Anomalous origin of circumflex coronary artery from right pulmonary artery Report of a rare anomaly A heretofore unreported anomaly of the coronary arteries in which a large circumflex artery arises from the pulmonary artery is described. The circumflex coronary artery in an 8-year-old girl was filled in a retrograde fashion via collaterals from a dominant left anterior descending coronary artery, and the right coronary artery was small and nondominant. Successful surgical correction of this defect was performed bx use of total cardiopulmonarx bypass and cardioplegic arrest. The orifice of the anomalous vessel was detached from the right pulmonary artery and implanted into the posterolateral aspect of the ascending aorta. Cardiopulmonarx bypass with cardioplegic arrest is recommended for reconstruction of coronary arterial anomalies with abnormal sites of origin.
David A. Ott, M . D . , Denton A. Cooley, M.D., William W. Pinsky, M.D., and Charles E. Mullins, M . D . , Houston, Texas
A nomalous origin of the coronary arteries is a rare and serious form of congenital heart disease. 1 Death usually occurs in infancy and sudden cardiac arrest is common in patients who survive childhood without surgical correction. 2 - 5 Numerous types of coronary artery anomalies have been described in autopsy specimens and summarized in a review by Ogden. 6 An extremely rare anomaly in which a large circumflex coronary artery arose from the right pulmonary artery was recently encountered at the Texas Heart Institute (Texas Children's Hospital). To our knowledge this specific anomaly has not been described previously, and thus it prompted the following case report. C a s e report An 8-year-old girl was admitted to Texas Children's Hospital for the first time on May 2, 1977. The patient was born after a normal pregnancy, labor, and delivery and weighed 7 pounds, 9 ounces at birth. On the fifth day of life a diagnosis of congestive heart failure was made by the family pediatriFrom the Division of Surgery of the Texas Heart Institute, St. Luke's Episcopal and Texas Children's Hospitals, and the Department of Pediatrics, Section of Cardiology of Texas Children's Hospital, Houston, Texas. Received for publication March 7, 1978. Accepted for publication April 24, 1978. Address for reprints: Dr. Denton A. Cooley, Texas Heart Institute, P.O. Box 20345, Houston, Texas 77025.
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cian. Digitalis was administered and the patient became asymptomatic. No further diagnostic evaluation was made until a murmur of aortic stenosis was noted when the patient was 5 years of age. Cardiac catheterization performed at that time (1973) demonstrated a bicuspid aortic valve. In November of 1976 a stress electrocardiogram revealed significant anterolateral ischemia. Cardiac catheterization demonstrated an anomalous coronary artery, and she was referred to this institution for treatment. Repeat catheterization confirmed the presence of a bicuspid aortic valve with a 5 mm. Hg gradient from the left ventricle to the aorta. Aortic root injection revealed a nondominant right coronary artery and a large circumflex coronary artery arising from the right pulmonary artery (Fig. 1). The circumflex artery filled retrogradely via large collaterals from the left anterior descending coronary artery, which produced a "coronary steal" phenomenon (Fig. 1). Operation was performed on May 5, 1977, through a median sternotomy incision. The superior and inferior venae cavae were cannulated, and the ascending aorta was cannulated and cross-clamped. Cold cardioplegic solution containing potassium chloride was injected into the root of the aorta to produce cardiac arrest. After a sump cannula was placed in the left atrium, the main pulmonary artery was opened. When the occluding clamp on the ascending aorta was released briefly, the orifice of the anomalous vessel was identified in the right pulmonary artery. The circumflex artery was detached from the pulmonary artery and implanted into the right posterolateral aspect of the ascending aorta (Fig. 2,C and/}). The postoperative course was uncomplicated. The parents of the child refused permission for repeat cardiac catheterization, but a radionucleotide cardiac scan at the time of dis-
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Anomalous origin of circumflex coronary artery
Fig. 1. Serial biplane angiograms demonstrating retrograde filling of the anomalous circumflex coronary artery via collaterals from the left anterior descending coronary artery (LAD). Lateral views showing (A) injection of dye into the LAD and (B) retrograde filling of the circumflex coronary artery from the LAD. Anteroposterior views showing (C) injection of dye into the aortic root with filling of the LAD and (D) retrograde filling of the circumflex (arrows) from the LAD.
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Orifice of circumfkx. artery identified through Pulmonary arteriotomy.
a.Anomlou$ CircuMex Coronary A bMormal left Anterior Iksc. Cor A cNormal Right Cor A.
Anomalous circumfkx coronary a. ligaied.
Circumfkx arlzry anastomosed to posterior surface of aorta. Fig. 2. Drawing showing surgical technique used in transferring the origin of the anomalous circumflex coronary artery to the right side of the ascending aorta. The anastomosis was performed with 6-0 polypropylene sutures. In future cases consideration will be given to removing a plug of right pulmonary artery with the coronary artery to facilitate the aortic anastomosis. A patch graft of pericardium would then be used to close the defect in the pulmonary artery.
charge revealed no evidence of recent myocardial damage. One year after operation the patient is asymptomatic and has a normal multistage stress electrocardiogram without arrhythmia or ischemic changes. Discussion Surgical treatment of coronary artery anomalies has evolved during the past 20 years. Early attempts at palliation such as pulmonary artery banding and aor-
topulmonary shunts were unsuccessful. 7, 8 Similarly, ligation of the anomalous vessel at its site of origin does little to improve perfusion and makes the heart totally dependent on collaterals from the remaining single vessel. The most satisfactory operations are those that channel systemic oxygenated blood to the anomalous vessel. Mustard 9 attempted such a shunt in 1953 using the carotid artery, but the patient died. Other surgeons
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have constructed shunts to the anomalous vessel using the right or left subclavian artery through a posterolateral thoracotomy incision. 1 0 - 1 4 The first successful total correction of anomalous origin of a coronary artery was performed at this institution in 1964, by constructing a Dacron bypass graft to the anomalous vessel and ligating the vsssel at its site of origin. 15 The success of this method with an autogenous saphenous vein graft has been documented in previous publications. 16 - 17 The bypass technique, however, is difficult to perform in infants, and the longterm patency of the graft is not dependable. The procedure which provides the most ideal anatomic correction is transplantation of the orifice of the anomalous vessel to the ascending aorta. When the left coronary artery arises from the posterior base of the main pulmonary artery, it can usually be transferred without tension to the adjacent aortic root. If the attachment is located laterally, the coronary artery may be under considerable tension when the transfer is attempted, and in such cases ligation of the artery with vein or internal mammary bypass should be used. We recently described our technique for correcting this anomaly using total cardiopulmonary bypass and dividing the main pulmonary artery. l s ' 19 Neches and associates 20 have reported success with a similar approach. The anterior location of an anomalous right coronary artery facilitates such a transfer of the origin of the vessel to the adjoining right aortic sinus, and this procedure may be performed with or without cardiopulmonary bypass. 21 In the patient reported herein the exact site of origin of the anomalous vessel could not be determined by angiography. When the main pulmonary artery was opened at operation, the orifice of the anomalous circumflex coronary artery was not immediately apparent. Briefly releasing the aortic clamp allowed localization of retrograde flow to the right pulmonary artery, and the orifice of the anomalous vessel was then identified (Fig. 2, B). Dissection and transplantation of the anomalous vessel was accomplished by retracting the aorta ot the left, and complete division of the pulmonary artery was unnecessary (Fig. 2, C and D). We have now successfully used the technique of direct reimplantation of an anomalous left or circumflex coronary artery in seven patients who were from 10 weeks to 21 years of age. The use of cardiopulmonary bypass with cardioplegic arrest and transplantation of the origin of the anomalous vessel offers definite advantages in terms of adequate visualization, technical simplicity, and applicability to small infants. Exposure
Anomalous origin of circumflex coronary artery
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is enhanced by the median sternotomy incision and flaccid heart. The anatomy can then be adequately evaluated and alterations in the planned correction made when unusual anomalies are found. This flexibility is not possible when the left or right thoracotomy approach is used. In addition, the more anatomic correction provided by direct reimplantation of the origin of the anomalous vessel should result in higher longterm patency of the artery. We, therefore, consider cardiopulmonary bypass with cardioplegic arrest and direct implantation the technique of choice for correction of most anomalies of the coronary arteries. REFERENCES 1 Agustsson MH, Gasul BM, Fell EH, Graettinger JS, Bicoff JP, Waterman DF: Anomalous origin of left coronary artery from the pulmonary artery. JAMA 180:15, 1962 2 Wesselhoeft H, Fawcett JS, Johnson AL: Anomalous origin of the left coronary artery from the pulmonary trunk. Circulation 38:403, 1968 3 Gasul BM, Arcilla RA, Lev M: Anomalous origin of coronary artery from pulmonary artery. Heart Disease in Children, Philadelphia, 1966, J. B. Lippincott Company, pp 976-1000 4 Perry LW, Scott LP: Anomalous left coronary artery from pulmonary artery. Report of 11 cases; review of indications for and result of surgery. Circulation 41:1043, 1970 5 Likar I, Criley JM, Lewis KB: Anomalous left coronary artery arising from the pulmonary artery in an adult. A review of the therapeutic problem. Circulation 33:727, 1966 6 Ogden JA: Congenital anomalies of the coronary arteries. AmJ Cardiol 25:474, 1970 7 Potts WJ: Cited by Kittle CF, Diehl AM, Heilbrunn A: Anomalous left coronary artery arising from the pulmonary artery. J Pediatr 47:198, 1955 8 Case RB, Morrow AG, Stainsby W. Nestor JO: Anomalous origin of the left coronary artery. The physiologic defect and suggested surgical treatment. Circulation 17:1062, 1958 9 Mustard WT: Anomalies of the coronary artery, Pediatric Surgery, DC Benson, WT Mustard, MM Ravitch, WH Synder Jr. KJ Welch, eds., Chicago, 1962, Year Book Medical Publishers, Inc., p 433 10 Apley J, Horton RE, Wilson MG: The possible role of sugery in the treatment of anomalous left coronary artery. Thorax 12:28,1957 11 Meyer BW, Stefanik G. Stiles QR. Lindesmith GG, Jones JC: A method of definitive surgical treatment of anomalous origin of left coronary artery. J THORAC CARDIOVASC SURG 56:104, 1968
12 Doty DB, Chandramouli G, Schieken RE, Lauer RM. Ehrenhaft JL: Anomalous origin of the left coronary ar-
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17 Meyer J, Reul GJ Jr, Chiariello L, Hallman GL, Cooley DA: Anomalous origin and distribution of coronary arteries. Review of 38 patients who underwent operation. J Cardiovasc Surg 16:500, 1975 18 Grace RR, Angelini P, Cooley DA: Aortic implantation of anomalous left coronary artery arising from pulmonary artery. Am J Cardiol 39:608, 1977 19 Cooley DA, Norman JC: Techniques in Cardiac Surgery, Houston, 1975, Texas Medical Press, p 170 20 Neches WH, Mathews RA, Park SC, Lenox CC, Zuberbuhler JR, Siewers RD, Bahnson HT: Anomalous origin of the left coronary artery from the pulmonary artery. A new method of surgical repair. Circulation 50:582, 1974 21 Tingelstad JG, Lower RR, Eldredge WJ: Anomalous origin of the right coronary artery from the main pulmonary artery. Am J Cardiol 30:670, 1972
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