Anomalous origin of the left coronary artery from the right pulmonary artery Surgical repair in a 10-month-old child A previously unknown coronary artery anomaly is reported: origin of the left coronary artery from the right pulmonary artery. This unusual anomaly can probably be explained by the theory of dual embryonic development of the coronary arteries from angioblastic buds in the truncus arteriosus. It is thought that these buds anastomose with a capillary network that is developing on the surface of the ventricles. Complete repair of this lesion with restoration of a two-coronary artery system was accomplished in a 10-month-old baby. The coronary artery was detached from the pulmonary artery, with a button of pulmonary artery being retained around the coronary artery ostium. Direct systemic arterial flow to the left coronary artery was established by anastomosis of the left coronary to the right subclavian artery.
Donald B. Doty, M.D., B. Chandramouli, M.D., Richard E. Schieken, M.D., Ronald M. Lauer, M.D., and J. L. Ehrenhaft, M.D., Iowa City, Iowa
V-/rigin of the left coronary artery from the pulmonary artery is a rare anomaly. It is estimated to occur in 0.25 to 0.46 per cent of patients with congenital cardiac anomalies. 1 Most of the reported cases (about 200) 2 involve origin of the left coronary artery from the posterior sinus of the pulmonary artery. Rarely, the right coronary artery or the entire coronary artery system takes origin from the main pulmonary trunk. 3 Accessory coronary arteries from the pulmonary artery have also been reported. To date, no case of origin of the left coronary artery from the right branch of the pulmonary artery has been reported. This case is presented because of its importance in the understanding of the embryogenesis of the coronary arteries and the surgical principles used to simplify total correction of anomalous origin of the left coronary artery even in tiny babies. From the Division of Thoracic and Cardiovascular Surgery, Section of Pediatric Cardiology, Departments of Surgery and Pediatrics, University of Iowa Hospitals and Clinics, Iowa City, Iowa 52242. Received for publication Nov. 7, 1975. Accepted for publication Jan. 6, 1976. Address for reprints: Donald B. Doty, M.D., Division of Thoracic and Cardiovascular Surgery, Department of Surgery, University of Iowa Hospitals and Clinics, Iowa City, Iowa 52242.
Case report A 10-month-old girl was the product of a term pregnancy complicated by pre-eclampsia in the last trimester. The baby was thought to be normal at birth. Growth and development seemed normal, but a heart murmur was discovered at 5 months of age. She was referred to the University of Iowa Hospitals and Clinics at 10 months of age because of mild congestive cardiac failure. The infant was a small female weighing 7.3 kilograms, which is at the third percentile for weight at 10 months. The pulse was 130 beats per minute, blood pressure 90 mm. Hg systolic, and respiratory rate 55 breaths per minute. The heart was normal, and a Grade 3/6 continuous murmur was audible along the left sternal border. The liver was slightly enlarged. Peripheral pulses were normal, and there was no cyanosis. Findings from blood count, urinalysis, electrocardiogram, and chest x-ray study were all normal. Cardiac catheterization showed normal intracardiac pressures and no intracardiac shunts. No oxygen samples were taken in the right pulmonary artery. Cineangiocardiography provided the diagnosis. Injection into the left ventricle showed this chamber to be slightly dilated. The left ventricle appeared to have slightly reduced contractility and mild mitral valve incompetence. Injection into the ascending aorta showed normal brachiocephalic vessels from a left aortic arch. A large right coronary artery, originating from the anterior sinus of Valsalva, was the sole arterial blood supply to the heart. The sinus node artery and a large conal branch were the first branches of the right coronary artery which continued in the atrioventricular groove to the posterior descending artery. The circumflex coronary artery filled 787
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Left Coronary Artery RCA Circumflex Artery
Posterior Descendin Artery
Fig. 1. Coronary angiogram (composite tracing). Large right coronary artery (RCA) fills left coronary artery via conal and septal collateral vessels. Left coronary artery enters midportion of right pulmonary artery. (RPA). PA, Pulmonary artery.
Fig. 2. Surgeon's view of right pulmonary artery showing origin of left coronary artery.
DETACHMENT OF LEFT CORONARY ARTERY Right Pulmonary Artery Left Coronary Artery
RECONSTRUCTION OF RIGHT PULMONARY ARTERY Pericordiol Patch
Fig. 3. Operative technique. Left coronary artery detached, retaining a button of pulmonary artery. Defect in pulmonary artery reconstructed with pericardial patch. retrograde from the right coronary artery, and the left anterior descending coronary artery filled through the conal arteries and via intercoronary collaterals at the apex. The right pulmonary artery was then opacified by retrograde flow from the left coronary artery. Pulmonary angiography showed nonopacified blood entering the midportion of the caudal aspect of the right pulmonary artery from the left coronary artery. A composite of the angiogram is outlined in Fig. 1. We operated knowing that the origin of the left coronary artery was accessible by control of the right pulmonary artery and that occlusion of the left coronary artery should cause no deleterious effect because of extensive collateral circulation
from the right coronary artery. Cardiopulmonary bypass was not required to establish a two-coronary artery system. A right posterolateral thoracotomy incision was made and the diagnosis confirmed. A photograph of this unusual and complex anomaly is shown in Fig. 2. The right pulmonary artery and the origin of the left coronary were isolated and occluded (Fig. 3). The origin of the coronary artery was excised, with a button of pulmonary artery 5mm. in diameter being retained around the coronary artery ostium. The pulmonary artery was reconstructed by closing the defect with a patch of pericardium by means of a continuous monofilament suture. The right subclavian artery was
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COMPLETED ANASTOMOSIS Fig. 4. Operative technique. Right subclavian artery anastomosed end-to-end to left coronary artery. mobilized by dividing its branches. The artery was divided at its thoracic exit and the proximal end withdrawn from beneath the recurrent branch of the vagus nerve and taken caudad to approximate the end of the left coronary artery. An end-to-end anastomosis of the right subclavian artery to the left coronary artery was constructed with fine monofilament suture (Fig. 4). The subclavian artery, which measured 5 mm. in diameter, provided a large anastomosis when approximated to the coronary artery with its retained button of pulmonary artery, even though the internal diameter of the left coronary artery was only 1.5 mm. (Fig. 5). Blood flow (mean) measured by an electromagnetic flowmeter was 45 ml. per minute (Fig. 6). Postoperative convalescence was routine, and the patient went home 7 days after the operation. Cineangiograms performed 4 months following the operation demonstrated the left coronary artery filling from the right subclavian artery (Fig. 7). Comment The unusual feature of this case is in the site of origin of the left coronary artery from the right branch of the pulmonary artery. Other previously reported cases of anomalous origin of the left coronary artery have involved the pulmonary trunk. This curious anomaly should provide further understanding of the embryogenesis of the coronary arteries. The coronary arteries first appear as solid angioblastic buds between the sixth and seventh weeks of fetal life. These buds rapidly extend throughout the epicardial layer of the heart, so that by the ninth week all the major branches of the coronary artery system are present. The primordial cusps of the aortic and
Fig. 5. Anastomosis of right subclavian artery to left coronary artery. pulmonary valves develop and become functionally effective, and the muscular walls of the ascending aorta and pulmonary trunk are being defined concurrent with the development of the coronary arteries. Also, the distal end of the pulmonary trunk is marked by a prominent dilatation, the point from which the branch pulmonary arteries are given off. Thus the coronary arteries, the semilunar valve cusps, the ascending aorta, the pulmonary trunk, and its branches all develop within a similar period. 4 The theory of dual embryonic origin of the coronary arteries was proposed by Ogden. 5 The distal portion was thought to develop initially as a capillary network within the atrioventricular and intraventricular grooves and to form a peritruncal ring around the developing great vessels. The proximal portions of the coronary arteries develop as coronary buds, which anastomose later with the peritruncal ring. The coronary artery anomaly observed in this patient supports Ogden's thesis in that the distal or intracardiac coronary arterial structure developed normally while the proximal left coronary angioblastic bud probably became displaced onto the simultaneously developing right pulmonary artery. Later anastomosis of these two portions of the developing left coronary artery probably
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Fig. 6. Phasic and mean blood flow (45 ml. per minute) into left coronary artery.
Right Subclavian Artery
ANASTOMOSIS
Left Coronary Artery
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Fig. 7. Postoperative coronary angiogram (composite tracing). Two-coronary artery system with left coronary artery filled via right subclavian artery. RCA, Right coronary artery. resulted in this rare anomaly with the left coronary artery originating well out on the right branch of the pulmonary artery. The surgical repair represents a logical restoration of a two-coronary artery system, with autogenous artery used for the reconstruction. The principle of retaining a button of pulmonary artery around the ostium of the anomalous coronary artery to make the anastomosis larger and easier to perform was probably first proposed by Mustard 6 in unsuccessful attempts at reconstruction with the carotid artery. Neches and associates 7 have also shown that this technique can simplify anastomosis during successful reconstruction of a twocoronary system. They used it in 2 patients, one of whom was only 7 months old. This principle should allow correction of the defect regardless of the patient's age. Most of the successful reconstructive operations for anomalous origin of the left coronary artery have utilized autogenous saphenous vein grafts to establish a two-coronary artery system in patients over the age of
2 years. 8 Recently, a saphenous vein homograft obtained from a sibling was successfully used in a 17 month old patient. 9 Histocompatibility factors are obviously of major significance with this technique. Furthermore, long-term durability of venous grafts in the coronary arterial system is still sufficiently in question so that use of systemic artery to provide direct input to the coronary artery seems the preferred treatment. Meyer 10 reported the first successful systemic artery-coronary artery anastomosis for anomalous origin of the left coronary artery, with the left subclavian artery used for the reconstruction. Pinsky, 11 Replogle, 12 and their associates used the left subclavian artery in infants less than 6 months old. Potential kinking of the left subclavian artery at its origin over the aortic arch may be a justified criticism of this approach. In future cases, however, this could be modified via a proximal left subclavian angioplasty proposed by Laks and Castaneda. 13 Since the origin of the left coronary artery was to the right of the midline in the case reported here, it was possible to base the right subclavian artery on the innominate artery without concern for kinking or distortion. REFERENCES 1 Nora, J. J., and McNamara, D. G.: Anomalies of the Coronary Arteries and Coronary Artery Fistula, in Watson, H., editor: Pediatric Cardiology, 1968, The C. V. Mosby Company, p. 295. 2 Askenazi, J., and Nadas, A. S.: Anomalous Left Coronary Artery Originating From the Pulmonary Artery: Report on 15 Cases, Circulation 51: 976, 1975. 3 Blake, H. A., Manion, W. C , Mattingly, T. W., and Baroldi, G.: Coronary Artery Anomalies, Circulation 30: 927, 1964. 4 Licata, R. H.: The Human Embryonic Heart in the Ninth Week, Am. J. Anat. 94: 73, 1954. 5 Ogden, J.: The Origin of the Coronary Arteries, Circulation 38: 150, 1968 (Suppl. VI). 6 Mustard, W. T.: Anomalies of the Coronary Artery, in Pediatric Surgery, Chicago, 1962, Year Book Medical Publishers, Inc., p. 434. 7 Neches, W. H., Matthews, R. A., Park, S. C , Lenox, C.
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C , Zuberbuhler, J. R., Siewers, R. D., and Bahnson, H. T.: Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery: A New Method of Surgical Repair, Circulation 50: 582, 1974. 8 Chiariello, L., Meyer, J., Reul, G. J., Hallman, G. L., and Cooley, D. A.: Surgical Treatment for Anomalous Origin of Left Coronary Artery From Pulmonary Artery, Ann. Thorac. Surg. 19: 443, 1975. 9 Venugopal, P., and Subramanian, S.: Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery, Ann. Thorac. Surg. 19: 451, 1975. 10 Meyer, B. W., Stefanki, G., Stiles, Q. R., Lindesmith, G. G., and Jones, J. C : A Method of Definitive Surgical Treatment of Anomalous Origin of Left Coronary Artery:
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A Case Report, J. THORAC. CARDIOVASC. SURG. 56: 104,
1968. 11 Pinsky, W. W., Fagan, L. R., Kraeger, R. R., Mudd, J. G. F., and Willman, V. L.: Anomalous Left Coronary Artery: Report of Two Cases, J. THORAC. CARDIOVASC. SURG. 65: 810,
1973.
12 Replogle, R., Cooperman, M., Arcilla, R., and Liu, C Y.: Surgical Correction of Anomalous Left Coronary Artery, motion picture - color, University of Chicago, Pritzker School of Medicine, 1973. 13 Laks, H., and Castaneda, A. R.: Subclavian Arterioplasty for Ipsilateral Blalock-Taussig Shunt, Ann. Thorac. Surg. 19: 319, 1975.