Single coronary artery (SCA) anomaly is characterized by the presence of a single coronary ostium, with both the left and right coronary artery systems arising from it. This congenital abnormality is very rare (0.006%) and is usually discovered as an incidental finding at the time of coronary angiography.1 We describe the case of a lady presenting with an acute coronary syndrome, in whom coronary angiography showed single coronary ostium and multivessel atherosclerotic disease.
A 71-year-old female presented to the emergency department of our hospital complaining of severe dyspnea. Her cardiovascular risk factors included type 2 diabetes mellitus, obesity (BMI 31.3) and arterial hypertension; her heart rate was 95 bpm and blood pressure was 170/90 mmHg.
Both physical examination and chest x-ray showed pulmonary congestion; lung examination showed basal rales, so the patient was given intravenous (IV) furosemide with improvement of dyspnea and physical signs of heart failure. High-sensitivity troponin I was 0.146 ng/ml at admission and 0.466 ng/ml after 3 hours (normal range 0-0.12 ng/ml). The electrocardiogram (ECG) showed paced rhythm (a DDDR pacemaker had been implanted 8 months ago for complete heart block). Estimated glomerulal filtration rate (Cockroft-Gault) was 93 ml/min.
The echocardiogram showed reduced left ventricle ejection fraction (35%) with global hypokinesia and increased pulmonary pressure (65 mmHg); interestingly, the previous echocardiogram performed at the time of pacemaker implant showed a preserved left ventricular ejection fraction.
The patient underwent coronary angiography. Given the absence of the left main ostium in the left coronary sinus, aortography was performed, which showed a SCA arising from the right sinus of Valsalva (Figure 1), with a very small circumflex branch, a dominant right coronary artery (RCA) with multiple significant stenoses (Figure 2, left panel), and a left anterior descending coronary artery (LAD) with diffuse disease and a tight stenosis in the mid portion (Figure 3, left panel).
Given the presence of multivessel coronary artery disease, diabetes mellitus and low ejection fraction, the procedure was interrupted and the case was discussed by the heart team. The collegial decision was in favor of percutaneous coronary intervention (PCI), mainly because of the small caliber of the distal LAD.
One week later, after effective medical treatment of heart failure and pretreatment with double antiplatelet treatment and statins, percutaneous coronary intervention was performed via the left radial approach; the single ostium was engaged with a 6F right Judkins 4.0 guide catheter (Cordis Corporation). The RCA was treated first with predilatation and stenting of the first and second posterolateral (PL) branches. Two drug-eluting stents were implanted: a 2.25 x 14 mm Resolute Integrity (Medtronic) on the first and a 2.25 x 15 mm Xience Prime (Abbott Vascular) on the second PL, respectively (Figure 2, right panel).
We treated a calcific lesion on the posterior descending artery (PD), but we failed to implant a stent despite predilatation and use of a buddy wire. Similar difficulties were encountered in the treatment of the LAD, which presented a tortuous course with a tight, calcific lesion in the mid portion. In both vessels, the result after plain old balloon angioplasty (POBA) was acceptable (Figure 2, right panel; Figure 3, right panel). Fluoroscopy time was 17 minutes and 36 seconds, whereas dose area product was 14716 cGy.cm2.
The patient’s in-hospital course was unremarkable. She was discharged asymptomatic and without physical signs of heart failure. She did well in the following months, but unfortunately, she died suddenly at home 5 months post procedure.
SCA is a rare congenital anomaly in which both the left and the right coronary system arise from a single aortic ostium. Depending on the origin (left or right aortic sinus), and the course of coronary vessels, several classifications of SCA have been proposed.2,3 Although usually asymptomatic and discovered at coronary angiography, SCA may be associated with myocardial ischemia and sudden cardiac death4,5, even in the absence of obstructive coronary atherosclerotic disease; this is more frequent in the variants with a course of coronary vessels between the aorta and pulmonary artery, possibly due to an aberrant course within the aortic wall and lateral compression.6,7 Percutaneous coronary intervention in SCA is feasible but technically demanding, because guide catheter back up may be suboptimal and intracoronary navigation of devices may be difficult due to tortuous angles and an unusual vessel course. Moreover, the presence of a single coronary ostium requires very careful engagement and manipulation of the guide catheter, in order to prevent potentially catastrophic vessel dissections.8,9 The transradial approach has been reported to be feasible in percutaneous coronary intervention of single coronary artery.10,11
In this case, although the first diagnostic procedure was performed by a right transradial approach, the second procedure was scheduled as a left transradial approach for two reasons: first, the patient was an obese female and the transradial approach is associated with a lower risk of vascular complications in these patients as compared to the transfemoral approach12; second, since the catheter back up with the right transradial approach is lower when compared to the transfemoral approach13, a left transradial approach was selected in order to have optimal guide catheter back up.14 In spite of this, coronary stenting was successful in the PL branches of the right coronary artery, but it failed in both the PD and LAD. In attempting PD stenting, we used the buddy wire technique, which can be useful in challenging anatomies15, but it was not successful. However, given the good angiographic result achieved with balloon angioplasty and the small vessel size, we accepted this result without attempting more aggressive maneuvers (e.g., deep guide engagement, use of more supportive wires, debulking devices, guide catheter extensions), in order to avoid trauma to the single coronary ostium. Indeed, although some additional devices were reported to be effective when dealing with coronary anomalies, their use is not free from complications.16
Unfortunately, our therapeutic strategy was not successful, since the patient died suddenly 5 months post procedure. According to her relatives who witnessed the event, the patient experienced epigastric pain followed by collapse before the arrival of emergency medical services. In the preceding months, the patient had been free from angina; therefore, in our opinion, one explanation could be a possible stent thrombosis, according to the Academic Research Consortium definition. Indeed, failure of stenting both the PD and the LAD, treated only with POBA, would have probably led to symptomatic restenosis rather than to sudden death; the case of a missed dissection leading to acute vessel occlusion seems unlikely, given the amount of time between the procedure and the adverse event.
The authors can be contacted via Stefano Rigattieri, MD, at firstname.lastname@example.org.
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- Shirani J, Roberts WC. Solitary coronary ostium in the aorta in the absence of other major congenital cardiovascular anomalies. J Am Coll Cardiol. 1993; 21: 137-143.
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- Basso C, Maron BJ, Corrado D, Thiene G. Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes. J Am Coll Cardiol. 2000; 35(6): 1493-1501.
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- Benamer H, Louvard Y, Sanmartin M, et al. A multicenter comparison of transradial and transfemoral approaches for coronary angiography and PTCA on obese patients: the TROP registry. EuroIntervention. 2007; 3: 327-332.
- Ikari Y, Masuda N, Matsukage T, et al. Backup force of guiding catheters for the right coronary artery in transfemoral and transradial interventions. J Invasive Cardiol. 2009; 21: 570-574.
- Ohanessian A, Spaulding C, Varenne O, et al. The left transradial approach. In: Hamon M, McFadden E (ed). Trans-radial Approach for Cardiovascular Interventions. 2nd ed. Colombelles: Europa Stethoscope Media Editions, 2010: 61-70.
- Rigattieri S, Hamon M, Grollier G. The Buddy wire technique is useful in transradial coronary stenting of complex, calcified lesions: report of three cases. J Invasive Cardiol. 2005; 17: 376-377.
- Chang YC, Fang HY, Chen TH, et al. Left main coronary artery bidirectional dissection caused by ejection of guideliner catheter from the guiding catheter. Catheter Cardiovasc Interv. 2013 Feb 14 (Epub ahead of print).