Subclavian-coronary artery anastomosis A technique for definitive correction of anomalous origin of left coronary artery A case of anomalous origin of the left coronary artery from the pulmonary artery in a S-year-old child is presented. Surgical correction by means of a direct subclavian-coronary anastomosis was performed at normothermic temperatures without cardiopulmonary bypass. Pre- and postoperative studies including angiography are presented. The rationale for this approach and its benefits are compared to previously utilized surgical techniques. We feel that this method is superior in that it creates a two-coronary system without utilizing prosthetic or vein bypass grafts. It thus can be used in infancy, allows for future growth and development, and is not subject to the inherent deficiencies of prosthetic or vein grafts. All 4 known cases of subclavian-coronary anastomosis previously reported are discussed. Elliott Senderoff, M.D., F.A.C.S., Arnold J. Slovis, M.D., Anita Moallem, M.D., and Robert E. Kahn, M.D., New York, N. Y.
A he anomalous origin of the left coronary artery arising from the pulmonary artery is encountered infrequently. However, when diagnosed, it presents a fascinating medical and surgical challenge. Within the past 20 years there have been various surgical approaches toward palliation and definitive correction of this condition in both infancy and early childhood. 1-9 Our recent experience utilizing a direct subclavian-coronary artery anastomosis for definitive therapy prompts us to review the current concepts of therapy and recommend consideration of this procedure in the treatment of this condition. The clinical spectrum based on a review of 147 cases by Wesselhoeft and associates10 suggests that the diagnosis was determined in infancy in 82 per cent of patients. They had angina-like symptoms, cardiomyopathy, or mitral insufficiency. The majority of patients died in the first year of life. The surviving children and adults presented with murmurs, anginalike syndromes, or sudden death.8 Myocardial ischemia results from the perfusion of the left ventricle with unoxygenated blood originating from the pulmonary From the Departments of Surgery, Pediatrics, and Radiology, Flower-Fifth Avenue Hospitals, New York Medical College, Fifth Avenue and 106th Street, New York, N. Y. 10029. Received for publication April 14, 1975. Address for reprints: Arnold J. Slovis, M.D., 1249 Fifth Avenue, New York, N. Y. 10029. 142
artery under low pressure. As the right coronary artery forms collaterals with the left coronary system, retrograde perfusion occurs to provide proper oxygenation to the left ventricular myocardium. With sufficient functioning collaterals, a complete reversal of flow into the pulmonary artery may result. Early surgical attempts at palliation were based upon ligation of the anomalous coronary artery at its pulmonary origin to prevent a runoff of blood from the myocardium. This resulted in a high mortality rate among infants with poor collateral flow.11 Those who survived were also deprived of the obvious benefits of a two-coronary system. Surgical attempts to devise a two-coronary system include the use of the common carotid artery1 and the left subclavian artery 3,7 ' 9 for direct anastomosis to the left coronary artery and use of the saphenous vein bypass between the aorta and coronary artery.5, 6> 12 The use of cardiopulmonary bypass has also been advocated.9, 13 Use of the subclavian artery was first proposed by Apley3 in 1957. The attempt was unsuccessful. In 1967 Meyer7 successfully performed a subclavian-coronary anastomosis in a 4-monthold infant without cardiopulmonary support. Table I summarized all operative cases reported and known. Cardiopulmonary bypass was used in 2 cases. However, the fact that 2 other patients survived indicates that the procedure can be done without bypass
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Table I. Known cases of direct subclavian-coronary Author
Date of operation
Apley et al.3
1951
Meyer et al.7
Mar. 13, 1967
Pinsky et al.9
Nov. 7, 1970
Senderoff et al.
Dec. 5, 1972
Pinsky et al.9
Dec. 27, 1972
Table II. Preoperative
Location SVC IVC RA RV PA LV Aorta
cardiac
Pressure (mm. Hg.)
3 (mean) 25/5 25/10 80/9 80/50
artery anastomosis
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bypass
Type of operation Subclavian-coronary anastomosis; no pump Posterolateral approach; normothermia; no pump Median sternotomy; cardiopulmonary bypass; hypothermia Posterolateral approach; no pump Median sternotomy: cardiopulmonary bypass; hypothermia
Result
Age 11 mo. 4 mo. 8'/2 mo. 5 yr. 4 mo.
Unsuccessful Successful; patient alive and well, 6 mo. follow-up Successful; asymptomatic and well Alive and well IVi yr. postop.; angiogram 3 mo. postop. Alive and well; angiogram 1 week postop.
catheterization Oxygen content (vol. %)
Oxygen sat. (%)
10.0 10.4 10.1 10.0 11.0 12.0 12.0
76.0 79.0 77.0 76.0 84.0 91.5 91.5
Legend: SVC. Superior vena cava. IVC. Interior vena cava. RA, Right atrium. RV. Right ventricle. PA. Pulmonary artery. LV. Left ventricle.
support if the left coronary artery fills by collateral flow from the right coronary system. This method would appear to be simpler than the recently proposed procedure of direct aorta-coronary anastomosis which requires deep hypothermia and cardiopulmonary bypass. In regard to long-term considerations, this procedure is superior to the aorta-coronary saphenous vein bypass as it allows for future growth and development without the problems of vein thrombosis and stenosis at two anastomotic sites. Case report A 5-year-old child was admitted to Flower-Fifth Avenue Hospital in October, 1972, for evaluation of a heart murmur. The child was the product of a term pregnancy and normal delivery, having weighed 7 pounds, 11 ounces at birth. She had been completely asymptomatic as an infant. When she was approximately 2 years of age, a murmur was first heard. Over the past year, the child has complained of easy fatigability. Physical examination. The child weighed 36 pounds. Cyanosis and clubbing were absent. All pulses were palpable and equal. Blood pressure was 90/50 mm. Hg. The point of maximum impulse was at the fifth intercostal space, 1 cm. outside the midclavicular line. No heave was noted. There
Fig. 1. Preoperative retrograde aortogram showing a markedly dilated right coronary artery which supplied, by way of fistulas, collateral connections to a left coronary artery originating from the pulmonary artery. was a Grade 3/6 continuous high-pitched murmur heard loudest at the fourth intercostal space to the right of the sternum. This murmur was transmitted somewhat medially and superiorly. The pulmonary second sound was normally split. No other murmurs were noted. The lungs were clear to percussion and auscultation, and there were no rales present. Hepatosplenomegaly was not evident. Laboratory data. The hemoglobin value was 11.5 Gm. per cent, hematocrit 34 per cent, and white count 6,500 per cubic millimeter with a normal differential. The electrocar-
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Fig. 2A. Surgical approach via left posterolateral thoracotomy. The anomalous coronary artery is detached from the pulmonary artery with a cuff of vessel wall and anastomosed end-to-end to the left subclavian artery through a left thoracotomy.
Fig. 2B. Operative photograph of the completed subclavian-coronary anastomosis. diogram showed evidence of left ventricular hypertrophy with prominent Q waves in Lead aVL and Lead I suggestive of an anterior wall infarction. Chest x-ray films showed mild cardiomegaly with evidence of increased pulmonary vascular markings. On Oct. 4, 1972, the child underwent cardiac catheterization and angiography, which demonstrated normal right-sided pressures with evidence of a significant oxygen step-up in the pulmonary artery (Table II). Through a right femoral arteriotomy, a No. 6 Fr. NIH catheter was passed retrograde into the left ventricle. Left ventricular pressures were normal. The end-diastolic pressure was 9 mm. Hg. There was no gradient across the aortic valve. Angiography (Fig. 1) demonstrated a large right coronary artery supplying, by way of large collaterals, a left
coronary artery which emptied into the main pulmonary artery. The child was discharged and scheduled for future surgery. Surgical technique. The operation was performed on Dec. 5, 1972. A direct anastomosis between the left subclavian and the left coronary artery was created via a left posterolateral thoracotomy at normothermic temperatures without cardiopulmonary bypass. Prior to thoracotomy a saphenous vein segment was removed from the left groin in case direct coronary-subclavian anastomosis was not feasible and an interposed vein segment should be necessary for additional length. The chest was entered through the fourth intercostal space. The subclavian artery was dissected from its origin to its point of exit from the thoracic cavity. The vertebral and
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Fig. 4. Selective retrograde aortogram, taken 3 months after the operation, demonstrating a functioning left subclavianleft coronary artery anastomosis.
Fig. 3. Retrograde aortogram, taken 3 months after the operation, showing a smaller right coronary artery and a decrease in the number and size of collaterals. internal mammary branches were individually ligated with silk ties before the artery was transected. The mobilized subclavian artery appeared to be satisfactory, measuring 36 mm. in length and 6 mm. in diameter. The pericardium was then entered anterior to the phrenic nerve and the pulmonary artery was identified. The trunk of the main left coronary artery arose in the posterolateral aspect of the pulmonary artery just above the valve level. It was approximately 6 to 7 mm. in diameter and approximately 6 mm. in length before branching into the circumflex and anterior descending coronary arteries. The circumflex artery was approximately 6 mm. in diameter. Dissection of the main coronary trunk was performed. Several collaterals were individually ligated and transected to facilitate mobilization. The pulmonary origin of the left coronary artery was test clamped to determine the adequacy of the right-to-left coronary arterial perfusion as reflected in electrocardiographic changes of ischemia or arrhythmia. No difficulty was encountered. We felt that the subclavian artery could not reach the main left coronary trunk with an end-to-side anastomosis but would reach with an end-to-end anastomosis, if the common trunk were removed from the pulmonary artery origin with a portion of the pulmonary artery wall. Accordingly, excluding vascular clamps were placed on the pulmonary artery side in such a fashion as to exclude the origin of the left main trunk. The pulmonary artery attachment was then incised at the portion within the clamp and was oversewn with 4-0 Tevdek continuous suture. The detached coronary and pulmonary
artery cuff were then anastomosed to the distal subclavian artery in end-to-end fashion with 6/0 Tevdek continuous sutures (Figs. 2A and 2B). Occasional interrupted sutures were also placed. The subclavian clamp was then removed and good perfusion at the left coronary sytem was observed. The patient received 240 c.c. of blood during the procedure. Cardiopulmonary bypass had been available on a standby basis but was not utilized. At the termination of the procedure the patient was alert and reactive. Vital signs were within normal limits. The electrocardiogram recorded regular sinus rhythm with no evidence of arrhythmia. Blood gases were satisfactory. In March, 1973, some 3 months after definitive surgery, the child was admitted for postoperative evaluation. Electrocardiography showed no appreciable difference from the preoperative electrocardiogram. A chest x-ray film demonstrated a decrease in the cardiac silhouette. At this hospitalization, repeat angiography (Fig. 3) demonstrated that the right coronary artery had decreased in size and the collaterals were not as prominent or as abundant as before. The catheter was withdrawn to a position just proximal to the subclavian artery. This study demonstrated that the left subclavian directly perfused the left coronary artery (Fig. 4). The patient was discharged shortly thereafter and has continued to be asymptomatic at most recent follow-up 1V4 years (June 5, 1974) after the operation. Discussion Anomalous origin of the left coronary from the pulmonary artery can be divided into two clinical types: one in which the flow is antegrade from the pulmonary artery and the other in which the right coronary artery through collaterals supplies the left coronary artery and empties into the main pulmonary artery. The antegrade
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type is mainly seen in infants who have symptoms of severe coronary insufficiency. These symptoms are manifested especially when the infant is feeding. The mortality rate in this group is reported to be as high as 86 per cent. In the other group, in which collaterals are extensive, the children may be asymptomatic or have minimal symptoms. The concepts underlying medical and surgical care of these infants and children can and should now be critically revised. These infants in difficulty should undergo cardiac catheterization and be considered for surgical treatment. However, simple ligation of the anomalous coronary trunk at its origin should no longer be a surgical consideration. The establishment of a two-coronary system is superior and can be achieved in early infancy (Table I). Hemodynamic studies performed recently 12 demonstrate the advantage of aorta-coronary bypass over ligation of the anomalous vessels, as reflected in systolic and diastolic pressure measurements in the coronary vascular bed. The subclavian-coronary anastomosis negates the use of perfusion or vein grafts, a fact which makes correction feasible in the infant. In the older patient as in the case presented, we favor the coronary-subclavian anastomosis rather than the aorta-coronary vein bypass. The problems of vein thrombosis, stricture formation at two anastomotic sites, and lack of vein growth are thus avoided. The postoperative angiogram (Fig. 3) is the second known visualization of the left coronary system directly perfused by the left subclavian artery in end-to-end anastomosis. It reveals that in a relatively short time there is a most complete supply of the left coronary artery by way of the left subclavian artery and that the right coronary artery has diminished in size with decrease in the number of collaterals to the left coronary system. This case is presented in the hope that this procedure may be the procedure of choice for both the symptomatic infant and the older child with an anomalous left coronary artery arising from pulmonary artery. We feel that the direct anastomosis between the subclavian artery and the coronary artery has proved to be technically feasible and surgically successful. The vessel is capable of future growth and development in providing a two-coronary system necessary for satisfactory myocardial perfusion. REFERENCES 1 Mustard, W. T.: Anomalies of the Coronary Artery, in Berson, E. D., Mustard, W. T., and Rovitch, N. M.,
2
3 4 5
editors: Pediatric Surgery, vol. I, Chicago, 1953, Year Book Medical Publishers, Inc., p. 433. Kittle, C. F., Diehl, A. M., and Heilbrunn, A: Anomalous Left Coronary Artery Arising From the Pulmonary Artery: Report of Case and Surgical Consideration, J. Pediatr. 47: 198, 1955. Apley, J., Horton, R. E., and Wilson, M. G.: The Possible Role of Surgery in the Treatment of Anomalous Left Coronary Artery, Thorax 12: 23, 1957. Keith, J. D.: The Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery, Br. Heart J. 21: 149, 1959. Cooley, D. A., Hallman, G. L., and Bloodwell, R. D.: Definitive Surgical Treatment of Anomalous Origin of Left Coronary Artery From Pulmonary Artery: Indications and Results, J. THORAC. CARDIOVASC. SURG. 52:
798, 1966. 6 Nora, J. J., McNamara, D. G., Hallman, G. L., et al.: Medical and Surgical Management of Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery, Pediatrics 42: 405, 1968. 7 Meyer, B. W., Stefanik, G., Stiles, Q. R., Lindesmith. G. G., and Jones, J. C : A Method of Definitive Surgical Treatment of Anomalous Origin of Left Coronary Artery: A Case Report, J. THORAC. CARDIOVASC. SURG. 56: 104,
1968. 8 Perry, L. W., and Scott, L. P.: Anomalous Left Coronary Artery From Pulmonary Artery. Report of 11 Cases: Review of Indications for and Results of Surgery, Circulation 41: 1043, 1970. 9 Pinsky, W. W., Fagan, L. R., Kraeger, R. R., Mudd, J. F. G., and William V. L.: Anomalous Left Coronary Artery: Report of Two Cases, J. THORAC. CARDIOVASC. SURG. 65: 810,
1973.
10 Wesselhoeft, H., Fawcett, J. S., and Johnson, A. L.: Anomalous Origin of the Left Coronary Artery From the Pulmonary Trunk: Its Clinical Spectrum, Pathology, and Pathophysiology, Based on a Review of 140 Cases With Seven Further Cases, Circulation 38: 403, 1968. 11 Likar, I., Criley, J. M., and Lewis, K. B.: Anomalous Left Coronary Artery Arising From the Pulmonary Artery in an Adult: A Review of the Therapeutic Problem, Circulation 33: 727, 1966. 12 Endo, M., Takayasu, S., Obunai, Y., Nakazawa, M., and Konno, S.: Anomalous Origin of Left Coronary Artery From Pulmonary Artery, J. THORAC. CARDIOVASC. SURG. 67: 896,
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13 El-Said, G. M., Ruzyllo, W., Williams, R. L., et al.: Early and Late Results of Saphenous Vein Graft for Anomalous Origin of Left Coronary Artery From Pulmonary Artery, Circulation 48: 2, 1973 (Suppl. III).