent ductus and pulmonary hypertension, died at a second operation made necessary by the development of a false aneurysm about the anastomosis. POSTOPERATIVE COMPLICATIONS
A rare but serious complication of operative treatment has been ischemic injury of the spinal cord. This has happened once in our experience in a 44 year old man who has had almost complete return of function. Adams and van Geertruyden have collected and reported on a number of these, together with a larger group that were the result of operations upon the aorta at different levels for aneurysms and occlusive lesions. In most but not all instances in which this occurred in patients with coarctation, several pairs of intercostals had been occluded or divided. Several of these patients had a patent ductus which, of course, was interrupted. It is significant that a few patients had a coarctation with a lumen that was reduced to no less than half the normal size. Under this situation, where collateral circulation was not rich, the ischemia would seen to result from the temporary occlusion of aorta while the anastomosis was being done. In dogs, this damage may take place after 20 minutes. The incidence of this complication.has been reduced by several factors: (1) decrease in time of complete occlusion of aorta, (2) use of hypothermia during the operation and/or in the immediate postoperative period and (3) minimal occlusion of collateral vessels. Another infrequent complication, occurring twice in our experience,
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has been attacks of abdominal pain with intestinal obstruction. It has been reported that resection of gangrenous loops of bowel has sometimes been necessary. Histologic examination has revealed a periarteritis of the abdominal vessels. Infection of the anastomotic site in the first few weeks postoperatively has been reported and has been responsible for some instances of fatal hemorrhage. Measures directed toward prevention of this complication in so far as possible include selection of the date for surgery when the patient is free from recent infection, use of antibiotics in therapeutic doses from the time he leaves the operating room till the first week has passed, prompt treatment of any infection and avoidance of dental work during the first six to eight weeks after operation. Because the anastomotic site may be a potential focus for organisms in the blood stream to lodge and because of the frequent coexistence of abnormalities of the aortic valve, we believe that antibiotics should be used in the years after surgery, just as preoperatively, at the time of a predictable bacteremia following dental extraction, tonsillectomy, vaginal delivery, etc. LATE RESULTS OF SURGICAL TREATMENT
The results of surgical treatment have been gratifying indeed in those patients in the ideal age group as previously defined. Femoral pulses have been strong from the moment aortic continuity is re-established, and in the subsequent 10 to 14 days blood pressure in the arms has decreased to normal levels and in the legs has risen to approximate or exceed that in the arms. Cardiac enlargement has decreased or disappeared. Postoperative follow-up of 10 to 15 years is now available on a number of patients. Recurrence of stenosis in those patients operated upon in late childhood or beyond has not, to our knowledge, been reported. Nor has aneurysm formation at the site of anastomosis been reported, other than those that formed in the immediate postoperative period which are actually false aneurysms. Pregnancies ending in normal deliveries have been without cardiovascular event, and strenuous physical exertion including the heaviest of manual labor and competitive athletics has been well tolerated. PERTINENT LITERATURE Adams, H. D., and van Geertruyden, H. H.: Neurologic Complications of Aortic Surgery. Ann. Surg. 14-1: 574, 1956. Campbell, M. and Bayles, J. H.: Course and Prognosis of Coarctation of Aorta. Brit. Heart J. 18: 475, 1956. Crafoord, C. and Nylin, G.: Congenital Coarctation of Aorta and Its Surgical Treatment. J. Thoracic Surg. 14-: 347,1945. Freundlich E., Engle, M. A. and Goldberg, H. P.: Coarctation of Aorta in Infancy. Analysis of Ten Years' Experience with Medical Management. Pediatrics, 1961.
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Glass, 1. H., Mustard, W. T. and Keith J. D.: Coarctation of Aorta in Infants. A Review of Twelve Years' Experience. Pediatrics 26: 109, 1960. Gross, R. E. and Hufnagel, C. A.: Coarctation of Aorta; Experimental Studies Regarding Its Surgical Correction. New England J. Med. 233: 287, 1945. Lober, P. H. and Lillehei, C. W.: Necrotizing Panarteritis Following Repair of Coarctation of Aorta; Report of Two Cases. Surgery 35: 950. 1954. Sloan, R. D. and Cooley, R. N.: Coarctation of Aorta: Roentgenologic Aspects of 125 Surgically Confirmed Cases. Radiology 61: 701, 1953. Wright, J. L., Burchell, H. B., Wood, E. H., Hines, E. A. Jr. and Clagett, O. T.: Hemodynamic and Clinical Appraisal of Coarctation Four to Seven Years After Resection and End-to-End Anastomosis of Aorta. Circulation 14: 806, 1956.
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