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Anomalous origin of the left coronary artery from the pulmonary artery in an adult
Anomalous origin of the left coronary artery from the pulmonary artery in an adult Report of a case treated by aorto-coronary bypass with the saphenous vein Nasim Akhtar, M.D., John W. Hyland, M.D., and Maurice Adam, M.D., Dallas, Texas
Anomalous ongm of the coronary artery is a rare congenital abnormality. Origin of the left coronary artery from the pulmonary artery represents the commonest form of this unusual lesion. Since the description of this abnormality by Abbott,' a number of cases have been reported in infants and children. However, reports of such cases in the adult age group (above 18 years of age) are few since only 15 per cent of these patients survive beyond infancy." The mean life expectancy in the adult age group is approximately 35 years." Due to the availability of surgical treatment, the diagnosis of this rare anomaly during life is of more than academic interest. Until recently the timehonored treatment for patients with this abnormality had been the ligation of the anomalous left coronary artery. However, in 1966 Cooley and associates" reported From the Division of Cardiology and Internal Medicine and the Departments of Thoracic and Cardiovascular Surgery, Baylor University Medical Center, Dallas, Texas 75246. Received for publication April 21, 1973. Address for reprints: Dr. Nasim Akhtar, Veterans Administration
Hospital,
Washington, D. C. 20422.
112
50
Irving
Street,
N.W.,
treating 2 patients by anastomosis of the anomalous left coronary artery to the aorta, done with a Dacron graft in 1 case and an autologous saphenous vein in the other; since that time, a few more such cases have been reported.>" The purpose of this paper is to describe the case history of a 49-year-old patient with this anomaly who was treated by an aortocoronary bypass graft with the saphenous vein. To the best of our knowledge, she is the oldest patient with this anomaly (anomalous left coronary artery arising from the pulmonary artery) to have the diagnosis made during life and be treated by aortocoronary anastomosis with a saphenous vein graft. Case report B. D., a 49-year-old white woman, was admitted to Baylor University Medical Center for the first time on Sept. 24, 1971, with a history of episodes of palpitation for the past 5 years. These periods were sudden in onset, lasted for 30 to 40 minutes, and at times were associated with dizziness and chest pain. One episode in August of 1971 lasted for 3 hours and prompted her admission to a local hospital, where she was found to be in a state of atrial flutter with
Volume 66 Number 1 July, 1973
Anomalous origin of coronary artery
113
Fig. 1. Preoperative electrocardiogram demonstrating Q wave in Lead aVL and diffuse ST-T changes.
1.
AVR
2.
AVL
tT
Fig. 2. Electrocardiogram after 6 minutes of exercise showing an increase in ST-T depression. varying degrees of block which converted spontaneously to normal sinus rhythm. During this hospitalization she developed various kinds of atrial arrhythmias and premature ventricular beats. She was treated with antiarrhythmic drugs without significant improvement. The patient denied any chest pain except during the arrhythmias and had no shortness of breath or ankle edema. The family history revealed that two brothers had died suddenly during exertion at the ages of 12 and 19 years. Physical examination showed a healthy woman in no acute distress. The blood pressure was 130/60 mm. Hg, The apex pulse was 60 beats per minute with occasional premature beats. There was no cyanosis or clubbing. Neck veins were not distended. The lungs were clear, and the liver and spleen were not palpable. Examina-
tion of the heart revealed that the point of maximal impulse was in the fifth left intercostal space in the midclavicular line. The heart sounds were normal. A soft third heart sound was heard at the apex, and there was a Grade 1/6 ejection systolic murmur at the apex. Chest x-ray study revealed slight left ventricular enlargement. The electrocardiogram showed normal sinus rhythm with left atrial enlargement (Fig. 1). There was a prominent Q wave in Lead aVL, and there were ST-T changes in the anterolateral leads. After 6 minutes of a bicycle exercise stress test, the ST depression became more prominent although the patient did not develop any chest pain (Fig. 2). Phonocardiography confirmed the auscultatory findings. Right and left heart catheterization and coronary angiography were performed on Sept. 30,
114
The Journal of
Akhtar, Hyland, Adam
Thoracic and Cardiovascular Surgery
Over the next several days the cardiac rhythm varied from atrial flutter to atrial fibrillation. Large doses of digoxin were used. Finally the rhythm became normal. Otherwise her recovery was uneventful. Since discharge the patient has been completely asymptomatic. An 8 month follow-up showed no further episodes of palpitation, dizziness, or chest pain.
Discussion
Fig. 3. Preoperative selective right coronary arteriogram demonstrating a large right coronary artery (RCA) communicating with the left coronary artery through collateral vessels. The left coronary artery is filled from the right side in a retrograde manner and is draining into the pulmonary artery (PA). ALCA, Anomalous left coronary artery. 1971. Right and left heart pressures were normal. There was no evidence of an intracardiac shunt. Coronary angiography revealed an anomalous left coronary artery arising from the pulmonary artery. The right coronary artery was dominant, large, and tortuous. It communicated with the left coronary artery through large collateral vessels. The left coronary artery filled from the right side in a retrograde manner and drained into the pulmonary artery (Fig. 3) . Surgical correction was accomplished on Nov. I 1, 1971. The orifice of the anomalous left main coronary was situated on the posterior left lateral aspect of the pulmonary artery. An atrial branch arose close to the origin, and just distal to this point, 1.3 cm. from the pulmonary artery, the short left main coronary divided into the circumflex and left anterior descending arteries. The left anterior descending artery was opened near its origin. Proximally, the artery was 4 mm. in diameter. Distally, a 3 mm. probe was easily passed into the vessel. Correction consisted of closure of the orifice of the left main coronary artery and creation of a bypass graft between the aorta and the proximal quarter of the left anterior descending coronary artery with a saphenous vein segment (5 mm. in diameter). Flow through the bypass graft was found by electromagnetic flowmeter to be 240/0 with a mean flow of 150 c.c. per minute. Postoperatively, the patient had frequent premature atrial contractions and occasional bursts of atrial tachycardia. Digitalization was begun.
Bland and associates" in 1933 first described the clinical syndrome produced by this anomaly. Agustsson and co-workers" divided these patients into two groups, those with the infantile and those with the adult types. In the infantile type, the symptoms are usually those of congestive heart failure with cardiomegaly. Mitral insufficiency may also be present. Wesselhoeft's group," in a review of 140 cases, found 105 infants with symptoms resembling angina or indicating cardiomyopathy which they called the "infant syndrome." A total of 88 per cent of these patients died during the first year. There were 11 infants with mitral insufficiency. In the adult type, the patient may present with a systolic, a diastolic, or a continuous murmur.t ' The continuous murmur may be mistaken for patent ductus arteriosus. This condition may also masquerade as mitral insufficiency.P It is very essential that the cause of mitral insufficiency be ascertained before operation because a misdiagnosis could lead to fatal results. The electrocardiogram in these patients usually reveals a leftward axis, a prominent Q wave in Leads I and aV L, abnormal T waves, and voltage criteria for left ventricular hypertrophy. The chest x-ray film may be normal or show left ventricular and left atrial enlargement. There is another group of adult patients who are relatively healthy, without any significant cardiac symptoms, but who die suddenly, primarily during exertion.!" Presence of arrhythmias in these patients is regarded by some as a warning of the threat of sudden death from ventricular fibrillation.':' Because of this hazard of sudden death and the presence of arrhythmias, we decided that surgical inter-
Volume 66 Number 1
Anomalous origin of coronary artery
1 15
July, 1973
vention was indicated in our patient. Two of her brothers died suddenly at the ages of 12 and 19 years. Whether they had the same abnormality in their coronary circulations was not known as no autopsies had been performed. Brooks," in 1886 and Abbott ' in 1908 were the first to point out that the flow of blood in an anomalous coronary artery is toward the pulmonary artery. This was later re-emphasized by Edwards" and confirmed during surgery and aortography or selective right coronary angiography. Ligation of the aberrant coronary artery as a treatment is based on this observation. Edwards,"; Nadas," Talner," and their associates described three hemodynamic phases of this abnormality. In the initial phase, which exists during the fetal and newborn periods when the pulmonary arterial pressure is high, the flow of blood is from the pulmonary artery to the left coronary artery. The infant is asymptomatic at birth and for a short time afterward. The second phase is a critical transition period. The pulmonary artery pressure becomes normal, and survival depends upon the development of adequate collaterals. During this phase, signs and symptoms of myocardial ischemia may be present. In the third phase of the clinical spectrum, rich collateral circulation is present permitting survival into adult life. Baue and associates" recently suggested a fourth phase, in which there is an exaggeration of the intercoronary anastomosis between the right and left coronary artery. This produces such a large arteriovenous shunt that blood flow to the myocardium is again reduced, resulting in a "coronary steal syndrome.' As pointed out earlier, most of these patients die in infancy. Those who survive may have symptoms of angina or congestive heart failure or may remain completely asymptomatic. Due to the danger of sudden death in these patients, the symptoms of ischemia or arrhythmia are regarded as indications for surgical treatment. Operations which have been advocated for the treatment of anomalous left coro-
nary artery from the pulmonary artery have been reviewed recently by Cooley's group' and include main pulmonary artery constriction, creation of a systemic-pulmonary shunt, pericardial poudrage and de-epicardialization, and ligation of the anomalous vessel. Likar-" reported 16 survivors among 27 patients who underwent ligation. In the absence of adequate collateral circulation between the right and the left coronary arteries, ligation has frequently resulted in death and should not be attempted. Transplantation of the left coronary artery is a more direct approach to the treatment of this anomaly, producing a two coronary arterial system. Mustard-' anastomosed the left coronary artery to the left common carotid artery. More recently, Meyer and his associates" anastomosed the left coronary artery to the left subclavian artery. Cooley's group' used a Dacron graft to achieve a two coronary system and later used a saphenous vein bypass graft between the aorta and coronary artery. Reis and associates" performed the same operation on a 20-year-old man and observed that the pattern of instantaneous coronary flow and its relation to ventricular and aortic pressure were similar to the pattern that has been observed in normal animals. Somerville and Ross" showed improvement in ventricular function after bypass surgery. Our patient is the oldest reported patient to undergo this kind of operation. Flow measurements at the time of operation showed the large amount of myocardial flow which the left coronary system can accept. The establishment of a two coronary arterial system restores normal anatomy and results in normal circulation in the coronary artery. It appears more desirable; however, because of the rarity of this lesion and the small number of patients operated upon, it is not possible to compare the results of ligation with those of anastomosis. However, long-term follow-up of these patients and comparison of longevity between patients with a two coronary arterial system and those with ligation of the anomalous left
116
The Journal of Thoracic and Cardiovascular Surgery
Akhtar, Hyland, Adam
coronary artery should yield more conclusive information.
Summary Anomalous ongm of the left coronary artery from the pulmonary artery, an unusual cardiovascular lesion, can be surgically managed. Ligation of the anomalous left coronary artery has been the method of choice, but it should not be attempted in the absence of adequate intercoronary anastomoses. Aorto-coronary bypass offers a more definitive approach, establishing a two coronary arterial system. In this paper, we have described a 49year-old patient with this anomaly who was treated by coronary bypass graft with an autogenous saphenous vein. The clinical manifestations and pathophysiology of this condition are briefly discussed. REFERENCES
2
3
4
5
6
7
8
Abbott, M. E.: Congenital Cardiac Disease, in Osler's Modern Medicine, vo!. 4, Philadelphia, 1908, Lea & Febiger, Publishers, p. 296. Harthrone, J. W., Scannell, J. G., and Dinsmore, R. E.: Anomalous Origin of the Left Coronary Artery, N. Eng!. J. Med. 275: 660, 1966. George, 1. M., and Knowlen, D. M.: Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery in an Adult, N. Eng!. J. Med. 261: 993, 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. Reis, R. L., Cohen, L. S., and Mason, D. T.: Direct Measurement of Instantaneous Coronary Blood Flow After Total Correction of Anomalous Left Coronary Artery, Circulation 39: 1, 1969. Somerville, J., and Ross, D. N.: Left Coronary Artery From the Pulmonary Artery: Physiological Consideration of Surgical Correction, Thorax 25: 207, 1970. Gaisor, R. M., Winters, W. L., Glick, H., Sandiford, F., Chapman, D. W., and Morris, G. C.: Anomalous Origin of Left Coronary Artery From Pulmonary Artery: Treatment by Aorto-Left Coronary Saphenous Vein Bypass, Am. J. Cardio!. 27: 215, 1971. Bland, E. F., White, P. D., and Garland, J.:
9
10
II 12
13 14
15
16 17
18
19
Congenital Anomalies of the Coronary Arteries: Report of an Unusual Case Associated With Cardiac Hypertrophy, Am. Heart J. 8: 787, 1933. Agustsson, M. H., Gasul, B. M., Fell, E. H., Graettinger, J. S., Waterman, D. F., and Bicoff, 1. P.: Anomalous Origin of Left Coronary Artery From Pulmonary Artery: Diagnosis and Treatment of Infantile and Adults Types, J. A. M. A. 180: 15, 1962. Wessel hoeft, 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. Bayliss, J. H., and Campbell, M: Unusual Cause for a Continuous Murmur, Guys Hosp. Rep. 101: 174, 1952. Burchell, H. B., and Brown, A L., Jr.: Anomalous Origin of the Coronary Artery From the Pulmonary Artery Masquerading as Mitral Insufficiency, Am. Heart J. 63: 388, 1962. Ruddock, J. C., and Stehly, C. c.: Anomalous Origin of Left Coronary Artery: Case Report, U. S. Nav. Med. Bull. 41: 175, 1943. Wright, N. L., Baue, A E., Baum, S., Blakemore, W. S., and Zinsser, H. F.: Coronary Artery Steal Due to an Anomalous Left Coronary Artery Originating From the Pulmonary Artery, 1. THoRAc. CARDIOVASC. SURG. 59: 461, 1970. Brooks, H. St. J.: Two Cases of an Abnormal Coronary Artery of the Heart Arising From the Pulmonary Artery: With Some Remarks Upon the Effect of This Anomaly in Producing Cricoid Dilatation of the Vessels, 1. Anat. Physio!. 20: 26, 1886. Edwards, J. E.: Direction of Blood Flow in Coronary Arteries Arising From the Pulmonary Trunk, Circulation 29: 163, 1964. Nadas, A. S., Gamboa, R., and Hugenholtz, P. G.: Anomalous Left Coronary Artery Originating From the Pulmonary Artery. Report of Two Surgically Treated Cases With a Proposal of Hemodynamic and Therapeutic Classification, Circulation 29: 167, 1964. Talner, N. S., Halloran, K. H., Mahdavy, M., Gardner, T. H., and Hipona, F.: Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery: A Clinical Spectrum, Am. J. Cardio!. 15: 689, 1965. Baue, A. E., Baurn, S., Blakemore, W. S., and Zinsser, H. F.: A Later Stage of Anomalous Coronary Circulation With Origin of Left Coronary Artery From the Pulmonary Artery: Coronary Artery Steal, Circulation 36: 878, 1967.
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20 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. 21 Mustard, W. T.: Anomalies of the Coronary Artery in Pediatric Surgery, vol. 1, Chicago,
1962, Year Book Medical Publishers, Inc., p.433. 22 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, J. THoRAc. CARDIOVASC. SURG. 56: 104, 1968.
Anomalous ongm of the coronary artery is a rare congenital abnormality. Origin of the left coronary artery from the pulmonary artery represents the commonest form of this unusual lesion. Since the description of this abnormality by Abbott,' a number of cases have been reported in infants and children. However, reports of such cases in the adult age group (above 18 years of age) are few since only 15 per cent of these patients survive beyond infancy." The mean life expectancy in the adult age group is approximately 35 years." Due to the availability of surgical treatment, the diagnosis of this rare anomaly during life is of more than academic interest. Until recently the timehonored treatment for patients with this abnormality had been the ligation of the anomalous left coronary artery. However, in 1966 Cooley and associates" reported From the Division of Cardiology and Internal Medicine and the Departments of Thoracic and Cardiovascular Surgery, Baylor University Medical Center, Dallas, Texas 75246. Received for publication April 21, 1973. Address for reprints: Dr. Nasim Akhtar, Veterans Administration
Hospital,
Washington, D. C. 20422.
112
50
Irving
Street,
N.W.,
treating 2 patients by anastomosis of the anomalous left coronary artery to the aorta, done with a Dacron graft in 1 case and an autologous saphenous vein in the other; since that time, a few more such cases have been reported.>" The purpose of this paper is to describe the case history of a 49-year-old patient with this anomaly who was treated by an aortocoronary bypass graft with the saphenous vein. To the best of our knowledge, she is the oldest patient with this anomaly (anomalous left coronary artery arising from the pulmonary artery) to have the diagnosis made during life and be treated by aortocoronary anastomosis with a saphenous vein graft. Case report B. D., a 49-year-old white woman, was admitted to Baylor University Medical Center for the first time on Sept. 24, 1971, with a history of episodes of palpitation for the past 5 years. These periods were sudden in onset, lasted for 30 to 40 minutes, and at times were associated with dizziness and chest pain. One episode in August of 1971 lasted for 3 hours and prompted her admission to a local hospital, where she was found to be in a state of atrial flutter with
Volume 66 Number 1 July, 1973
Anomalous origin of coronary artery
113
Fig. 1. Preoperative electrocardiogram demonstrating Q wave in Lead aVL and diffuse ST-T changes.
1.
AVR
2.
AVL
tT
Fig. 2. Electrocardiogram after 6 minutes of exercise showing an increase in ST-T depression. varying degrees of block which converted spontaneously to normal sinus rhythm. During this hospitalization she developed various kinds of atrial arrhythmias and premature ventricular beats. She was treated with antiarrhythmic drugs without significant improvement. The patient denied any chest pain except during the arrhythmias and had no shortness of breath or ankle edema. The family history revealed that two brothers had died suddenly during exertion at the ages of 12 and 19 years. Physical examination showed a healthy woman in no acute distress. The blood pressure was 130/60 mm. Hg, The apex pulse was 60 beats per minute with occasional premature beats. There was no cyanosis or clubbing. Neck veins were not distended. The lungs were clear, and the liver and spleen were not palpable. Examina-
tion of the heart revealed that the point of maximal impulse was in the fifth left intercostal space in the midclavicular line. The heart sounds were normal. A soft third heart sound was heard at the apex, and there was a Grade 1/6 ejection systolic murmur at the apex. Chest x-ray study revealed slight left ventricular enlargement. The electrocardiogram showed normal sinus rhythm with left atrial enlargement (Fig. 1). There was a prominent Q wave in Lead aVL, and there were ST-T changes in the anterolateral leads. After 6 minutes of a bicycle exercise stress test, the ST depression became more prominent although the patient did not develop any chest pain (Fig. 2). Phonocardiography confirmed the auscultatory findings. Right and left heart catheterization and coronary angiography were performed on Sept. 30,
114
The Journal of
Akhtar, Hyland, Adam
Thoracic and Cardiovascular Surgery
Over the next several days the cardiac rhythm varied from atrial flutter to atrial fibrillation. Large doses of digoxin were used. Finally the rhythm became normal. Otherwise her recovery was uneventful. Since discharge the patient has been completely asymptomatic. An 8 month follow-up showed no further episodes of palpitation, dizziness, or chest pain.
Discussion
Fig. 3. Preoperative selective right coronary arteriogram demonstrating a large right coronary artery (RCA) communicating with the left coronary artery through collateral vessels. The left coronary artery is filled from the right side in a retrograde manner and is draining into the pulmonary artery (PA). ALCA, Anomalous left coronary artery. 1971. Right and left heart pressures were normal. There was no evidence of an intracardiac shunt. Coronary angiography revealed an anomalous left coronary artery arising from the pulmonary artery. The right coronary artery was dominant, large, and tortuous. It communicated with the left coronary artery through large collateral vessels. The left coronary artery filled from the right side in a retrograde manner and drained into the pulmonary artery (Fig. 3) . Surgical correction was accomplished on Nov. I 1, 1971. The orifice of the anomalous left main coronary was situated on the posterior left lateral aspect of the pulmonary artery. An atrial branch arose close to the origin, and just distal to this point, 1.3 cm. from the pulmonary artery, the short left main coronary divided into the circumflex and left anterior descending arteries. The left anterior descending artery was opened near its origin. Proximally, the artery was 4 mm. in diameter. Distally, a 3 mm. probe was easily passed into the vessel. Correction consisted of closure of the orifice of the left main coronary artery and creation of a bypass graft between the aorta and the proximal quarter of the left anterior descending coronary artery with a saphenous vein segment (5 mm. in diameter). Flow through the bypass graft was found by electromagnetic flowmeter to be 240/0 with a mean flow of 150 c.c. per minute. Postoperatively, the patient had frequent premature atrial contractions and occasional bursts of atrial tachycardia. Digitalization was begun.
Bland and associates" in 1933 first described the clinical syndrome produced by this anomaly. Agustsson and co-workers" divided these patients into two groups, those with the infantile and those with the adult types. In the infantile type, the symptoms are usually those of congestive heart failure with cardiomegaly. Mitral insufficiency may also be present. Wesselhoeft's group," in a review of 140 cases, found 105 infants with symptoms resembling angina or indicating cardiomyopathy which they called the "infant syndrome." A total of 88 per cent of these patients died during the first year. There were 11 infants with mitral insufficiency. In the adult type, the patient may present with a systolic, a diastolic, or a continuous murmur.t ' The continuous murmur may be mistaken for patent ductus arteriosus. This condition may also masquerade as mitral insufficiency.P It is very essential that the cause of mitral insufficiency be ascertained before operation because a misdiagnosis could lead to fatal results. The electrocardiogram in these patients usually reveals a leftward axis, a prominent Q wave in Leads I and aV L, abnormal T waves, and voltage criteria for left ventricular hypertrophy. The chest x-ray film may be normal or show left ventricular and left atrial enlargement. There is another group of adult patients who are relatively healthy, without any significant cardiac symptoms, but who die suddenly, primarily during exertion.!" Presence of arrhythmias in these patients is regarded by some as a warning of the threat of sudden death from ventricular fibrillation.':' Because of this hazard of sudden death and the presence of arrhythmias, we decided that surgical inter-
Volume 66 Number 1
Anomalous origin of coronary artery
1 15
July, 1973
vention was indicated in our patient. Two of her brothers died suddenly at the ages of 12 and 19 years. Whether they had the same abnormality in their coronary circulations was not known as no autopsies had been performed. Brooks," in 1886 and Abbott ' in 1908 were the first to point out that the flow of blood in an anomalous coronary artery is toward the pulmonary artery. This was later re-emphasized by Edwards" and confirmed during surgery and aortography or selective right coronary angiography. Ligation of the aberrant coronary artery as a treatment is based on this observation. Edwards,"; Nadas," Talner," and their associates described three hemodynamic phases of this abnormality. In the initial phase, which exists during the fetal and newborn periods when the pulmonary arterial pressure is high, the flow of blood is from the pulmonary artery to the left coronary artery. The infant is asymptomatic at birth and for a short time afterward. The second phase is a critical transition period. The pulmonary artery pressure becomes normal, and survival depends upon the development of adequate collaterals. During this phase, signs and symptoms of myocardial ischemia may be present. In the third phase of the clinical spectrum, rich collateral circulation is present permitting survival into adult life. Baue and associates" recently suggested a fourth phase, in which there is an exaggeration of the intercoronary anastomosis between the right and left coronary artery. This produces such a large arteriovenous shunt that blood flow to the myocardium is again reduced, resulting in a "coronary steal syndrome.' As pointed out earlier, most of these patients die in infancy. Those who survive may have symptoms of angina or congestive heart failure or may remain completely asymptomatic. Due to the danger of sudden death in these patients, the symptoms of ischemia or arrhythmia are regarded as indications for surgical treatment. Operations which have been advocated for the treatment of anomalous left coro-
nary artery from the pulmonary artery have been reviewed recently by Cooley's group' and include main pulmonary artery constriction, creation of a systemic-pulmonary shunt, pericardial poudrage and de-epicardialization, and ligation of the anomalous vessel. Likar-" reported 16 survivors among 27 patients who underwent ligation. In the absence of adequate collateral circulation between the right and the left coronary arteries, ligation has frequently resulted in death and should not be attempted. Transplantation of the left coronary artery is a more direct approach to the treatment of this anomaly, producing a two coronary arterial system. Mustard-' anastomosed the left coronary artery to the left common carotid artery. More recently, Meyer and his associates" anastomosed the left coronary artery to the left subclavian artery. Cooley's group' used a Dacron graft to achieve a two coronary system and later used a saphenous vein bypass graft between the aorta and coronary artery. Reis and associates" performed the same operation on a 20-year-old man and observed that the pattern of instantaneous coronary flow and its relation to ventricular and aortic pressure were similar to the pattern that has been observed in normal animals. Somerville and Ross" showed improvement in ventricular function after bypass surgery. Our patient is the oldest reported patient to undergo this kind of operation. Flow measurements at the time of operation showed the large amount of myocardial flow which the left coronary system can accept. The establishment of a two coronary arterial system restores normal anatomy and results in normal circulation in the coronary artery. It appears more desirable; however, because of the rarity of this lesion and the small number of patients operated upon, it is not possible to compare the results of ligation with those of anastomosis. However, long-term follow-up of these patients and comparison of longevity between patients with a two coronary arterial system and those with ligation of the anomalous left
116
The Journal of Thoracic and Cardiovascular Surgery
Akhtar, Hyland, Adam
coronary artery should yield more conclusive information.
Summary Anomalous ongm of the left coronary artery from the pulmonary artery, an unusual cardiovascular lesion, can be surgically managed. Ligation of the anomalous left coronary artery has been the method of choice, but it should not be attempted in the absence of adequate intercoronary anastomoses. Aorto-coronary bypass offers a more definitive approach, establishing a two coronary arterial system. In this paper, we have described a 49year-old patient with this anomaly who was treated by coronary bypass graft with an autogenous saphenous vein. The clinical manifestations and pathophysiology of this condition are briefly discussed. REFERENCES
2
3
4
5
6
7
8
Abbott, M. E.: Congenital Cardiac Disease, in Osler's Modern Medicine, vo!. 4, Philadelphia, 1908, Lea & Febiger, Publishers, p. 296. Harthrone, J. W., Scannell, J. G., and Dinsmore, R. E.: Anomalous Origin of the Left Coronary Artery, N. Eng!. J. Med. 275: 660, 1966. George, 1. M., and Knowlen, D. M.: Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery in an Adult, N. Eng!. J. Med. 261: 993, 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. Reis, R. L., Cohen, L. S., and Mason, D. T.: Direct Measurement of Instantaneous Coronary Blood Flow After Total Correction of Anomalous Left Coronary Artery, Circulation 39: 1, 1969. Somerville, J., and Ross, D. N.: Left Coronary Artery From the Pulmonary Artery: Physiological Consideration of Surgical Correction, Thorax 25: 207, 1970. Gaisor, R. M., Winters, W. L., Glick, H., Sandiford, F., Chapman, D. W., and Morris, G. C.: Anomalous Origin of Left Coronary Artery From Pulmonary Artery: Treatment by Aorto-Left Coronary Saphenous Vein Bypass, Am. J. Cardio!. 27: 215, 1971. Bland, E. F., White, P. D., and Garland, J.:
9
10
II 12
13 14
15
16 17
18
19
Congenital Anomalies of the Coronary Arteries: Report of an Unusual Case Associated With Cardiac Hypertrophy, Am. Heart J. 8: 787, 1933. Agustsson, M. H., Gasul, B. M., Fell, E. H., Graettinger, J. S., Waterman, D. F., and Bicoff, 1. P.: Anomalous Origin of Left Coronary Artery From Pulmonary Artery: Diagnosis and Treatment of Infantile and Adults Types, J. A. M. A. 180: 15, 1962. Wessel hoeft, 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. Bayliss, J. H., and Campbell, M: Unusual Cause for a Continuous Murmur, Guys Hosp. Rep. 101: 174, 1952. Burchell, H. B., and Brown, A L., Jr.: Anomalous Origin of the Coronary Artery From the Pulmonary Artery Masquerading as Mitral Insufficiency, Am. Heart J. 63: 388, 1962. Ruddock, J. C., and Stehly, C. c.: Anomalous Origin of Left Coronary Artery: Case Report, U. S. Nav. Med. Bull. 41: 175, 1943. Wright, N. L., Baue, A E., Baum, S., Blakemore, W. S., and Zinsser, H. F.: Coronary Artery Steal Due to an Anomalous Left Coronary Artery Originating From the Pulmonary Artery, 1. THoRAc. CARDIOVASC. SURG. 59: 461, 1970. Brooks, H. St. J.: Two Cases of an Abnormal Coronary Artery of the Heart Arising From the Pulmonary Artery: With Some Remarks Upon the Effect of This Anomaly in Producing Cricoid Dilatation of the Vessels, 1. Anat. Physio!. 20: 26, 1886. Edwards, J. E.: Direction of Blood Flow in Coronary Arteries Arising From the Pulmonary Trunk, Circulation 29: 163, 1964. Nadas, A. S., Gamboa, R., and Hugenholtz, P. G.: Anomalous Left Coronary Artery Originating From the Pulmonary Artery. Report of Two Surgically Treated Cases With a Proposal of Hemodynamic and Therapeutic Classification, Circulation 29: 167, 1964. Talner, N. S., Halloran, K. H., Mahdavy, M., Gardner, T. H., and Hipona, F.: Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery: A Clinical Spectrum, Am. J. Cardio!. 15: 689, 1965. Baue, A. E., Baurn, S., Blakemore, W. S., and Zinsser, H. F.: A Later Stage of Anomalous Coronary Circulation With Origin of Left Coronary Artery From the Pulmonary Artery: Coronary Artery Steal, Circulation 36: 878, 1967.
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20 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. 21 Mustard, W. T.: Anomalies of the Coronary Artery in Pediatric Surgery, vol. 1, Chicago,
1962, Year Book Medical Publishers, Inc., p.433. 22 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, J. THoRAc. CARDIOVASC. SURG. 56: 104, 1968.