Coronary arteries arising from a solitary ostium in the aorta are rare congenital malformations that usually do not produce ischemia. Like normally arising arteries, however, such single coronary arteries and their branches are prone to atherosclerosis that causes ischemia. We describe a patient with a solitary coronary ostium in whom an angina-producing stenosis in the ramus intermedius branch of the left coronary artery was relieved by balloon angioplasty and then review 17 earlier individual case reports of angioplasty of various branches of single coronary arteries. Case Report. A 71-year-old woman with a history of systemic hypertension presented to the emergency department with angina pectoris. Coronary arteriography revealed a solitary coronary artery arising from the right sinus of Valsalva of the aorta. After an early bifurcation from the right coronary artery, the left passed behind the aorta to reach the left ventricle where it divided into three branches: the left anterior descending, the circumflex, and, between them, the ramus intermedius, which had a 95% luminal diameter stenosis proximally (Fig. 1). This narrowing was dilated using a 2.5 mm and then a 2.75 mm Hartzler ACX balloon catheter (Guidant Corp., Minneapolis, MN) passed through a left Amplatz 3 guiding catheter and over a High-Torque Floppy guidewire (Guidant Corporation). The stenosis was reduced to 10–20% of the luminal diameter (Fig. 1). The post-procedural course was uneventful. Discussion. In 1993, Shirani and Roberts reviewed the world’s literature and found reports of 35 solitary coronary arteries confirmed at necropsy and 52 diagnosed by coronary arteriography in patients without other major congenital cardiovascular malformations.1 The authors added 10 more cases established at necropsy and classified all 97 cases into 20 categories based on the location of the solitary coronary ostium (left sinus of Valsalva in 46 and right sinus in 51), the presence or absence of an aberrantly coursing coronary artery, and the course taken by the aberrantly coursing coronary artery. The patient we have described fits into category IIB4 of Shirani and Roberts, as did 11 of their 97 patients. Those authors also found that 8 of 53 patients without significant atherosclerotic narrowing of the branches of the single coronary artery had evidence of ischemia, whereas 30 of 32 with significant narrowing had angina or myocardial infarction. Soon after Gruentzig's initial description of coronary balloon angioplasty,2 reports of angioplasty of atherosclerotic narrowings in anomalously arising coronary arteries began to appear.3 Subsequent reports have described angioplasty, with or without stenting, of one or more branches of a single coronary artery in 18 patients including ours (Table 1).4–20 The solitary ostium was in the left sinus of Valsalva in 8 patients and in the right sinus in 10. Sixteen patients had one coronary arterial branch dilated, and two had two branches angioplastied. The branches dilated have been the right coronary artery in 9 patients, the left circumflex or its obtuse marginal branch in 5, the left anterior descending in 4, the left main in 1, and the ramus intermedius in 1 (our patient). Seven of the arteries received stents, and 13 were angioplastied without stenting. A 73-year-old patient who presented with ventricular fibrillation followed by cardiogenic shock was the only patient to die in the immediate post-procedural period.17 Atherosclerotic stenoses in anomalously arising coronary arteries are just as amenable to successful angioplasty with a good long-term result as are stenoses in normally arising arteries. Some diagnostic, strategic, and clinical problems may arise, but these are solvable. The first problem is diagnosing the anomaly itself, and it is usually solved during the diagnostic study. In the case of a single coronary artery, its orifice must be located accurately, another orifice excluded, and the course of each of the major branches defined, especially the course of the branch to be dilated. The second problem is deciding how to approach the stenosis. Here selection of the appropriate guiding catheter is of utmost importance. Usually a configuration similar to that of the diagnostic catheter will work for the guide, but because more support is needed for the angioplasty, occasionally a differently configured guide will be needed. In general, guides need to match the sinus of Valsalva from which the artery arises rather than the name of the artery. As indicated in Table 1, the guides that were used successfully in these 18 patients with single coronary arteries are, with rare exceptions, guides that frequently are employed successfully when coronary arteries arise normally from the same sinus. Finally, technical difficulties may arise because of the course of the branch to be dilated, but with good coaxial guide support such difficulties usually are easily overcome. When working through a solitary coronary ostium, the operator should always be mindful that this is the myocardium’s sole source of blood.
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