BfWEF C-CATIONS
Tr ansluminal angi coronary artery
ty of a single
J. C. Stauffer, MD, U. Sigwart, MD, P. Vogt, MD, D. Aymon, RN, and L. Kappenberger, MD. Lausanne, Switzerland
Coronary artery anomalies are rare and occur in 0.2% to 1.2% of the population. 1,2 The incidence of a single coronary ostium has been approximated at 0.03% to 0.4% .rm3 This rare congenital coronary abnormality has been reported in association with other congenital defects such as pulmonary stenosis and has been implicated in myocardial ischemia, infarction, and sudden death.4*6 Anomalous vessels are thought to significantly alter myocardial perfusion through mechanisms different from those of atherosclerotic disease; on the other hand, they can develop “typical” atherosclerotic coronary artery disease. A few studies have even suggested a predilection for accelerated atherosclerosis in some forms of coronary artery anomalies.7 Transluminal coronary angioplasty is an established alternative for treating patients with ischemic coronary artery disease. Improvements in technology and operator expertise have allowed an increase in the number of patients eligible for this therapeutic option. A 63-year-old woman with hypertension, who had a 2-year history of typical angina, was admitted to our institution because of worsening symptoms over the last month while she was on a regimen of beta blockers, nitrates, and calcium channel blockers. The chest pain was associated with ECG changes in the inferior leads and always responded to sublingual nitroglycerin. Results of subsequent cardiac catheterization showed normal global left ventricular systolic function with inferior wall hypokinesia. The left ventricular end-diastolic pressure was elevated to 20 mm Hg. Selective coronary angiography was performed by means of the Judkins technique, but it was impossible to find the ostium of the left coronary artery. This vessel was found to originate from the right coronary sinus together with the right coronary artery as a single vessel (Fig. 1). The transverse trunk passed anterior to the conus of the right ventricle (proved by aortic root injection). The “right coronary artery” was dominant, and a long eccentric 95% lesion was noted at its midsegment. The proximal and distal right coronary artery and the midcircumflex artery showed narrowing in the range of 30 % to 40 % , which was not considered significant. The left anterior descending artery was free of disease. The common ostium was intubated with an 8F right Judkins guiding catheter with side holes. The From the Division of Cardiology, Centre Hospitalier Universitaire Vaudois. Reprint requests: J. C. &au&r, MD, Division of Cardiology, Centre Hospitalier Universitaire Vaudois, 1011 Lausanne, Switzerland. 4/4/29738
stenosis was initially crossed with a skinny 25/2.5 mm balloon (Scimed Life Systems, Inc., Maple Grove, Minn.) and a 0.014-inch high-torque floppy J-tipped guide wire (Advanced Cardiovascular Systems, Inc., Santa Clara, Calif.). Care was taken whenever possible to keep the guiding catheter away from the common ostium to minimize ischemia of the coronary tree. Three balloon inflations were performed at 6, 8, and 12 bars, A dissection at the site of the lesion was noted, which was associated with transient chest pain and EGG changes. The 2.5 mm balloon was then exchanged for a 25/3.0 mm balloon (Scimed), and the three inflations were repeated at a maximum pressure of 12 bars for 30 seconds. After the angioplasty procedure, an angiogram showed that the dilated vessel had a residual stenosis of less than 50% (Fig. 2). The patient continued to receive intravenous heparin for 24 hours. Bicycle ergometry performed 3 days later showed no angina or ischemia ECG changes while the patient was on a regimen of calcium channel blockers and aspirin only. The patient has been asymptomatic for 8 months since the angioplasty. This case is an example of successful percutaneous transluminal coronary angioplasty performed on a patient with active ischemia and a severe atherosclerotic lesion of the “right coronary artery” branch of a single coronary artery. This therapeutic approach to treating patients with anomalous coronary arteries is presently not well defined. It has been suggested that some of the ischemia observed in these patients could be the result of a diminished vasodilatation response or in some instances compression of the anomalous arteries by the pulmonary artery and the aorta11 6 The clinical significance of this rare anomaly has been a matter for debate. Sudden death in young patients with this anomaly has been reported and it has been suggested that external compression during exercise could limit coronary flow in these patients. The prolonged history of angina and the inferior ECG changes associated with lesions of the “right coronary artery” strongly suggest that the stenosis was responsible for the symptoms of angina. The transverse trunk of the single coronary artery, crossing anteriorly to the conus of the right ventricle, did not predispose this artery to external compression, as would have been the case with passage between the aorta and the pulmonary artery. There is definite increased procedural risk when angioplasty is performed in a patient with a single ostium. Dissection of this ostium by the guiding catheter would have led to a catastrophic event. Soft-tip guiding catheters, steerable high-torque floppy guide wires, and low-profile balloons make it easier to consider symptomatic patients with severe stenosis of the anomalous coronary artery as candidates for percutaneous transluminal coronary angioplast$ Care should be taken to determine the exact meehanism responsible for the ischemia and define as precisely as possible the exact anatomy. According to the site of origin and anatomic distribution of the branches, isolated single coronary arteries are classified into three different 569
570
Brief Communications
Amsdcan
August 1901 Heart Journal
1. Preangioplasty coronary arteriogram of single coronary artery arising from right coronary sinus in right anterior oblique projection. Circumflex vessel is not well demonstrated (catheter tip being past its orifice). A 95% midsegment right coronary artery stenosis is present.
Fig.
2. Postangioplasty coronary arteriogram in right anterior oblique projection. Residual stenosis is estimated to be 40%.
Fig.
group~,~ which can be further subdivided into two major variants. One consists of an essentially normal coronary artery tree arising from a single right main trunk with fairly typical right and left anterior descending and circumflex vessels (our patient). The other variant involves a large single right coronary vessel with extensive ramifications supplying the whole myocardium. Certainly angioplasty of a lesion in a proximal or midportion of the second variant would not be appropriate. Apart from the potential risk to the ostium, a dissection at the site of angioplasty could greatly compromise coronary blood flow to a major portion of the myocardium and thus put the patient at a prohibi-
tive risk. Once it is decided that angioplasty is the therapeutic modality, the equipment should be chosen carefully. Inasmuch as some technical difficulties may be encountered more frequently and carry significant risk, only physicians experienced with angioplasty should perform this procedure. REFERENCES
1. Liberthson RR, Dinsmore Rl3, Bharatis S, Ruben&in JJ, Caulfield J, Wheeler EO, Harthorne JW, Leu M. Aberrant coronary artery origin from the aorta. Circulation 1974;50: 774-9.
Volume 122 Number 2
Brief Communications
2. Kimbiris D, Iskandrian AS, Segal BL, Bemis CE. Anomalous aortic origin of coronary arteries. Circulation 1978;5:606-15. 3. Lipton MJ, Barry WH, Obrez I, Silverman JF, Wexler L. Isolated single coronary artery: diagnosis, angiographic classification and clinical significance. Radiology 1979;130:39-47. 4. Perloff J. Congenital pulmonic stenosis in the clinical recognition of congenital heart disease. Philadelphia: WI3 Saunders, 1978:185-221. 5. Cheitlin MD, De Castro CM, McAllister HA. Sudden death as a complication of anomalous left coronary origin from the anterior sinus of Valsalva. Circulation 1974;50:780-7. 6. Sharbaugh AH, White RS. Single coronary artery: analysis of the anatomic variation, clinical importance and report of five cases. JAMA 1974;230:243-6. 7. Click RL, Holmes DR, Vliestra RE, Kosinski AS, Kronmal RA. Anomalous coronary arteries: location, degree of atheroselerosis and effect on survival-a report from the Coronary Artery Surgery Study. J Am Co11 Cardiol 1989;13:531-7.
Tra geal color Doppler diagnosis of anomabus origin of teft circumflex coronary artery Tandaw E. Samdarshi, MD, Douglas L. Hill, MD, and Navin C. Nanda, MD. Birmingham, Ala.
From the University
Division of Cardiovascular of Alabama at Birmingham.
Disease,
Department
Reprint requests: Navin C. Nanda, MD, University of Alabama ham, Heart Station, SWUiSlO2, Birmingham, AL 35294. 414129746
of Medicine, at Birming-
57 1
Evaluation of the anatomy of the coronary arteries is usually done by coronary arteriography. Transesophageal color Doppler echocardiography has added a new dimension to the scanning of coronary arteries. We report here, for the first time, a case of anomalous origin of the left circumflex coronary artery from the right sinus of Valsalva diagnosed by tranaesophageal color Doppler echocardiography (TEE). The patient was a 33-year-old white woman with a history of several episodes of near syncope in the preceding few months. On examination, she had a heart rate of 98 beats/min and a blood pressure of 124/80 mm Hg. Precordial auscultation reveafed a grade III/VI systolic ejection murmur with radiation to the neck. Blood and other laboratory analysis were unremarkable. A conventional twodimensional color Doppler examination revealed a congenital bicuspid aortic valve with a peak gradient of 75 mm Hg (mean 44 mm Hg) across it, and a valve area of 1.05 cm2. Mild aortic regurgitation was seen. Other cardiac parameters were normal. Coronary angiograms demonstrated a normal left main and left anterior descending coronary artery. The left circumflex artery arose anomalously from the right coronary sinus, traversed posteriorly behind the aorta, and supplied the left circumflex system. The right coronary artery was normal. The bicuspid aortic valve had a mean gradient of 45 mm Hg across it. Transesophageal color Doppler examination was performed intraoperatively using a Hewlett-Packard ultrasound imaging system (model 77020A, Hewlett-Packard Co., Medical Products Group, Andover, Mass.) and a transesophageal 5 MHz model 21362A probe. A short-axis view of the aortic valve was obtained at the level of the aortic root, and then the
Fig. 1. Anomalous origin of the left circumflex coronary artery from the right sinus of Valsalva. Transesophageal color Doppler examination. A, Oblique aortic short-axis view demonstrates the right-sided origin of the anomalous vessel (CX), which then angles sharply posteriorly and subsequently takes on a leftward course between the aorta (AO) and the left atrium (LA). Note the presence of color flow signals within the vessel lumen. RA, Right atrium.