- Email: [email protected]
Anomalous origin of left coronary artery from pulmonary artery
Anomalous origin of left coronary artery from pulmonary artery Case report and review of literature concerning teen-agers and adults An adult with angina was found to have anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Review of the literature regarding this anomaly in teen-agers and adults disclosed only 25 cases diagnosed during life. Eighteen additional cases of ALCAPA in this age group have been diagnosed post mortem. In this report, we will review the management of teen-agers and adults in whom ALCAPA was correctly diagnosed during life. We shall also describe the eighth case of successful aorta-left coronary artery grafting with the saphenous vein in this age group. This case brings the total in the literature to 44. Of those patients offered surgical therapy, 13 underwent successful ligation of the anomalous artery. Saphenous vein grafts were employed in 8. Five did not undergo ligation or saphenous vein grafting. There was one death. It would appear that saphenous vein grafting is the definitive means of surgically correcting ALCAPA, because it restores the dual coronary circulation.
Charles L. Wilson, M . D . , F . A . C . C , Paul W. Dlabal, M.D., Roy W. Holeyfield, M.D., Cary W. Akins, M.D., and Daniel G. Knauf, M.D., Lackland Air Force Base, Texas
A nomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiovascular defect. Over two hundred cases have been reported during the past century. 1 ' 2 The mortality rate among infants and children without operation has been eighty to ninety per cent. 2 Survival to teen-age and adult life has been infrequent; only forty-four cases including this case have been reported in this age group. Prior to corrective surgical therapy, eighty to ninety per cent of the adults died suddenly at a mean age of thirty-five years. 3 , 4 Less commonly, patients survived with mitral regurgitation or heart failure. Development of angina pectoris is infrequent in older patients with ALCAPA. 2
From the Cardiology Service, Department of Medicine, and the Thoracic Surgery Service, Department of Surgery, Wilford Hall USAF Medical Center, Lackland Air Force Base, Texas 78236. The opinions expressed are those of the authors and do not necessarily represent official USAF policy. Received for publication Nov. 10, 1976. Accepted for publication Jan. 12, 1977. Address for reprints: C. L. Wilson, M.D., 9 Flintstone Court, San Antonio, Texas 78213.
Long-term survival depends upon the development of collateral vessels from the right coronary artery which are adequate to perfuse the myocardium in the territory of the anomalous left coronary artery. Additionally, there must be no significant steal of flow into the pulmonary artery from the myocardium supplied by the ALCAPA. Various authors have classified patients with ALCAPA into infant and adult groups. 5 " 6 The groupings are arbitrary. Occasionally, infants meet adult criteria. The clinical condition of those surviving to teen-age resembles that of adults. All patients 13 years of age and older, who have been studied, have had ALCAPA of the adult type. We have chosen to study patients aged 13 or older in order to expand the data base of the adult population. Previously, 20 teen-aged and adult patients have had surgical correction of ALCAPA. We are reporting a case which is unique because of the duration of symptoms. This patient was the eighth to have successful aorta-left coronary artery grafting with the saphenous vein in this group. Postoperatively, a new diastolic murmur was heard, which may reflect the torrential diastolic intracoronary blood flow. Both ligation and aorta-left coronary artery grafting with the saphenous 887
888
The Journal of Thoracic and Cardiovascular Surgery
Wilson et al.
IL_——Jl
^MMv4_4__^
m
^ - ^ - | V _ ^ - - ^ ^—y^ p-y*-
l-^W** III S A M ^ M J ^ ^ J H ^
Fig. 1. Preoperative electrocardiogram, taken in February of 1976, and tracings from 1955 and 1960 are identical. Table I. Cardiac catheterization compared with 1976
data: 1956
Oxygen saturation (%) Location
1956
SVC IVC RA RV MPA PCW
64 79 74 67 75
Radial Aorta LVED QSI QPI
95
1976 (preop.) 70 71 75 72 82
98.6 3.6 3.6
Pressures (mm Hg) 1956
26/1-6* 19/10-17t 10t (rest) 15% (after exercise) 105/58-78t
1976 (preop.)
24/0-7 24/12-16t
110/60-85 110/0, 13*
2.6 3.9
Legend: SVC, Superior vena cava. IVC, Inferior vena cava. RA, Right atrium. RV, Right ventricle. MPA, Main pulmonary artery. PCW, Pulmonary capillary wedge. LVED, Left ventricular end-diastolic. QSI, Systemic flow index. QPI, Pulmonary flow index. *End-diastolic pressure. tMean pressure.
vein relieve symptoms and perhaps prolong life in patients with ALCAPA. Saphenous vein grafting appears to be the definitive surgical correction for this anomaly. Because surgical correction is available, it is essential that the diagnosis be made during life. Case report J. S., a 42-year-old white woman, was referred in February, 1976, for evaluation of two decades of intermittent
angina pectoris and a recent syncopal episode. The patient described the onset of joint pains at the age of 7 years. A heart murmur was first noted then. She was healthy until the age of 23 years, when, during her third pregnancy, shortness of breath and exertional dyspnea developed, without paroxysmal nocturnal dyspnea, orthopnea, or edema. Because systolic and diastolic heart murmurs were heard and because she had angina pectoris, she was first referred in 1955 to a diagnostic medical center in New England. A Grade 3/6 systolic murmur and a Grade 2/6 diastolic murmur were noted. The murmurs were most intense in the left third and fourth intercostal spaces. The electrocardiogram indicated an anterolateral myocardial infarction (Fig. 1). Right heart catheterization in September, 1956, revealed no abnormalities (Table I). No left-to-right shunt was detected, and no diagnosis was established. Following delivery of a healthy infant, she became asymptomatic and remained so until the age of 41 years, when exertional dyspnea and generalized fatigue developed. She then noted a recurrence of angina pectoris precipitated by emotion and exertion and relieved by rest. The pain occasionally radiated to both sides of the neck, but shortness of breath, diaphoresis, and palpitations never developed. In December, 1975, the patient felt a pressure in her chest after eating. She became nauseated and fainted. She awakened in the emergency room of a small community hospital. The woman was examined and released that night without further diagnostic studies. Physical examination on admission to Wilford Hall USAF Medical Center disclosed a healthy appearing, short, white woman, height 153 cm. and weight 56.6 kilograms. Her temperature was 36.8° C. The pulse rate was 72 beats per minute and regular and the respiratory rate 16 breaths per minute. Blood pressure was 118/80 mm. Hg in both arms. Jugular venous and carotid pulses and contours were within normal limits. The point of maximum impulse was normal. The first heart sound was normal, and the second heart sound was physiologically split. A Grade 3/6 ejection murmur was heard through the first two thirds of systole. It was loudest in
Volume 73 Number 6 June, 1977
the fourth intercostal space at the left sternal border. An intermittent Grade 1 /6 early diastolic decrescendo murmur was reported by some observers. The systolic murmur decreased with amyl nitrite and during a Valsalva maneuver, but it returned within 10 beats after release of the maneuver. The electrocardiogram (Fig. 1) demonstrated Q waves in leads I and L and poor R-wave progression in Leads Vj to V3. The tracing was unchanged from the electrocardiograms taken between 1955 and 1960. The vectorcardiogram revealed the horizontal loop to be posterior, but within normal limits. The chest x-ray film was considered normal. The phonocardiogram showed a normal carotid contour and a long systolic murmur continuing beyond the second sound, and a fourth heart sound was recorded. The QS2 interval was 450 msec, left ventricular ejection time (LVET) 285 msec, and pre-ejection period (PEP) 130 msec. The PEP/LVET of 45 per cent was increased, an indication of left ventricular dysfunction. An exercise tolerance test was not performed since there is a close association between vigorous exercise and sudden death in teen-agers and adults with ALCAPA.12, n The history of recent syncope was a further contraindication. ALCAPA was a major consideration before catheterization, as was a high ventricular septal defect. In February, 1976, the patient underwent a second cardiac catheterization (Table I). The right-sided pressures were unchanged. The cardiac index had decreased from 3.6 to 2.6 L. per square meter per minute. The pulmonary capillary wedge values were within normal limits, and the left ventricular end-diastolic pressure had increased minimally. A left-toright shunt ratio of 1.5 at the main pulmonary artery was detected in 1976. Selective right coronary angiograms requiring 5 c.c. of Renografin-76 dye per second for 3 seconds revealed ALCAPA. A dilated and tortuous right coronary artery was noted (Fig. 2). The anomalous left coronary artery filled through large collaterals by retrograde flow from the right. The dye then flowed into the pulmonary artery. At operation, the anomalous left main coronary artery was separated at its origin from the posterior cusp of the pulmonary artery. Oxygenated blood flowed briskly from the anomalous coronary artery when the arterial tape was released briefly. A pericardial patch was placed over the pulmonary artery at the point from which the anomalous left coronary artery had been detached. The thin-walled, veinlike left coronary artery was anastomosed to the aorta with the aid of a saphenous vein graft. Flows were measured in the newly grafted vein segment at 550 to 750 c.c. per minute by electromagnetic flowmeters. After the operation, the systolic murmur remained unchanged. A distinct and constant diastolic murmur was then noted. It was loudest in the fourth left costosternal area. Postoperative electrocardiograms were unchanged from preoperative tracings except for minor lateral ST depression. The patient was given digoxin, 0.25 mg. daily, during the postoperative period. On March 19, 1976, repeat left heart catheterization was performed. The saphenous vein graft was patent with high flow (Fig. 3). Contrast media, 5 ml. per second for 3 seconds, were required to visualize both the right and left coronary arteries. Whereas preoperatively the posterior descending coronary artery had been well visualized by injection of the right coronary artery, postoperatively the posterior descending branch was filled predominantly by the greater competitive flow from the newly grafted left coronary artery. On discharge, the patient had mild fatigue and no
Anomalous origin of left coronary artery
889
Fig. 2. Preoperative angiogram. Catheter in dilated, S-shaped right coronary artery (center) with dilated, tortuous left coronary artery filling late (upper right) by collateral vessels.
Fig. 3. Postoperative angiogram of saphenous vein graft. Catheter tip (upper left) in patent saphenous vein graft to dilated and tortuous left coronary artery. angina. At follow-up, 3 months later, she continued to be fatigued as prior to the operation, but she no longer has angina or syncope. Discussion Abbott 7 first described ALCAPA in a 60-year-old woman in 1908. Abrikossoff8 first described ALCAPA in an infant in Moscow Children's Hospital in 1911. Although Brooks 9 had been credited with the first description, he had actually described an anomalous right coronary artery from the pulmonary artery in 1884, as did W. Krause in 1865. The syndrome of ALCAPA was described by Bland, White, and Garland 10 when they assembled the clinical and postmortem findings from an infant whose symptoms were dyspnea, sweat-
890
The Journal of Thoracic and Cardiovascular Surgery
Wilson et al.
Table II. Management of teen-aged and adult patients first diagnosed during life as having an ALCAPA Ref. No.
Patient No.
Age (yr.) at diagnosis/sex
Presenting complaint and age (yr.) at onset
16 17 12 18 19 19 20 21 21 22 22 23 24 25 26
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
16/M 16/F 18/M 29/F 31/F 29/F 45/F 23/F 47/M 15/M 27/F 14/F 22/M 41/M 20/M
Heart murmur, 16 yr. Heart murmur Dyspnea, 17 hr. Murmur at birth Heart murmur Heart murmur Heart murmur, 10 yr. Heart murmur, 12 yr. Angina, 46 yr. Murmur, 15 yr. Enlarged heart, 7 yr.; murmur Heart murmur, 10 yr. Heart murmur, 18 yr. Angina Murmur, 15 yr.
Explored Ligation Ligation Declined surgery Ligation Ligation Ligation Ligation Ligation Ligation Ligation Explored twice Ligation Ligation SVG
27 28 29
16 17 18
39/F 13/M 27/F
Angina, 39 yr.
30 31 32 32 32
19 20 21 22 23
34/F 49/F 46/F 40/F 30/F
Angina, 25 yr., and atrial arrhythmia Murmur, birth; angina, 28 yr. Angina, 47 yr. Abnormal ECG; no murmur Heart murmur 5 yr.; angina Murmur
Ligation Declined surgery Ligation
24 25 26
29/M 22/F 42/F
Heart murmur, 5 yr. Heart murmur, 15 yr. Angina, 23 yr.
33 34 Present case
Operation on ALCAPA
SVG SVG SVG SVG SVG SVG Death SVG
Remarks Asymptomatic Asymptomatic Asymptomatic Well Well Asymptomatic Well Well Asymptomatic Asymptomatic Well; no definitive operation Asymptomatic Asymptomatic Asymptomatic; doing strenuous physical exercise Decreased exercise tolerance Well Uneventful convalescence Asymptomatic Asymptomatic Asymptomatic Asymptomatic postop. Gentsch— unreported Asymptomatic Death at operation Well; no angina
Legend: ALCAPA, Anomalous origin of left coronary artery from the pulmonary artery. ECG, Electrocardiogram. SVG, Saphenous vein graft.
ing, crying with exertion, and myocardial infarction. ALCAPA is a rare congenital abnormality, estimated to occur once in 300,000 live births.11 In several series, it has a frequency of 0.5 per cent of all congenital cardiac defects.21 Eighty-five per cent of patients die in infancy or childhood, more than 200 such cases having been described. Only one patient with ALCAPA survives to teen-age or adult life for each two million live births. To date, 44 patients have survived to teen agers or adulthood, but in 41 per cent the diagnosis was made post mortem. 3 ' 4> 12, 13~36 Many lacked signs or symptoms during life, and of those who died, most did so suddenly at a mean age of 35 years. The mean age at the time of diagnosis during life was 29 years (Table II). There was a 1.9:1 female predominance. Other patients, who are now teen-agers, have undergone surgical correction of ALCAPA,1, 2' 37 but they are not included in this review since the correct diagnosis was made before age 13 years, the selected entrance age for
this study. Our patient, with diagnosis at the age of 42 years, is the fifth oldest individual in whom the diagnosis was made during life. The clinical presentation of patients beyond childhood is usually nonspecific; syncope, exertional fatigue, nocturnal dyspnea, and, less commonly, angina pectoris have been described. 1,2 Of those in whom the diagnosis was made during life, only 8 of 25 (31 per cent), including our patient, had angina. Our patient had intermittent exertional angina for 20 years as well as fatigue and syncope. Systolic murmurs have been noted in 69 per cent of cases diagnosed ante mortem and were often heard early in life (Table II). Murmurs which are holosystolic, continuous, or diastolic have also been reported. 14,34,35 Our patient had both systolic and diastolic murmurs, with the systolic murmur spilling into and beyond the second heart sound on the phonocardiogram, as might have been expected for a left-to-right shunt. Electrocardiographic studies are
Volume 73 Number 6 June, 1977
helpful if there is evidence of lateral ischemia or infarction. Anterior myocardial damage has also been reported.1' 2 Both areas were damaged in this case. Ventricular fibrillation1 and sudden unexpected death3' 4 are frequently reported in patients with this congenital defect, the latter occurring especially in teen-agers and adults during vigorous exercise. It is remarkable and fortunate that our patient survived to undergo surgical correction after 20 years of intermittent angina, exertional dyspnea, and even one episode of syncope. Right heart catheterization is of help when an increase in oxygen saturation is found at the level of the pulmonary artery. Supravalvular aortic or selective coronary angiograms are necessary for definitive diagnosis.1 Our patient did indeed have a step-up in oxygen saturation which had not been observed 20 years earlier. Either it was missed through technical error on the first catheterization, or it was not present at that time (vide infra). In our case, diagnosis was made by selective coronary angiograms (Fig. 2). None of the teenaged or adult patients had ventricular fibrillation during or after the cardiac catheterization. However, ventricular fibrillation and three deaths1 have been directly associated with the procedure, all in infants and young children. The pathophysiology of this condition is intimately dependent upon the pressure difference between the systemic and pulmonary circuits and upon the type and extent of communications between them. Before birth, pulmonary artery pressure equals systemic and dictates antegrade flow in the ALCAPA with blood which is saturated. Following birth, reduction of pressure in the pulmonary artery allows the development of a left-toright shunt; this shunt persists since intrapulmonary resistance remains low from that point on. Survival depends upon the development or prior existence of adequate collaterals between the left and the right coronary arteries, for it is shortly after birth that most infants with this disorder become symptomatic. 5,6 Those in whom an adequate left-to-right shunt develops may survive to adulthood; optimally, no significant steal of flow from the myocardium to the pulmonary artery should be present. The left ventricular myocardium and the pulmonary artery represent parallel circuits, both receiving blood from the right coronary artery. Each limb of the parallel circuit receives the same arterial pressure. Therefore, the relative flow in each circuit is determined by resistance in it. Early in life, if left ventricular resistance is normal, flow to the myocardium should be adequate. If left ventricular enddiastolic pressure rises, as with congestive heart fail-
Anomalous origin of left coronary artery
89 1
ure, or if intramyocardial resistance rises, as with ischemia and secondary fibrosis, flow to the left ventricular myocardium must be impeded; therefore, more runoff into the pulmonary artery must occur. Wright and colleagues29 have suggested that the coronary artery steal syndrome is due to increased collateral circulation. An equally tenable hypothesis is that a decrease in compliance of the left ventricle is the cause of increased flow to the pulmonary artery. In our patient, physical findings, electrocardiograms, murmurs, and pulmonary artery pressure had not changed in 20 years (Table I). Why was the left-to-right shunt not seen at age 26? As previously mentioned, technical error is possible. Perhaps it was not present. The shunt may have developed as the left ventricular wall compliance decreased, causing reduced myocardial flow and increased shunting to the pulmonary artery. Of the 26 patients offered surgical treatment, 13 of 26 (50 per cent) underwent ligation of the ALCAPA with good results. Two patients had three operations without successful treatment. Two patients declined surgery. Eight of 26 (31 per cent), including our patient, have now had a saphenous vein graft from the ALCAPA to the aorta (Table II). Of the 13 patients who underwent ligation, all were well at the time of last follow-up. Of the 8 patients with saphenous vein grafts, all were free of angina and in improved condition. One patient had a patent graft when studied 8 years following bypass.33 There was one massive intraoperative infarct during attempted saphenous vein grafting which resulted in death.34 Ligation and saphenous vein graft of the ALCAPA are clearly beneficial since they relieve symptoms. Our patient was discharged from the hospital free of angina and with improved exercise tolerance. Such procedures are expected to prolong life beyond that of an age-matched group not treated surgically. However, we agree with others that follow-up studies are required to support this expectation. A newly acquired diastolic murmur as well as tachycardia at rest and systolic murmurs have been observed postoperatively and deserve comment. Chaitman33 first reported systolic and diastolic murmurs persisting 8 years after successful saphenous vein grafting to an ALCAPA. Our patient had similar murmurs along the left sternal border in the third and fourth intercostal spaces. Cooley and associates37 reported postoperative tachycardia and a systolic murmur in one 5-year-old boy after successful saphenous vein grafting. All patients had patent vein grafts when last studied. It is probable that the murmurs and tachycardia are due to the torrential blood flow from the capacious right and left coronary arteries into the dilated myocardial
892
The Journal of Thoracic and Cardiovascular Surgery
Wilson et al.
sinusoids often noticed in the septum. 35 The enormous flow of arterial blood to the coronary sinus serves as an intracardiac arteriovenous fistula. It may be responsible for the murmurs and tachycardia. One might expect a significant step-up in oxygen saturation at the right atrial level because of the high flow rate of well-saturated venous blood entering that chamber from the coronary sinus. This has not been observed by others following saphenous vein grafting 26 and, despite earnest effort, it was not possible expeditiously to catheterize the right side of the heart from the left groin in our patient after the operation. Pulmonic insufficiency would be a less likely cause of the diastolic murmurs. The pulmonary artery was patched immediately above the pulmonic semilunar valves in our case, and the valve remained intact. In Chaitman's case, the pulmonary artery was not entered. Although patients who undergo simple ligation of the ALCAPA survive, saphenous vein grafting, when technically feasible, is the procedure of choice. Not only does it eliminate the left-to-right shunt, as does ligation, it also restores dual antegrade coronary circulation. REFERENCES 1 Askenazi, J., and Nadas, A. S.: Anomalous Left Coronary Artery Originating From the Pulmonary Artery: Report on 15 Cases, Circulation 51: 976, 1975. 2 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. 3 Kaunitz, P. E.: Origin of Left Coronary Artery From the Pulmonary Artery: Review of the Literature and Report of Two Cases, Am. Heart J. 33: 182, 1947. 4 George, J. M., and Knowlan, D. M.: Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery in an Adult, N. Engl. J. Med. 261: 993, 1959. 5 Agustsson, M. H., Gasul, B. M., and Lundquist, R.: Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery (Adult Type), Pediatrics 29: 274, 1962. 6 Edwards, J. E.: The Direction of Blood Flow in Coronary Arteries Arising From the Pulmonary Trunk (Edit.), Circulation 29: 163, 1964. 7 Abbott, M. E.: Congenital Cardiac Disease, in Osier, W., editor: Modern Medicine, Philadelphia, 1908, Lea & Febiger, Publishers. 8 Abrikossoff, A.: Aneurysm des liuken Herzventrikels mit abnormer Abgangstelle der linken Koronararterie von der Pulmonalis bei einem funfmonatlichen Kinde, Virchows Arch. 203: 413, 1911. 9 Brooks, H.: Two Cases of an Abnormal Coronary Artery
10
11
12
13 14
15
16
17
18
19 20
21
22
23 24
of the Heart Arising From the Pulmonary Artery, J. Anat. Physiol. 20: 26, 1886. Bland, E. F., White, P. D., and Garland, J.: Congenital Anomalies of Coronary Arteries: Report of an Unusual Case Associated With Cardiac Hypertrophy, Am. Heart J. 8: 787, 1933. Keith, J. D.: The Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery, Br. Heart J. 21: 149, 1959. Harthorne, J. W., Scannell, J. G., and Dinsmore, R. E.: Anomalous Origin of the Left Coronary Artery: Remedial Cause of Sudden Death in Adults, N. Engl. J. Med. 275: 660, 1966. Bergnes, M. A.: Anomalous Left Coronary Artery Arising From the Pulmonary Artery in an Athlete, J. Forensic Sci. Soc. 15: 243, 1970. 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. Gouley, B. A.: Anomalous Left Coronary Artery Arising From the Pulmonary Artery: Adult Type, Am. Heart J. 40: 630, 1950. Lampe, C. F. J., and Verheugh, A. P. M.: Anomalous Left Coronary Artery: Adult Type, Am. Heart J. 59: 769, 1960. Massih, N. A., and Lawler, V. M.: Myocardial Ischemia After Ligation of an Anomalous Left Coronary Artery Arising From the Pulmonary Artery, N. Engl. J. Med. 269: 483, 1963. Likar, I., Criley, J. M., and Lewis, K. B.: Anomalous Left Coronary Artery Arising From the Pulmonary Artery in an Adult: Review of the Therapeutic Problem, Circulation 33: 727, 1966. Sabiston, D. C , and Orme, S. K.: Congenital Origin of the Left Coronary Artery From the Pulmonary Artery, J. Cardiovasc. Surg. 9: 534, 1968. Baue, A. E., Baum, S., Blackmore, W. S., et al.: A Later Stage of Anomalous Coronary Circulation With Origin of the Left Coronary Artery From the Pulmonary Artery: Coronary Artery Steal, Circulation 36: 878, 1967. Roche, A. H. G.: Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery in the Adult: Report of Uneventful Ligation in Two Cases, Am. J. Cardiol. 20: 561, 1967. Flamm, M. D., Stinson, E. B., Hultgren, H. N., et al.: Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery, Circulation 38: 113, 1968. Lam, C. R., Taber, R. E., Ziegler, R. F., et al.: Surgical Treatment of Anomalies of the Coronary Arteries, Prog. Carciovasc. Dis. 11: 156, 1968. Summer, G. L., and Hendrix, G. H.: Surgical Ligation of an Anomalous Left Coronary Artery Arising From the Pulmonary Artery in an Adult, Am. Heart J. 76: 812, 1968.
Volume 73 Number 6 June, 1977
25 Yamane, Y., Ikuta, K., Kamada, M., et al.: Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery, Jpn. Circ. J. 33: 445, 1969. 26 Reis, R., Cohen, L. S., and Mason, D. T.: Direct measurement of Instantaneous Coronary Blood Flow After Total Correction of Anomalous Left Coronary Artery, Circulation 39, 40: 229, 1969 (Suppl. I). 27 Dalton, M. L., Jr., Arrington, J. O., and King, S. M.: Surgical Treatment of Adult-Type Anomalous Origin of the Left Coronary Artery Arising From the Pulmonary Artery, Ann. Thorac. Surg. 7: 333, 1969. 28 Perry, L. W., and Scott, L. P.: Anomalous Left Coronary Artery From Pulmonary Artery: Report of 11 cases, Circulation 41: 1043, 1970. 29 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
893
in an Adult, J. THORAC. CARDIOVASC. SURG. 66:
112,
1973. 32 Thomas, C. S., Campbell, W. B., and Alford, W. C : Complete Repair of Anomalous Origin of Left Coronary Artery in an Adult, J. THORAC. CARDIOVASC. SURG. 66:
33
34
35 36
Pulmonary Artery, J. THORAC. CARDIOVASC. SURG. 59:
461, 1970. 30 Gasior, R. M., Winters, W. L., Glick, H., et al.: Anomalous Origin of the Left Coronary Artery From Pulmonary Artery, Am. J. Cardiol. 27: 215, 1971. 31 Akhtar, N., Hyland, J. W., and Adam, M.: Anomalous Left Coronary Artery Arising From the Pulmonary Artery
Anomalous origin of left coronary artery
37
439, 1973. Chaitman, B. R., Bourassa, M. G., and Lespe'rance, J.: Anomalous Left Coronary Artery From Pulmonary Artery: 8 Year Follow-up After SVG Bypass Graft, Circulation 51: 552, 1975. Barrand, K., Brooksby, A. B., and Webb-Peploe, M. M.: Anomalous Origin of Left Coronary Artery From Pulmonary Artery: Surgical Considerations in the Adult, Br. Heart J. 37:441, 1975. Jurishica, A. J.: Anomalous Left Coronary Artery: Adult Type, Am. Heart J. 54: 429, 1957. Usman, A., Fernandez, B., Uricchio, J. F., et al.: Aberrant Origin of the Left Coronary Artery Combined With Mitral Regurgitation, Am. J. Cardiol. 8: 130, 1961. 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.
Charles L. Wilson, M . D . , F . A . C . C , Paul W. Dlabal, M.D., Roy W. Holeyfield, M.D., Cary W. Akins, M.D., and Daniel G. Knauf, M.D., Lackland Air Force Base, Texas
A nomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiovascular defect. Over two hundred cases have been reported during the past century. 1 ' 2 The mortality rate among infants and children without operation has been eighty to ninety per cent. 2 Survival to teen-age and adult life has been infrequent; only forty-four cases including this case have been reported in this age group. Prior to corrective surgical therapy, eighty to ninety per cent of the adults died suddenly at a mean age of thirty-five years. 3 , 4 Less commonly, patients survived with mitral regurgitation or heart failure. Development of angina pectoris is infrequent in older patients with ALCAPA. 2
From the Cardiology Service, Department of Medicine, and the Thoracic Surgery Service, Department of Surgery, Wilford Hall USAF Medical Center, Lackland Air Force Base, Texas 78236. The opinions expressed are those of the authors and do not necessarily represent official USAF policy. Received for publication Nov. 10, 1976. Accepted for publication Jan. 12, 1977. Address for reprints: C. L. Wilson, M.D., 9 Flintstone Court, San Antonio, Texas 78213.
Long-term survival depends upon the development of collateral vessels from the right coronary artery which are adequate to perfuse the myocardium in the territory of the anomalous left coronary artery. Additionally, there must be no significant steal of flow into the pulmonary artery from the myocardium supplied by the ALCAPA. Various authors have classified patients with ALCAPA into infant and adult groups. 5 " 6 The groupings are arbitrary. Occasionally, infants meet adult criteria. The clinical condition of those surviving to teen-age resembles that of adults. All patients 13 years of age and older, who have been studied, have had ALCAPA of the adult type. We have chosen to study patients aged 13 or older in order to expand the data base of the adult population. Previously, 20 teen-aged and adult patients have had surgical correction of ALCAPA. We are reporting a case which is unique because of the duration of symptoms. This patient was the eighth to have successful aorta-left coronary artery grafting with the saphenous vein in this group. Postoperatively, a new diastolic murmur was heard, which may reflect the torrential diastolic intracoronary blood flow. Both ligation and aorta-left coronary artery grafting with the saphenous 887
888
The Journal of Thoracic and Cardiovascular Surgery
Wilson et al.
IL_——Jl
^MMv4_4__^
m
^ - ^ - | V _ ^ - - ^ ^—y^ p-y*-
l-^W** III S A M ^ M J ^ ^ J H ^
Fig. 1. Preoperative electrocardiogram, taken in February of 1976, and tracings from 1955 and 1960 are identical. Table I. Cardiac catheterization compared with 1976
data: 1956
Oxygen saturation (%) Location
1956
SVC IVC RA RV MPA PCW
64 79 74 67 75
Radial Aorta LVED QSI QPI
95
1976 (preop.) 70 71 75 72 82
98.6 3.6 3.6
Pressures (mm Hg) 1956
26/1-6* 19/10-17t 10t (rest) 15% (after exercise) 105/58-78t
1976 (preop.)
24/0-7 24/12-16t
110/60-85 110/0, 13*
2.6 3.9
Legend: SVC, Superior vena cava. IVC, Inferior vena cava. RA, Right atrium. RV, Right ventricle. MPA, Main pulmonary artery. PCW, Pulmonary capillary wedge. LVED, Left ventricular end-diastolic. QSI, Systemic flow index. QPI, Pulmonary flow index. *End-diastolic pressure. tMean pressure.
vein relieve symptoms and perhaps prolong life in patients with ALCAPA. Saphenous vein grafting appears to be the definitive surgical correction for this anomaly. Because surgical correction is available, it is essential that the diagnosis be made during life. Case report J. S., a 42-year-old white woman, was referred in February, 1976, for evaluation of two decades of intermittent
angina pectoris and a recent syncopal episode. The patient described the onset of joint pains at the age of 7 years. A heart murmur was first noted then. She was healthy until the age of 23 years, when, during her third pregnancy, shortness of breath and exertional dyspnea developed, without paroxysmal nocturnal dyspnea, orthopnea, or edema. Because systolic and diastolic heart murmurs were heard and because she had angina pectoris, she was first referred in 1955 to a diagnostic medical center in New England. A Grade 3/6 systolic murmur and a Grade 2/6 diastolic murmur were noted. The murmurs were most intense in the left third and fourth intercostal spaces. The electrocardiogram indicated an anterolateral myocardial infarction (Fig. 1). Right heart catheterization in September, 1956, revealed no abnormalities (Table I). No left-to-right shunt was detected, and no diagnosis was established. Following delivery of a healthy infant, she became asymptomatic and remained so until the age of 41 years, when exertional dyspnea and generalized fatigue developed. She then noted a recurrence of angina pectoris precipitated by emotion and exertion and relieved by rest. The pain occasionally radiated to both sides of the neck, but shortness of breath, diaphoresis, and palpitations never developed. In December, 1975, the patient felt a pressure in her chest after eating. She became nauseated and fainted. She awakened in the emergency room of a small community hospital. The woman was examined and released that night without further diagnostic studies. Physical examination on admission to Wilford Hall USAF Medical Center disclosed a healthy appearing, short, white woman, height 153 cm. and weight 56.6 kilograms. Her temperature was 36.8° C. The pulse rate was 72 beats per minute and regular and the respiratory rate 16 breaths per minute. Blood pressure was 118/80 mm. Hg in both arms. Jugular venous and carotid pulses and contours were within normal limits. The point of maximum impulse was normal. The first heart sound was normal, and the second heart sound was physiologically split. A Grade 3/6 ejection murmur was heard through the first two thirds of systole. It was loudest in
Volume 73 Number 6 June, 1977
the fourth intercostal space at the left sternal border. An intermittent Grade 1 /6 early diastolic decrescendo murmur was reported by some observers. The systolic murmur decreased with amyl nitrite and during a Valsalva maneuver, but it returned within 10 beats after release of the maneuver. The electrocardiogram (Fig. 1) demonstrated Q waves in leads I and L and poor R-wave progression in Leads Vj to V3. The tracing was unchanged from the electrocardiograms taken between 1955 and 1960. The vectorcardiogram revealed the horizontal loop to be posterior, but within normal limits. The chest x-ray film was considered normal. The phonocardiogram showed a normal carotid contour and a long systolic murmur continuing beyond the second sound, and a fourth heart sound was recorded. The QS2 interval was 450 msec, left ventricular ejection time (LVET) 285 msec, and pre-ejection period (PEP) 130 msec. The PEP/LVET of 45 per cent was increased, an indication of left ventricular dysfunction. An exercise tolerance test was not performed since there is a close association between vigorous exercise and sudden death in teen-agers and adults with ALCAPA.12, n The history of recent syncope was a further contraindication. ALCAPA was a major consideration before catheterization, as was a high ventricular septal defect. In February, 1976, the patient underwent a second cardiac catheterization (Table I). The right-sided pressures were unchanged. The cardiac index had decreased from 3.6 to 2.6 L. per square meter per minute. The pulmonary capillary wedge values were within normal limits, and the left ventricular end-diastolic pressure had increased minimally. A left-toright shunt ratio of 1.5 at the main pulmonary artery was detected in 1976. Selective right coronary angiograms requiring 5 c.c. of Renografin-76 dye per second for 3 seconds revealed ALCAPA. A dilated and tortuous right coronary artery was noted (Fig. 2). The anomalous left coronary artery filled through large collaterals by retrograde flow from the right. The dye then flowed into the pulmonary artery. At operation, the anomalous left main coronary artery was separated at its origin from the posterior cusp of the pulmonary artery. Oxygenated blood flowed briskly from the anomalous coronary artery when the arterial tape was released briefly. A pericardial patch was placed over the pulmonary artery at the point from which the anomalous left coronary artery had been detached. The thin-walled, veinlike left coronary artery was anastomosed to the aorta with the aid of a saphenous vein graft. Flows were measured in the newly grafted vein segment at 550 to 750 c.c. per minute by electromagnetic flowmeters. After the operation, the systolic murmur remained unchanged. A distinct and constant diastolic murmur was then noted. It was loudest in the fourth left costosternal area. Postoperative electrocardiograms were unchanged from preoperative tracings except for minor lateral ST depression. The patient was given digoxin, 0.25 mg. daily, during the postoperative period. On March 19, 1976, repeat left heart catheterization was performed. The saphenous vein graft was patent with high flow (Fig. 3). Contrast media, 5 ml. per second for 3 seconds, were required to visualize both the right and left coronary arteries. Whereas preoperatively the posterior descending coronary artery had been well visualized by injection of the right coronary artery, postoperatively the posterior descending branch was filled predominantly by the greater competitive flow from the newly grafted left coronary artery. On discharge, the patient had mild fatigue and no
Anomalous origin of left coronary artery
889
Fig. 2. Preoperative angiogram. Catheter in dilated, S-shaped right coronary artery (center) with dilated, tortuous left coronary artery filling late (upper right) by collateral vessels.
Fig. 3. Postoperative angiogram of saphenous vein graft. Catheter tip (upper left) in patent saphenous vein graft to dilated and tortuous left coronary artery. angina. At follow-up, 3 months later, she continued to be fatigued as prior to the operation, but she no longer has angina or syncope. Discussion Abbott 7 first described ALCAPA in a 60-year-old woman in 1908. Abrikossoff8 first described ALCAPA in an infant in Moscow Children's Hospital in 1911. Although Brooks 9 had been credited with the first description, he had actually described an anomalous right coronary artery from the pulmonary artery in 1884, as did W. Krause in 1865. The syndrome of ALCAPA was described by Bland, White, and Garland 10 when they assembled the clinical and postmortem findings from an infant whose symptoms were dyspnea, sweat-
890
The Journal of Thoracic and Cardiovascular Surgery
Wilson et al.
Table II. Management of teen-aged and adult patients first diagnosed during life as having an ALCAPA Ref. No.
Patient No.
Age (yr.) at diagnosis/sex
Presenting complaint and age (yr.) at onset
16 17 12 18 19 19 20 21 21 22 22 23 24 25 26
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
16/M 16/F 18/M 29/F 31/F 29/F 45/F 23/F 47/M 15/M 27/F 14/F 22/M 41/M 20/M
Heart murmur, 16 yr. Heart murmur Dyspnea, 17 hr. Murmur at birth Heart murmur Heart murmur Heart murmur, 10 yr. Heart murmur, 12 yr. Angina, 46 yr. Murmur, 15 yr. Enlarged heart, 7 yr.; murmur Heart murmur, 10 yr. Heart murmur, 18 yr. Angina Murmur, 15 yr.
Explored Ligation Ligation Declined surgery Ligation Ligation Ligation Ligation Ligation Ligation Ligation Explored twice Ligation Ligation SVG
27 28 29
16 17 18
39/F 13/M 27/F
Angina, 39 yr.
30 31 32 32 32
19 20 21 22 23
34/F 49/F 46/F 40/F 30/F
Angina, 25 yr., and atrial arrhythmia Murmur, birth; angina, 28 yr. Angina, 47 yr. Abnormal ECG; no murmur Heart murmur 5 yr.; angina Murmur
Ligation Declined surgery Ligation
24 25 26
29/M 22/F 42/F
Heart murmur, 5 yr. Heart murmur, 15 yr. Angina, 23 yr.
33 34 Present case
Operation on ALCAPA
SVG SVG SVG SVG SVG SVG Death SVG
Remarks Asymptomatic Asymptomatic Asymptomatic Well Well Asymptomatic Well Well Asymptomatic Asymptomatic Well; no definitive operation Asymptomatic Asymptomatic Asymptomatic; doing strenuous physical exercise Decreased exercise tolerance Well Uneventful convalescence Asymptomatic Asymptomatic Asymptomatic Asymptomatic postop. Gentsch— unreported Asymptomatic Death at operation Well; no angina
Legend: ALCAPA, Anomalous origin of left coronary artery from the pulmonary artery. ECG, Electrocardiogram. SVG, Saphenous vein graft.
ing, crying with exertion, and myocardial infarction. ALCAPA is a rare congenital abnormality, estimated to occur once in 300,000 live births.11 In several series, it has a frequency of 0.5 per cent of all congenital cardiac defects.21 Eighty-five per cent of patients die in infancy or childhood, more than 200 such cases having been described. Only one patient with ALCAPA survives to teen-age or adult life for each two million live births. To date, 44 patients have survived to teen agers or adulthood, but in 41 per cent the diagnosis was made post mortem. 3 ' 4> 12, 13~36 Many lacked signs or symptoms during life, and of those who died, most did so suddenly at a mean age of 35 years. The mean age at the time of diagnosis during life was 29 years (Table II). There was a 1.9:1 female predominance. Other patients, who are now teen-agers, have undergone surgical correction of ALCAPA,1, 2' 37 but they are not included in this review since the correct diagnosis was made before age 13 years, the selected entrance age for
this study. Our patient, with diagnosis at the age of 42 years, is the fifth oldest individual in whom the diagnosis was made during life. The clinical presentation of patients beyond childhood is usually nonspecific; syncope, exertional fatigue, nocturnal dyspnea, and, less commonly, angina pectoris have been described. 1,2 Of those in whom the diagnosis was made during life, only 8 of 25 (31 per cent), including our patient, had angina. Our patient had intermittent exertional angina for 20 years as well as fatigue and syncope. Systolic murmurs have been noted in 69 per cent of cases diagnosed ante mortem and were often heard early in life (Table II). Murmurs which are holosystolic, continuous, or diastolic have also been reported. 14,34,35 Our patient had both systolic and diastolic murmurs, with the systolic murmur spilling into and beyond the second heart sound on the phonocardiogram, as might have been expected for a left-to-right shunt. Electrocardiographic studies are
Volume 73 Number 6 June, 1977
helpful if there is evidence of lateral ischemia or infarction. Anterior myocardial damage has also been reported.1' 2 Both areas were damaged in this case. Ventricular fibrillation1 and sudden unexpected death3' 4 are frequently reported in patients with this congenital defect, the latter occurring especially in teen-agers and adults during vigorous exercise. It is remarkable and fortunate that our patient survived to undergo surgical correction after 20 years of intermittent angina, exertional dyspnea, and even one episode of syncope. Right heart catheterization is of help when an increase in oxygen saturation is found at the level of the pulmonary artery. Supravalvular aortic or selective coronary angiograms are necessary for definitive diagnosis.1 Our patient did indeed have a step-up in oxygen saturation which had not been observed 20 years earlier. Either it was missed through technical error on the first catheterization, or it was not present at that time (vide infra). In our case, diagnosis was made by selective coronary angiograms (Fig. 2). None of the teenaged or adult patients had ventricular fibrillation during or after the cardiac catheterization. However, ventricular fibrillation and three deaths1 have been directly associated with the procedure, all in infants and young children. The pathophysiology of this condition is intimately dependent upon the pressure difference between the systemic and pulmonary circuits and upon the type and extent of communications between them. Before birth, pulmonary artery pressure equals systemic and dictates antegrade flow in the ALCAPA with blood which is saturated. Following birth, reduction of pressure in the pulmonary artery allows the development of a left-toright shunt; this shunt persists since intrapulmonary resistance remains low from that point on. Survival depends upon the development or prior existence of adequate collaterals between the left and the right coronary arteries, for it is shortly after birth that most infants with this disorder become symptomatic. 5,6 Those in whom an adequate left-to-right shunt develops may survive to adulthood; optimally, no significant steal of flow from the myocardium to the pulmonary artery should be present. The left ventricular myocardium and the pulmonary artery represent parallel circuits, both receiving blood from the right coronary artery. Each limb of the parallel circuit receives the same arterial pressure. Therefore, the relative flow in each circuit is determined by resistance in it. Early in life, if left ventricular resistance is normal, flow to the myocardium should be adequate. If left ventricular enddiastolic pressure rises, as with congestive heart fail-
Anomalous origin of left coronary artery
89 1
ure, or if intramyocardial resistance rises, as with ischemia and secondary fibrosis, flow to the left ventricular myocardium must be impeded; therefore, more runoff into the pulmonary artery must occur. Wright and colleagues29 have suggested that the coronary artery steal syndrome is due to increased collateral circulation. An equally tenable hypothesis is that a decrease in compliance of the left ventricle is the cause of increased flow to the pulmonary artery. In our patient, physical findings, electrocardiograms, murmurs, and pulmonary artery pressure had not changed in 20 years (Table I). Why was the left-to-right shunt not seen at age 26? As previously mentioned, technical error is possible. Perhaps it was not present. The shunt may have developed as the left ventricular wall compliance decreased, causing reduced myocardial flow and increased shunting to the pulmonary artery. Of the 26 patients offered surgical treatment, 13 of 26 (50 per cent) underwent ligation of the ALCAPA with good results. Two patients had three operations without successful treatment. Two patients declined surgery. Eight of 26 (31 per cent), including our patient, have now had a saphenous vein graft from the ALCAPA to the aorta (Table II). Of the 13 patients who underwent ligation, all were well at the time of last follow-up. Of the 8 patients with saphenous vein grafts, all were free of angina and in improved condition. One patient had a patent graft when studied 8 years following bypass.33 There was one massive intraoperative infarct during attempted saphenous vein grafting which resulted in death.34 Ligation and saphenous vein graft of the ALCAPA are clearly beneficial since they relieve symptoms. Our patient was discharged from the hospital free of angina and with improved exercise tolerance. Such procedures are expected to prolong life beyond that of an age-matched group not treated surgically. However, we agree with others that follow-up studies are required to support this expectation. A newly acquired diastolic murmur as well as tachycardia at rest and systolic murmurs have been observed postoperatively and deserve comment. Chaitman33 first reported systolic and diastolic murmurs persisting 8 years after successful saphenous vein grafting to an ALCAPA. Our patient had similar murmurs along the left sternal border in the third and fourth intercostal spaces. Cooley and associates37 reported postoperative tachycardia and a systolic murmur in one 5-year-old boy after successful saphenous vein grafting. All patients had patent vein grafts when last studied. It is probable that the murmurs and tachycardia are due to the torrential blood flow from the capacious right and left coronary arteries into the dilated myocardial
892
The Journal of Thoracic and Cardiovascular Surgery
Wilson et al.
sinusoids often noticed in the septum. 35 The enormous flow of arterial blood to the coronary sinus serves as an intracardiac arteriovenous fistula. It may be responsible for the murmurs and tachycardia. One might expect a significant step-up in oxygen saturation at the right atrial level because of the high flow rate of well-saturated venous blood entering that chamber from the coronary sinus. This has not been observed by others following saphenous vein grafting 26 and, despite earnest effort, it was not possible expeditiously to catheterize the right side of the heart from the left groin in our patient after the operation. Pulmonic insufficiency would be a less likely cause of the diastolic murmurs. The pulmonary artery was patched immediately above the pulmonic semilunar valves in our case, and the valve remained intact. In Chaitman's case, the pulmonary artery was not entered. Although patients who undergo simple ligation of the ALCAPA survive, saphenous vein grafting, when technically feasible, is the procedure of choice. Not only does it eliminate the left-to-right shunt, as does ligation, it also restores dual antegrade coronary circulation. REFERENCES 1 Askenazi, J., and Nadas, A. S.: Anomalous Left Coronary Artery Originating From the Pulmonary Artery: Report on 15 Cases, Circulation 51: 976, 1975. 2 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. 3 Kaunitz, P. E.: Origin of Left Coronary Artery From the Pulmonary Artery: Review of the Literature and Report of Two Cases, Am. Heart J. 33: 182, 1947. 4 George, J. M., and Knowlan, D. M.: Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery in an Adult, N. Engl. J. Med. 261: 993, 1959. 5 Agustsson, M. H., Gasul, B. M., and Lundquist, R.: Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery (Adult Type), Pediatrics 29: 274, 1962. 6 Edwards, J. E.: The Direction of Blood Flow in Coronary Arteries Arising From the Pulmonary Trunk (Edit.), Circulation 29: 163, 1964. 7 Abbott, M. E.: Congenital Cardiac Disease, in Osier, W., editor: Modern Medicine, Philadelphia, 1908, Lea & Febiger, Publishers. 8 Abrikossoff, A.: Aneurysm des liuken Herzventrikels mit abnormer Abgangstelle der linken Koronararterie von der Pulmonalis bei einem funfmonatlichen Kinde, Virchows Arch. 203: 413, 1911. 9 Brooks, H.: Two Cases of an Abnormal Coronary Artery
10
11
12
13 14
15
16
17
18
19 20
21
22
23 24
of the Heart Arising From the Pulmonary Artery, J. Anat. Physiol. 20: 26, 1886. Bland, E. F., White, P. D., and Garland, J.: Congenital Anomalies of Coronary Arteries: Report of an Unusual Case Associated With Cardiac Hypertrophy, Am. Heart J. 8: 787, 1933. Keith, J. D.: The Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery, Br. Heart J. 21: 149, 1959. Harthorne, J. W., Scannell, J. G., and Dinsmore, R. E.: Anomalous Origin of the Left Coronary Artery: Remedial Cause of Sudden Death in Adults, N. Engl. J. Med. 275: 660, 1966. Bergnes, M. A.: Anomalous Left Coronary Artery Arising From the Pulmonary Artery in an Athlete, J. Forensic Sci. Soc. 15: 243, 1970. 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. Gouley, B. A.: Anomalous Left Coronary Artery Arising From the Pulmonary Artery: Adult Type, Am. Heart J. 40: 630, 1950. Lampe, C. F. J., and Verheugh, A. P. M.: Anomalous Left Coronary Artery: Adult Type, Am. Heart J. 59: 769, 1960. Massih, N. A., and Lawler, V. M.: Myocardial Ischemia After Ligation of an Anomalous Left Coronary Artery Arising From the Pulmonary Artery, N. Engl. J. Med. 269: 483, 1963. Likar, I., Criley, J. M., and Lewis, K. B.: Anomalous Left Coronary Artery Arising From the Pulmonary Artery in an Adult: Review of the Therapeutic Problem, Circulation 33: 727, 1966. Sabiston, D. C , and Orme, S. K.: Congenital Origin of the Left Coronary Artery From the Pulmonary Artery, J. Cardiovasc. Surg. 9: 534, 1968. Baue, A. E., Baum, S., Blackmore, W. S., et al.: A Later Stage of Anomalous Coronary Circulation With Origin of the Left Coronary Artery From the Pulmonary Artery: Coronary Artery Steal, Circulation 36: 878, 1967. Roche, A. H. G.: Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery in the Adult: Report of Uneventful Ligation in Two Cases, Am. J. Cardiol. 20: 561, 1967. Flamm, M. D., Stinson, E. B., Hultgren, H. N., et al.: Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery, Circulation 38: 113, 1968. Lam, C. R., Taber, R. E., Ziegler, R. F., et al.: Surgical Treatment of Anomalies of the Coronary Arteries, Prog. Carciovasc. Dis. 11: 156, 1968. Summer, G. L., and Hendrix, G. H.: Surgical Ligation of an Anomalous Left Coronary Artery Arising From the Pulmonary Artery in an Adult, Am. Heart J. 76: 812, 1968.
Volume 73 Number 6 June, 1977
25 Yamane, Y., Ikuta, K., Kamada, M., et al.: Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery, Jpn. Circ. J. 33: 445, 1969. 26 Reis, R., Cohen, L. S., and Mason, D. T.: Direct measurement of Instantaneous Coronary Blood Flow After Total Correction of Anomalous Left Coronary Artery, Circulation 39, 40: 229, 1969 (Suppl. I). 27 Dalton, M. L., Jr., Arrington, J. O., and King, S. M.: Surgical Treatment of Adult-Type Anomalous Origin of the Left Coronary Artery Arising From the Pulmonary Artery, Ann. Thorac. Surg. 7: 333, 1969. 28 Perry, L. W., and Scott, L. P.: Anomalous Left Coronary Artery From Pulmonary Artery: Report of 11 cases, Circulation 41: 1043, 1970. 29 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
893
in an Adult, J. THORAC. CARDIOVASC. SURG. 66:
112,
1973. 32 Thomas, C. S., Campbell, W. B., and Alford, W. C : Complete Repair of Anomalous Origin of Left Coronary Artery in an Adult, J. THORAC. CARDIOVASC. SURG. 66:
33
34
35 36
Pulmonary Artery, J. THORAC. CARDIOVASC. SURG. 59:
461, 1970. 30 Gasior, R. M., Winters, W. L., Glick, H., et al.: Anomalous Origin of the Left Coronary Artery From Pulmonary Artery, Am. J. Cardiol. 27: 215, 1971. 31 Akhtar, N., Hyland, J. W., and Adam, M.: Anomalous Left Coronary Artery Arising From the Pulmonary Artery
Anomalous origin of left coronary artery
37
439, 1973. Chaitman, B. R., Bourassa, M. G., and Lespe'rance, J.: Anomalous Left Coronary Artery From Pulmonary Artery: 8 Year Follow-up After SVG Bypass Graft, Circulation 51: 552, 1975. Barrand, K., Brooksby, A. B., and Webb-Peploe, M. M.: Anomalous Origin of Left Coronary Artery From Pulmonary Artery: Surgical Considerations in the Adult, Br. Heart J. 37:441, 1975. Jurishica, A. J.: Anomalous Left Coronary Artery: Adult Type, Am. Heart J. 54: 429, 1957. Usman, A., Fernandez, B., Uricchio, J. F., et al.: Aberrant Origin of the Left Coronary Artery Combined With Mitral Regurgitation, Am. J. Cardiol. 8: 130, 1961. 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.