Acta Cardiologica

ISSN: 0001-5385 (Print) 0373-7934 (Online) Journal homepage: http://www.tandfonline.com/loi/tacd20

Aberrant right coronary artery arising from the left sinus of Valsalva with an interarterial course Jan De Pooter, Luc Van Driessche & Jozef Bartunek To cite this article: Jan De Pooter, Luc Van Driessche & Jozef Bartunek (2014) Aberrant right coronary artery arising from the left sinus of Valsalva with an interarterial course, Acta Cardiologica, 69:2, 185-188, DOI: 10.1080/AC.69.2.3017300 To link to this article: https://doi.org/10.1080/AC.69.2.3017300

Published online: 23 May 2017.

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Acta Cardiol 2014; 69(2): 185-188

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doi: 10.2143/AC.69.2.3017300

[ Case report ]

Aberrant right coronary artery arising from the left sinus of Valsalva with an interarterial course Jan DE POOTER1, MD; Luc VAN DRIESSCHE2, MD; Jozef BARTUNEK3, MD, PhD 1

Dept. of Cardiology, University Hospital of Ghent, Belgium; 2Dept. of Cardiology, AZ Sint Blasius, Dendermonde, Belgium; 3Cardiovascular Center Aalst, OLV Hospital, Aalst, Belgium.

Abstract An anomalous origin of the right coronary artery from the left sinus of Valsalva with an interarterial course is a rather rare congenital anomaly. Treatment strategies are determined by the risk of sudden cardiac death and the presence of cardiac ischaemia. We present two cases of middleaged patients who were diagnosed with an interarterial course of a right coronary artery originating from the opposite sinus of Valsalva. As no cardiac ischaemia could be documented these patients were treated medically with excellent long-term follow-up.

Keywords Aberrant coronary artery – interarterial course.

PRESENTATION OF THE CASES Case report 1

A 61-year-old man presented with chest pain triggered by cold weather and intensive exercise. Cardiovascular risk factors included smoking cigars, up to 10 pack-years and hypercholesterolaemia. Habitual medication consisted only of alprazolam. An electrocardiogram at rest revealed no repolarization abnormalities suspect of cardiac ischaemia and the echocardiogram showed no regional wall motion abnormalities. Cyclo-ergometry showed upslope ST-segment depression in the inferior leads without clinical complaints. Coronary computed tomography angiography (CCTA) was performed and revealed an aberrant right coronary artery (RCA) arising from the left sinus of Valsalva with an interarterial course between the aorta and pulmonary artery (figures 1 and 2). The RCA showed coronary atherosclerosis with possible moderate stenosis on the proximal segment on the tomographic views. As the patient was planned for an

Address for correspondence: Jan De Pooter, Dept. of Cardiology, University Hospital of Ghent, De Pintelaan 185, Ghent, Belgium. E-mail: [email protected]. Received 1 October 2013; revision accepted for publication 22 January 2014.

elective robot-assisted prostatectomy a preoperative coronary angiogram was performed. The coronary angiography confirmed the aberrant origin of the RCA with mild atherosclerosis but without compression. As also myocardial scintigraphy revealed no arguments for cardiac ischaemia, this patient was treated with a betalytic agent, aspirin and a statin pre-operatively. No complications occurred during robot-assisted prostatectomy or postoperatively. The origin of the chest pain was assumed to be of non-cardiac aetiology. No recurrent complaints were documented during three-year follow-up. Case report 2

A 52-year-old man was referred to our outpatient clinic because of acute chest pain triggered by emotional stress. The patient stopped smoking 25 years ago and had actually no other cardiovascular risk factors except moderate obesity with hypercholesterolaemia. The electrocardiogram at rest showed a right bundle-branch block with diffuse negative T-waves. The echocardiogram revealed no regional hypo- or akinesia of the ventricular walls. Cyclo-ergometry demonstrated diffuse negative T-waves which were more pronounced in leads V1 and V2 as compared to the rest. At CCTA an aberrant RCA originating from the opposite left sinus of Valsalva with the RCA passing between the aorta ascendens and the main pulmonary artery was noted (figure 3). The ostial interarterial segment appeared narrowed on CCTA (figure 4). For

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Fig. 1 Patient 1: three-dimensional reconstruction of CCTA-images shows the right coronary artery (RCA) arising from the left sinus of Valsalva. Note the interarterial course between the aorta (AO) and pulmonary aorta (PA) and the normal origin of the left coronary artery (LCA).

Fig. 3 Patient 2: three-dimensional reconstruction of CCTA-images shows the right coronary artery (RCA) arising from the left sinus of Valsalva. Note the interarterial course between the aorta (AO) and pulmonary aorta (PA) and the normal origin of the left coronary artery (LCA). Superior view.

Fig. 2 Patient 1: axial CCTA-image which shows the aberrant RCA arising from the opposite sinus with an interarterial course between the aorta (AO) and pulmonary artery (PA).

Fig. 4 Patient 2: axial CCTA-image which shows the aberrant RCA arising from the opposite sinus with an interarterial course between the aorta (AO) and pulmonary artery (PA). Note the normal origin of the left coronary artery (LCA) and the impression of narrowing of the ostial segment of the RCA.

this reason a coronary angiogram was performed which confirmed the aberrant RCA. No significant atherosclerosis was observed. Compression of the interarterial course of the aberrant RCA was not noted at catheterization.

The patient was reassured as his chest pain was not attributed to a cardiac origin. This patient was treated medically with aspirin and a statin. No complications or recurrence occurred after three years of follow-up.

Treatment of patients with an anomalous origin of the RCA

DISCUSSION An aberrant origin of a coronary artery occurs in approximately 0.84 to 1.2% among patients referred for CCTA and coronary angiography1,2. An aberrant origin of a coronary artery is not strictly related to other congenital abnormalities3. The incidence of an anomalous origin of the right coronary artery has been reported up to 0.92% of the general population and is more frequent than an anomalous origin of the left coronary artery (LCA)2,3. The incidence of an aberrant RCA seems to differ among the races with higher incidences in Japan3. Diagnosis of an aberrant coronary artery is easily made by CCTA4. CCTA allows adequate 3-dimensional visualization and provides reliable information concerning the orifice location and the precise course of the anomalous coronary artery. Angiographic evaluation can be difficult as selective cannulation of the anomalous coronary artery is not always possible due to a small slit-like orifice. Some authors suggest intravascular ultrasound (IVUS) for obtaining cross-sectional luminal images and refining optimal treatment planning1. The two patients described in this case report were diagnosed with an aberrant coronary artery by CCTA (iCT scan Philips Brilliance 256 slice). The images obtained by reconstruction clearly showed the aberrant origin and the interarterial course. Congenital coronary anomalies are mostly asymptomatic and are detected incidentally. If a coronary anomaly gives rise to complaints, symptoms occur most frequently before the age of 30 years and may have a strong correlation with exercise5. Clinical manifestations may vary from angina, acute myocardial infarction, syncope, heart failure or sudden cardiac death (SCD). Cardiac arrest is a wellknown life-threatening complication in patients with congenital coronary anomalies. Retrospective cohort studies report 19-33% of sudden cardiac death in the young population to be attributable to coronary artery anomalies3. It seems that SCD occurs more frequently in patients with an anomalous LCA than in those with an aberrant RCA6. An interarterial course of an aberrant coronary artery may be a risk factor for SCD, due to a compromised coronary circulation5. IVUS studies demonstrated compression of the interarterial segment by the aorta, especially during exercise. In the absence of an interarterial course the coronary blood flow may become compromised due to a narrowed orifice or due to an acute angle take-off of the aberrant coronary artery. These two mechanisms are mostly observed in anomalous origins of the RCA2. Alternatively, a compression of an intramural segment by the aortic valve has also been described5. As an anomalous origin of a coronary artery is a rare congenital disease no large randomized controlled trials exist to evaluate proper treatment options. The ACC/ AHA guidelines of 2008 recommend treatment of an aberrant coronary artery in the following conditions4. As

SCD occurs frequently in patients with an anomalous LCA with interarterial course, these patients should be proposed for surgical repair (class 1, level B evidence). An aberrant origin of the RCA with an interarterial course is still associated with a risk of SCD, and surgical treatment is proposed in the case of documented cardiac ischaemia (class 1, level B evidence). One surgical option is re-implantation of the anomalous coronary artery on the aortic root, although this procedure carries the risk of stenosis of the neo-ostial orifice. In the presence of an intramural segment the unroofing technique, in which the intramural segment of the coronary artery is dissected and reimplanted on the correct sinus of Valsalva, yields good results. Coronary artery bypass grafting (CABG) is technically feasible, although this approach has been recently described as less favourable7. The advantage of CABG consists of avoiding opening of the aortic root, but carries the risk of competive blood flow of the native aberrant coronary artery thereby compromising the long-term bypass patency. Stent-angioplasty in the narrowed or obstructed segment of an aberrant RCA has been recently reported to be successfully performed in selected patients8. When the patient has an anomalous right coronary artery and no evidence of cardiac ischaemia is established, management remains controversial4. A conservative approach with initiation of beta blockers as presented in our cases may be reasonable in such cases. In addition, detection of coronary anomalies of wrong sinus origin in which a coronary artery passes between the great arteries should result in exclusion from participation in competitive sports9. To further determine the optimal treatment of adult patients or children with rare congenital coronary anomalies further comprehensive studies are needed. In this regard, the Registry of Anomalous Aortic Origin of the Coronary Artery has been established by the Congenital Heart Surgeons’ Society10.

CONCLUSION An aberrant origin of the RCA with an interarterial course between the aorta and pulmonary trunk is a rather rare congenital disorder. We presented two cases of middle-aged patients in whom an aberrant RCA with interarterial course was detected in the workup for chest pain. As no cardiac ischaemia was documented, both patients were treated medically. Further studies to address the therapeutic regimens in such patients are needed for tailored patient management.

CONFLICT OF INTEREST The authors have no conflicts of interest to declare.

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Aberrant right coronary artery arising from the left sinus of Valsalva with an interarterial course.

An anomalous origin of the right coronary artery from the left sinus of Valsalva with an interarterial course is a rather rare congenital anomaly. Tre...
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