Asian Cardiovasc Thorac Ann 2008;16:305-308
© 2008 Asia Publishing EXchange Ltd
Anomalous Origin of Right Coronary Artery From Left Coronary Sinus
Gadah Hamzeh, MD,
Alex Crespo, MD,
Rafael Estarán, MD1,
Miguel A Rodríguez, MD,
Roberto Voces, MD,
José I Aramendi, MD
Division of Cardiac Surgery, Hospital de Cruces, Barakaldo
1 Division of Cardiology, Hospital Santiago Apóstol, Vitoria, Spain
For reprint information contact: José I Aramendi, MD, Tel: 34 94 600 6339, Fax: 34 94 600 6079, Email: JI.ARAMENDI{at}terra.es, Division of Cardiac Surgery, Hospital de Cruces, Barakaldo 48903, Spain.
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ABSTRACT
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Anomalous aortic origin of the coronary arteries is uncommon but clinically significant. Manifestations vary from asymptomatic patients to those who present with angina pectoris, myocardial infarction, heart failure, syncope, arrhythmias, and sudden death. We describe 4 patients, aged 34 to 59 years, who were diagnosed with right coronary artery arising from the left sinus of Valsalva, confirmed by coronary angiography, which was surgically repaired. Three patients presented dyspnea and angina, and one with acute myocardial infarction. At operation, the right coronary artery was dissected at the take-off from the intramural course, and reimplanted into the right sinus of Valsalva. There was no mortality. One patient had associated coronary artery disease that required stent placement postoperatively. This reimplantation technique provides a good physiological and anatomical repair, eliminates a slit-like ostium, avoids compression of the coronary artery between the aorta and the pulmonary artery, and gives superior results to coronary artery bypass grafting or the unroofing technique.
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INTRODUCTION
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Anomalous aortic origin of the coronary arteries is uncommon but clinically significant. Congenital anomalies of the coronary arteries appear in coronary angiography with an incidence of 1%. Of these, 90% are abnormalities of the origin of the coronary arteries, and the rest are coronary artery fistulas.1 Two-thirds of anomalous origins are the circumflex artery arising from the right sinus and crossing behind the aorta, or the anterior descending artery and circumflex arising separately from the left sinus; the other third are aberrant origin of the right coronary artery (RCA) from the left sinus of Valsalva. This anomaly has been implicated as a possible cause of chest pain, myocardial ischemia, ventricular arrhythmias, and sudden death, and in most cases, it is associated with exercise.2,3 We describe 4 cases of RCA arising from the left sinus of Valsalva, which were confirmed by coronary angiography and surgically repaired. At operation, the RCA was reimplanted into the right sinus of Valsalva.
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PATIENTS AND METHODS
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We retrospectively reviewed our experience of anomalous origin of the RCA. From 2003 to 2007, 4 patients were diagnosed with anomalous aortic origin of the RCA arising from the left sinus. All of them were adults with ages ranging from 34 to 59 years (mean age, 49 years). Three patients had clinical symptoms of angina pectoris, dyspnea, and acute congestive heart failure. The 4th patient presented with acute septal myocardial infarction. One patient had a history of asthma and respiratory infection, the other 3 had at least 1 cardiovascular risk factor. The definitive diagnosis was obtained by cardiac catheterization in the first 3 cases, and by multislice computed tomography in the last case; all had an anomalous origin of the RCA from the left sinus of Valsalva, with an intramural segment (Figure 1
). The coronary angiogram showed diffuse stenosis of the anterior descending coronary artery in one patient. An echocardiographic study in this patient revealed severe mitral regurgitation due to chordal rupture at the posterior leaflet, moderate tricuspid regurgitation, and severe pulmonary hypertension. One patient had irregularities of the proximal RCA, which were considered < 50% stenosis. Exercise testing was performed in only one individual; it was clinically negative, but the stress electrocardiogram showed alterations.
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Figure 1. (A) Coronary angiogram showing a superior concavity in the proximal intramural course of the right coronary artery (arrow); (B) Multislice computed tomography: arrow indicates the intramural segment of the right coronary artery.
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The anomalous coronary arteries were repaired using standard cardiopulmonary bypass, aortic cross clamping, and cold blood cardioplegia. The RCA was located arising from the left sinus of Valsalva. The RCA was dissected and mobilized over a few centimeters up to the take-off of the conal branch, prior to cross clamping the aorta (Figure 2). The RCA was ligated and dissected immediately after its intramural course. It was trimmed and tailored for anastomosis. After aortic cross clamping, a small transverse aortotomy was performed, and the locations of the aortic valve commissure and right sinus of Valsalva were identified. A round incision was made in the sinus wall with a 3.5-mm punch. The RCA was reimplanted by end-to-side anastomosis using a running suture of 7/0 polypropylene. The aortotomy was closed, and the operation was finished in the usual manner. Mean ischemic and pump times were 56 and 82 min, respectively, for all 4 patients (34 and 50 min for the 3 patients without associated lesions). In the last 2 patients, coronary blood flow through the RCA was measured with a transit-time flowmeter (Medistim, Oslo, Norway). Flow rates of 30 and 38 mL·min–1 with good diastolic flow were registered, confirming the quality of the anastomoses. Mitral valve repair was performed in the patient with mitral regurgitation, with posterior leaflet resection and Carpentier ring implantation in the routine manner. In addition, coronary artery bypass grafting was undertaken by anastomosing the left internal mammary artery to the mid part of the anterior descending artery.
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Figure 2. Surgical view; (A) Arrow indicates the proximal intramural course of the right coronary artery from the left sinus; the right coronary artery is dissected free from the atrioventricular groove fat over a 3-cm length (between the vessel loops); (B) The right coronary artery is reimplanted into the corresponding sinus (arrow).
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RESULTS
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There was no operative death. Postoperatively, the patient with proximal irregularities of the RCA suffered angina pectoris during the first 48 hours, and coronary angiography revealed 50% stenosis of the RCA 1-cm distal to a well-patent anastomosis. This lesion had been considered insignificant in the preoperative study. A stent was implanted in the RCA. Postoperative recovery was uneventful in the other 3 patients, and follow-up showed no complications. Treadmill tests were carried out in 2 patients, which revealed no evidence of ischemia. An echocardiogram at 6 months postoperatively in the patient with concomitant mitral valve repair demonstrated no mitral regurgitation, normal left ventricular motion and pulmonary hypertension that had decreased to mild. Electrocardiograms were normal in all 4 patients. At a mean follow-up of 24 months, all patients were asymptomatic and had returned to their normal activities.
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DISCUSSION
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Anomalous origin of the coronary arteries was once considered an incidental finding without clinical significance; however, these abnormalities may be responsible for angina pectoris, heart failure and increased risk of sudden death in young individuals. Nowadays, anomalous origin of coronary arteries represents the 2nd most frequent cause of athletic-field deaths in the United States.4 Reported presentations vary from asymptomatic patients to those with angina pectoris, myocardial infarction, heart failure, syncope, arrhythmias and sudden death.5 Because of this, treatment must be targeted at those with increased risk of sudden death. That is, patients whose anomalous artery is dominant, those who have a proximal intramural course of the anomalous vessel, and those who present with symptoms before 35 years of age. With older patients, the chief aim is good symptomatic relief because the risk of sudden death decreases with age.
There are 2 types of anomalous origin of coronary arteries: from the pulmonary artery, and from the aorta. Aortic origin of the coronary arteries can be divided into abnormalities of number (excess or absence of ostium), and abnormalities of situation.1 The most common anomaly is aberrant origin of the left main coronary or RCA from the wrong sinus of Valsalva. In this report, we focused on anomalous origin of the RCA from an ostium in the left sinus. This ostium is located just above and ahead of the normal left coronary ostium, near the commissure between the left and right sinuses. The initial section of the artery has an oblique intramural course and its ostium is slit-like, narrowed, and collapses in a valve-like manner. After leaving the aortic wall, the artery follows its normal course through the atrioventricular groove.
Angina, infarction, and sudden death in these patients are triggered by myocardial ischemia. The ischemia appears to be secondary to a combination of several factors that produce obstruction, thereby limiting blood flow: compression of the anomalous vessel along its course between the aorta and the pulmonary artery, a slit-like coronary ostium, and an acute angle of take-off of the anomalous vessel from the aortic wall.6 Roberts and colleagues7 found pathological evidence of insufficient coronary blood flow and myocardial damage, such as patchy areas of myocardial fibrosis and small infarcts. These injuries appear in patients who suffer sudden death in the absence of atherosclerotic disease. Detailed assessment of the coronary arteries by imaging is the basis of the diagnosis. Transthoracic echocardiography is the recommended primary noninvasive modality.8,9 In most patients, it can delineate both coronary ostia; however, color Doppler provides good anatomic definition of the proximal intramural course of the anomalous vessel. In cases of diagnostic doubt, transesophageal echocardiography can identify the origin of the coronary arteries. The definitive diagnosis was achieved by coronary angiography in our patients. Noninvasive magnetic resonance imaging or multislice computed tomography can clearly delineate the anatomy and have recently replaced angiography as definitive diagnostic tools.10–12
Several techniques have been used to treat this anomaly, including intracoronary stent implantation by standard percutaneous techniques.12,13 Stent use in the RCA is limited because it does not correct either the aberrant ostium or the oblique proximal course of the artery. Coronary artery bypass grafting using saphenous vein or right internal mammary artery anastomosed to the RCA can be accomplished off-pump. There is no need for an aortotomy, but competitive flow between the native RCA and the bypass graft could develop, leading to graft malfunction. Furthermore, in a young patient, there is a potential need for late re-intervention due to bypass graft occlusion. Recently, Fedoruk and colleagues14 reported an unacceptable occlusion rate of right internal mammary artery bypass grafts due to competitive flow.15,16 Unroofing technique, by this surgical approach, the proximal intramural course of the coronary artery is longitudinally incised, unroofing this segment of the RCA. It requires detachment and re-suspension of the corresponding commissure, with a potential risk of aortic insufficiency. The intimas of the coronary artery and the aorta need to be sutured together to create a new long right coronary ostium. This is technically demanding and requires longer cross clamp times than the other techniques. It may be more adequate when the left coronary artery arises from the right sinus, because reimplantation of the left main coronary artery may be difficult to accomplish. Reimplantation of the RCA ostium provides a good physiological and anatomical repair.17–19 This way, anomalous implantation is corrected and a slit-like ostium is eliminated, avoiding an oblique course along the anterolateral aortic wall and compression of the section of artery that courses between the aorta and the pulmonary trunk. There is no manipulation of the aortic valve commissure, and the anastomosis is easy to perform with the aorta open. The ischemic time is short, and flow through the reimplanted artery can be measured with a flowmeter, thereby assessing the quality of the repair. We had one case of associated atherosclerotic lesions of the RCA, which were initially judged insignificant, but required stent placement postoperatively. According to Jim and colleagues,20 the presence of arteriosclerosis is not infrequent, and one must consider the need for coronary artery bypass grafting to the RCA, either alone or in association with RCA reimplantation.
It was concluded from this experience that direct reimplantation of the anomalous RCA into the right coronary sinus should be the preferred method of repair of this abnormality.
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ABSTRACT
INTRODUCTION
