SvDhilitic Coronarv Ostial Sclerosis R. W. M. Frater, F.R.C.S., and A. Jordan, M.D.
S
yphilitic coronary ostial sclerosis is a rare entity in which, with or without otherwise normal coronary arteries, the ostia are involved in the pathological process of syphilitic aortitis. T h e aortic valve may or may not be diseased. The clinical features are like those of other forms of coronary artery obstruction but tend to occur in quite young patients [3, 5, 71. Cases of successful surgical treatment have been described from France [Z, 41 and South Africa [1, 81, but apparently not from the United States. This report concerns syphilitic coronary ostial obstruction in an American male which has been successfully treated surgically. Particular emphasis is given to the surgical technique needed in order to achieve this. The patient was a 55-year-old male. He had contracted syphilis at the age of 21 and had had an incomplete course of arsenical treatment at the time. He had never had any of the obvious manifestations of rheumatic fever and had been well until 6% years before, when while climbing stairs he first noticed angina pectoris which subsided quite rapidly with rest. During the succeeding years this symptom had become progressively worse, and eventually he began to have angina decubitus three to four times a day brought on by no particular circumstance and lasting as long as five minutes. The pain was retrosternal, extremely severe, and radiated into the neck, jaw, and left shoulder. With the development of the angina the patient began to experience dyspnea on exertion and, for some time before admission to the hospital, had been having attacks of paroxysmal nocturnal dyspnea. On a number of occasions he had felt light-headed with the dyspnea on exertion and had slight numbness and weakness in the left arm. Physical examination revealed a gaunt, chronically ill man. There was quite a prominent arterial pulsation in his neck, and the jugular pressure was moderately elevated. There were fine, crepitant rales to be heard at both lung bases. While the right brachial pulse pressure was wide and suggested aortic insufficiency by its quality, that on the left was small and hard to feel. The heart was enlarged with a prominent left ventricular apex beat, and aortic systolic grade 3 (grading 1 to 6) and early diastolic grade 3 murmurs were audible. Examination of the nervous system was normal. An attack of angina decubitus occurred during one examination. The pain was obviously extremely severe. The patient sweated, was unable to speak, and had a rise in blood pressure. Clinical diagnoses of aortic insufficiency causing angina pectoris and early left ventricular failure and a probable left subclavian steal were made. The blood pressure at rest in the right arm was 160/60 and in the left 108/60 mm. Hg. The electrocardiogram showed left ventricular hypertrophy and From the Divisions of Thoracic Surgery and Cardiology, Albert Einstein College of Medicine, New York, N.Y. Accepted for publication June 10, 1968.
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FIG. 1. (a) L e f t coronary angiogram. N o t e the dense calcium, deposits in the
aorta, especially around the orifice of the artery. ( b ) Drawing of the angiogram. T h e catheter (c) leads up t o but is too large t o enter the orifice. LC = L e f t coronary artery; AD = anterior descending branch (overlapping angiographic med i u m ) in the sinus of Valsatva.
possible inferior ischemia. The hemoglobin was 12.3 gm. per 100 cc., and the serological tests for syphilis were positive. X-rays of the chest showed a striking calcification of the ascending aorta and the whole arch which, on fluoroscopy, was seen to be densest at the root of the aorta and was thought probably to involve the aortic valve as well. After digitalization and diuresis, cardiac catheterization was performed and cineangiograms of the root of the aorta and the coronary arteries were obtained. The aortic root pressure was 219!59 mm. Hg. There was no gradient across the aortic valve, and the left ventricular end-diastolic pressure was 24 mm. Hg. T h e root of aorta angiogram showed that the aortic insufficiency was moderate but not of itself enough to account for the symptoms. I t was not possible to pass catheters into either coronary orifice, but an injection of contrast medium in the region of the left coronary sinus revealed a minute orifice leading after a short distance to what appeared to be an abnormally dilated left coronary artery. This dilatation narrowed further on, but only to normal dimensions, and the rest of the left coronary system was normal (Fig. 1). Further injections of angiographic medium were made to show the right coronary artery. T h e orifice was less well shown than on the left but was also small. Again, as on the left, the right coronary beyond its orifice was normal. Angiograms were also made of the aortic arch and left subclavian system. These showed filling of the left subclavian artery by retrograde flow from the left vertebral artery. The exact nature of the obstruction to the left subclavian artery was not seen. On the basis of these observations the diagnosis was changed to calcific coronary ostial sclerosis, calcific aortitis, and aortic valvular insufficiency, all probably syphilitic in origin. In addition, there was a left subclavian obstruction. Surgery for relieving the ostial narrowing and for repair or replacement of the aortic valve was advised. It was decided to defer repair of the left subclavian artery for the time being. OPERATION
After a median sternotomy incision, the patient was placed on cardiopulmonary bypass and cooled to 28°C. The aorta was cross-clamped, and an oblique aortotomy was made reaching down into the noncoronary sinus of Valsalva. The aortic wall was completely calcified, and applying the aortic clamp produced the sensation of cracking an egg. The calcium deposits were in the intima and became thicker toward the root of the aorta, reaching their densest
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in the sinuses of Valsalva. T h e most extensive deposits were in the left sinus of Valsalva, where craggy heaps of calcium almost filled the sinus and completely obscured the coronary orifice. T h e commissure between left and noncoronary cusps was involved in a large mass of calcium, and another deposit stretched between the left side of the right coronary cusp and the aortic wall. T h e orifice of the right coronary artery was also obscured by aggregations of calcium which were, however, much less extensive than those around the left orifice. T h e valvular insufficiency was caused by a failure of the cusps to meet, at least in part because of the restriction of the movement of the right coronary cusp. T h e cusps themselves were reasonably pliable and only minimally thickened. I t was found impossible to cannulate either coronary orifice. Accordingly, the right coronary artery was dissected free outside the aorta. A longitudinal arteriotomy was made, and a Spencer-Mallette Silastic catheter was passed into i t and held i n place by means of a silk suture. Probes were passed retrograde through the right coronary orifice into the aorta. At first, calcific resistance was encountered, but this was felt to break and thereafter a 3 mm. probe passed comfortably. T h e color and action of the heart, which had become poor, were restored and were thereafter well maintained once perfusion of the right coronary system at 200 cc. per minute was established. A minute trickle of bright blood from the center of the calcific mass in the left sinus showed the position of the coronary orifice. Attempts were made to enlarge this, but a variety of knives, scissors, curettes, and rongeurs were used to absolutely no avail. It seemed possible that a dental drill might have helped, but none was readily available. These failures prompted an alternative approach i n which a plane was developed between the calcified intima and the remainder of the aortic wall at the left edge of the aortotomy. When this plane had been dissected for a distance of about 1 cm., it was found that the calcific layer could be cracked off rather in the way a concrete paving stone can be cracked by levering up one of its corners. This process of dissecting, raising, and breaking was continued right to the coronary orifice, where the depth of calcium was more than 1 cm. Even this very thick deposit, which had resisted all direct attacks, cracked quite easily when a levering force was applied to it. Most of the calcium in the left coronary sinus was removed, and the result was most gratifying. T h e aortic wall exposed by the removal of the calcium was found to shelve quite smoothly into a left coronary orifice which was about 4 mm. in diameter (Fig. 2). More calcium was removed in the region of the right coronary sinus so as to free the aortic valve cusp. Once this was done it seemed that the cusps would meet sufficiently well to make valve replacement unnecessary. T h e left coronary artery was then cannulated and perfused, and the right coronary perfusion was discontinued so that a precise closure of the arteriotomy could be made. T h e calcified intima was removed from both sides of the aortotomy before closure, which was achieved by means of a running horizontal mattress suture passed through strips of Teflon felt on each side and reinforced by a continuous over-and-over stitch. Coronary perfusion was interrupted only momentarily during the aortic closure, and cardiac action remained excellent. Cardiopulmonary bypass was discontinued without difficulty, and the patient made an uneventful recovery from the operation. T h e patient has now been followed for eighteen months. There has been no angina pectoris, and no nitroglycerine tablets have been taken during this time. Exercise tolerance has returned to normal, and there have been no symptoms to suggest cardiac failure. T h e left arm still does not feel quite as strong as it should, but there have been no further attacks of dizziness. T h e patient is very well satisfied with his condition, and no further operations are contemplated. O n physical examination, the murmurs of the aortic insufficiency persist, but the pulse pressure has narrowed somewhat. Coronary angiography was repeated two months after surgery. T h e left coronary orifice was then shown to be normal. VOL.
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FIG. 2. (a) Calcific deposits in the aortic root. R i g h t coronary artery perfused from outside the heart. ( b ) Technique of levering 08 the calcium piece by piece. (c) L e f t coronary perfused. R i g h t coronary orifice cleared in retrograde fashion. ( d ) E n d result.
The poststenotic dilatation of the first portion of the left coronary system was still present. A normal right coronary system was also demonstrated, although a good view of the orifice was not obtained. Some aortic insufficiency was still evident (Fig. 3).
4
3b FIG. 3. Postoperative angiogram and drawing of angiogram. M u c h of the calcium has gone, and the orifice is clear.
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DISCUSSION
A strong argument for regarding this case as syphilitic coronary ostial sclerosis is provided by the presence of positive serology with aortic pathology dominantly involving the intima and coronary artery obstruction confined to the ostia. T h e extremely gross nature of the calcific deposits in our patient is unusual: In a case previously observed by one of the authors (R. W. M. F.), the obstruction was entirely fibrous, a minute left coronary orifice was always evident, and perfusion of the right coronary artery was possible from inside the aorta. However, calcification of the aorta occurs in about 40% of cases of luetic aortic incompetence and is characteristically maximal in the ascending aorta, in distinct contrast to the calcification in the atheromatous aorta [9]. In the present case, difficulty with coronary cannulation was anticipated, and hypothermia was used to provide extra protection for the myocardium should there be more than the usual delay before establishing coronary perfusion. As it happened, the rather unusual technique of cannulating the right coronary artery outside the aorta was necessary to provide coronary perfusion. T h e normal state of the coronary arteries beyond their ostia made it possible to perfuse the whole heart adequately by this route. T h e major technical problem was presented by the densely calcified intima, and this was solved with surprising ease once the principle was adopted of starting a plane of dissection well away from the coronary orifice and then using leverage to break the calcific mass away piece by piece. T h e postoperative result has been very satisfactory, with objective and subjective improvement in complete agreement. It is interesting to speculate whether the left subclavian steal syndrome in this patient is syphilitic in origin. So-called pulseless disease has been described with syphilitic aortitis [5], and it is quite possible that calcific deposits in the aortic intima are obstructing the left subclavian orifice. However, in the absence of more serious symptoms, no surgery is contemplated at present and the answer will have to remain uncertain. REFERENCES 1. Beck, W., Barnard, C. N., and Schrire, V. Syphilitic obstruction of the coronary ostia successfully treated by endarterectomy. Brit. Heart J. 27:911, 1966. 2. Blondeau, P., and Dubost, C. L. Surgical treatment under deep hypothermia of syphilitic ostial coronaritis: Apropos of 2 cases operated on with success. A n n . Chir. Thorac. Cardiovasc. 1:802, 1962. 3. Heggtveit, H. A. Syphilitic aortitis: A clinic-pathologic autopsy study of 100 cases, 1950 to 1960. Circulation 29:346, 1964. 4. Michaud, P., Froment, R., Pont, M., Saubier, E., and Aimard, G. Bilateral syphilitic ostial coronaritis: Dis-obstruction under extracorporeal circulation. Arch. Mal. Coeur 56:286, 1963. VOL.
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5. Ross, R. S., and McKusick, V. A. Aortic arch syndrome and diminished or absent pulses in arteries arising from the arch of the aorta. Arch. Intern. Med.
92:701, 1953. 6. Scharfman, W. B., Wallach, J. B., Angrist, A. Myocardial infarction due to syphilitic coronary ostial stenosis. Amer. Heart J. 40:602, 1950. 7. Schoenmackers, J., and Campos, J. L. C. Ostiumbarrieren, eine spezielle Lokalisation der Koronarsklerose. Arch. Kreislauflorsch. 43:235, 1964. 8. Schrire, V., Barnard, C. N., and Beck, W. Syphilitic coronary ostial occlusion. S. Afr. Med. J. 40:553, 1966. 9. Smith, W. G., and Leonard, J. C. Radiological features of syphilitic aortic incompetence. Brit. Heart J. 21: 162, 1959.
NOTICE FROM T H E BOARD OF THORACIC SURGERY T h e 1969 spring examinations will be given as follows: Written Examination. T o be held at various centers throughout the country on February 14, 1969. Final date for filing applications is December 1, 1968. Oral Examination. T o be given in March, 1969. Final date for filing applications is December 1, 1968. Please address all communications to the Board of Thoracic Surgery, Inc., 1151 Taylor Ave., Detroit, Mich. 48202.
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