CLINICAL SPOTLIGHT
Heart, Lung and Circulation (2015) 24, e123–e126 1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2015.03.003
Percutaneous Coronary Intervention of an Anomalous Left Main Coronary Artery Arising from the Right Sinus of Valsalva Nadim Shah, FRACP *, Victoria E. Cheng, MBBS, Nicholas Cox, FRACP, Kean Soon, FRACP Western Health, Melbourne, Vic., Australia Received 19 January 2015; accepted 3 March 2015; online published-ahead-of-print 24 March 2015
Anomalous origination of a coronary artery from the opposite sinus of Valsalva is an uncommon congenital anomaly. Intervention for concurrent coronary artery disease is challenging due to the location of the ostia, the takeoff of the vessel as well as the course of the artery in question. It is also important, where possible, to exclude a ‘‘malignant’’ course as the most common adverse outcome from this anomaly is that of sudden cardiac death. Here we present a case of percutaneous coronary intervention in a patient with anomalous left main origination from the right coronary sinus of Valsalva and a brief discussion on the subject. Keywords
Percutaneous coronary intervention Coronary heart disease Coronary artery disease Anomalous coronary artery Coronary CT angiography
Case Presentation A 57 year-old male, with a history of depression and bipolar disorder, presented with history of central heavy chest pain lasting 20 minutes. He was a current smoker and was taking lithium, olanzapine and zopiclone. No other risk factors for coronary artery disease (CAD) were identified. Physical examination was unremarkable. Septal Q waves were demonstrated on an electrocardiogram and serum troponin I peaked at 2.98 mcg/L (ULN 0.05 mcg/L) within 24 hours of presentation. He was managed as per acute coronary syndrome guidelines. Coronary angiography via the right radial approach revealed an anomalous origin of the left main coronary artery (LMCA) from the right sinus of Valsalva with a distal vessel 90% angiographic stenosis [Fig. 1-2]. The right coronary artery (RCA) was dominant and free of severe angiographic stenosis. Given the critical nature of the stenosis ad hoc percutaneous coronary intervention (PCI) to the LMCA was contemplated, however there were concerns of a
‘‘malignant’’ course anomaly (i.e. with an interarterial course between the pulmonary artery and ascending aorta) where surgical revascularisation would be more appropriate. A Swan-Ganz (SG) catheter was placed in the pulmonary artery (PA) in an attempt to define the course of the anomalous coronary artery as suggested by the literature [1]. The SG catheter and anomalous coronary crossed in all projections suggesting the course was likely to be anterior to the pulmonary artery [Fig. 3]. However we were not sufficiently confident of this to proceed without CT imaging which has been shown to be a more accurate way of defining the anatomy [2]. Hence coronary CT angiography (CCTA) was performed that demonstrated a non-malignant course of the LMCA [Fig. 4-5] and therefore he was referred for percutaneous revascularisation. Percutaneous coronary intervention to the LMCA was undertaken via the right femoral approach for better guide support. Various 6 French guiding catheters were tried including Judkins Right 4, Amplatz 1.0, Hockey Stick, Multipurpose and XB 3.0. All of these catheters failed to provide
*Corresponding author at: Department of Cardiology, Western Hospital, Footscray, 3011, Victoria, Australia. Tel.: +61 414 636 080; fax: +61 3 8345 7357, Email: [email protected] © 2015 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier Inc. All rights reserved.
e124
Figure 1 Anteroposterior cranial projection demonstrating anomalous left main coronary artery originating from the right sinus of Valsalva with a distal vessel severe stenosis (black arrow). There is non selective opacification of the right coronary artery (white arrow).
N. Shah et al.
Figure 3 Anteroposterior projection demonstrating crossing of Swan-Ganz catheter (white arrow) and anomalous left main coronary artery (black arrow).
sufficient support for stenting of the lesion. Surprisingly, the best guide support appeared to come from a Judkins Left 3.5. The stenosis was predilated with a Sprinter balloon and stented with a Xience Prime 3.0 x 15 mm drug eluting stent. He made an uncomplicated recovery and was discharged two days post procedure.
Figure 2 Right anterior oblique projection demonstrating anomalous left main coronary artery originating from the right sinus of Valsalva with a distal vessel severe stenosis (black arrow).
Figure 4 3D reconstruction of coronary CT angiography clearly demonstrating anomalous left main coronary artery with a distal vessel severe stenosis (black arrows). The course of the anomalous artery is anterior to the pulmonary artery (PA) and not between the PA and aorta (Ao). i.e. it is a benign form of anomalous coronary artery. The right coronary artery can also be seen (white arrow).
e125
PCI of anomalous coronary artery
Figure 5 A curve multiplanar reformat (MPR) image of Coronary CT angiography demonstrating anomalous left main coronary artery with a distal vessel severe stenosis (white arrow). The aorta (Ao) and pulmonary artery (PA) are also seen.
Discussion Anomalous origination of a coronary artery from the opposite sinus of Valsalva (ACAOS) can be found in up to 1% of all coronary angiographies undertaken [3]. It is six times more common for the RCA to originate from the left sinus of Valsalva than vice versa [3]. ACAOS is estimated to account for 33% of sudden cardiac death in military recruits [4] in the United States of America and 13% of athletes [5]. In autopsies of athletes who have succumbed to sudden cardiac death, presumably due to ACAOS, the affected coronary artery is found to have an interarterial or ‘‘malignant’’ course between
the aorta and the PA in 80% of cases [5] and only this particular anatomy is associated with severe prognosis [3]. Strenuous physical activity is recognised to be a particular risk factor for sudden death [5,6] which may explain why it is described in military recruits and athletes. Sudden death appears to be a particular concern in the young and the risk appears to reduce beyond the age of 30 years [7]. The American Heart Association (AHA) suggests identification of possible ACAOS for evaluation of aborted sudden cardiac death or life threatening arrhythmias with a class I recommendation [8]. ACAOS, however, is usually identified incidentally as most individuals are asymptomatic and it is commonly recognised during coronary angiography performed for atypical chest pain [3]. CCTA or magnetic resonance angiography is recommended by the AHA for initial evaluation of suspected ACAOS [8] but a focussed transthoracic echocardiogram is also described in the literature as the initial imaging modality of choice [9,10]. Invasive coronary angiography is usually required but is poor at excluding ‘‘malignant’’ course and CCTA is well described as more suitable for this purpose [2]. SG catheter had been used in the past but it does not exclude an interarterial course of ACAOS convincingly, as in this case. Avoidance of strenuous physical activity is recommended for reduction in the risk of sudden cardiac death but beta blockade use is described as a surrogate [3]. Surgical coronary revascularisation has a class I recommendation by the AHA for anomalous origination of the LMCA with a ‘‘malignant’’ course and also the RCA, if ischaemia is demonstrated [8]. This usually involves direct reimplantation of the ectopic artery, unroofing of intramural segment or osteoplasty [3]. There is paucity of data on PCI and it is limited to case reports or series (Table 1). PCI in ACAOS can be particularly challenging due to the course of the artery, by the angulation and takeoff of the ostia as in this case. It is also confounded by the fact, coronary guiding catheters used to perform PCI are specifically designed for normally originating coronary arteries and do not necessarily adjust for anomalous anatomy.
Table 1 Case reports describing percutaneous coronary intervention to anomalous left coronary arteries arising from the right coronary sinus. (LAD, left anterior descending artery; RCA, right coronary artery; LCx, left circumflex artery; LV, left ventricle; AV, atrioventricular; STEMI, ST elevation myocardial infarction; PCI, percutaneous coronary intervention.). Case
PCI vessel
Course
Access
Guide
Presentation
Survival to
Author
discharge 75M
LAD
Not noted
Femoral
JR4
STEMI – delayed PCI
Yes
Hershey J et. al.
81F
LAD and RCA
Interarterial
Femoral
JR4 & Hockey stick
Unstable angina
Yes
Kang et. al.
42F 48M
LMCA RCA
Interarterial LV free wall
Femoral Femoral
AL1 JR4
Positive sestamibi STEMI - delayed PCI
Yes Yes
Schrale et. al. Zhou et. al.
59M
LAD
Retro-aortic
Femoral
ECR 4
Stable angina
Yes
Vijayvergiya et. al.
75M
LAD/RCA/LCx
AV groove
Femoral
AR2
Stable angina
Yes
Gupta et. al.
54M
LMCA
Retro-aortic
Femoral
RJ - OKA Mach 1
Unstable angina
Yes
Routoulas et. al.
62M
LCx
Retro-aortic
Radial
JR4
STEMI
Yes
Constantinides et. al.
e126
Occasionally the guiding catheter of choice can be quite the opposite of the routine guiding catheter as in this case.
Summary This case demonstrates the complexity of approaching PCI to anomalous origination of a coronary artery from the opposite sinus of Valsalva with concomitant CAD. PCI should only be undertaken with prior planning to exclude a ‘‘malignant’’ course of the artery as well as to ensure adequate guide support.
Conflict of Interest The authors report no relationships that could be construed as a conflict of interest.
References [1] Shirani J, Brofferio A, WP W, Talavera F, Compton SJ, Suleman A, et al. Isolated Coronary Artery Anomalies. Medscape. 2014, http://emedicine. medscape.com/article/153512-overview. [2] Soon KH, Chaitowitz I, Selvanayagam JB, Kelly AM, Zakhem B, Nguyen M, et al. Comparison of fluoroscopic coronary angiography and multi-slice coronary angiography in the characterization of anomalous coronary artery. International Journal of Cardiology 2008;130:96–8.
N. Shah et al.
[3] Angelini P. Coronary artery anomalies: an entity in search of an identity. Circulation 2007;115:1296–305. [4] Eckart RE, Scoville SL, Campbell CL, Shry EA, Stajduhar KC, Potter RN, et al. Sudden death in young adults: a 25-year review of autopsies in military recruits. Annals of Internal Medicine 2004;141:829–34. [5] Maron BJ, Shirani J, Poliac LC, Mathenge R, Roberts WC, Mueller FO. Sudden death in young competitive athletes. Clinical, demographic, and pathological profiles. JAMA: the Journal of the American Medical Association 1996;276:199–204. [6] Drory Y, Turetz Y, Hiss Y, Lev B, Fisman EZ, Pines A, et al. Sudden unexpected death in persons less than 40 years of age. The American Journal of Cardiology 1991;68:1388–92. [7] Taylor AJ, Byers JP, Cheitlin MD, Virmani R. Anomalous right or left coronary artery from the contralateral coronary sinus: ‘‘high-risk’’ abnormalities in the initial coronary artery course and heterogeneous clinical outcomes. American Heart Journal 1997;133:428–35. [8] Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Journal of the American College of Cardiology 2008;52:e143–263. [9] Ropers D, Moshage W, Daniel WG, Jessl J, Gottwik M, Achenbach S. Visualization of coronary artery anomalies and their anatomic course by contrast-enhanced electron beam tomography and three-dimensional reconstruction. The American Journal of Cardiology 2001;87:193–7. [10] Post JC, van Rossum AC, Bronzwaer JG, de Cock CC, Hofman MB, Valk J, et al. Magnetic resonance angiography of anomalous coronary arteries. A new gold standard for delineating the proximal course? Circulation 1995;92:3163–71.
Heart, Lung and Circulation (2015) 24, e123–e126 1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2015.03.003
Percutaneous Coronary Intervention of an Anomalous Left Main Coronary Artery Arising from the Right Sinus of Valsalva Nadim Shah, FRACP *, Victoria E. Cheng, MBBS, Nicholas Cox, FRACP, Kean Soon, FRACP Western Health, Melbourne, Vic., Australia Received 19 January 2015; accepted 3 March 2015; online published-ahead-of-print 24 March 2015
Anomalous origination of a coronary artery from the opposite sinus of Valsalva is an uncommon congenital anomaly. Intervention for concurrent coronary artery disease is challenging due to the location of the ostia, the takeoff of the vessel as well as the course of the artery in question. It is also important, where possible, to exclude a ‘‘malignant’’ course as the most common adverse outcome from this anomaly is that of sudden cardiac death. Here we present a case of percutaneous coronary intervention in a patient with anomalous left main origination from the right coronary sinus of Valsalva and a brief discussion on the subject. Keywords
Percutaneous coronary intervention Coronary heart disease Coronary artery disease Anomalous coronary artery Coronary CT angiography
Case Presentation A 57 year-old male, with a history of depression and bipolar disorder, presented with history of central heavy chest pain lasting 20 minutes. He was a current smoker and was taking lithium, olanzapine and zopiclone. No other risk factors for coronary artery disease (CAD) were identified. Physical examination was unremarkable. Septal Q waves were demonstrated on an electrocardiogram and serum troponin I peaked at 2.98 mcg/L (ULN 0.05 mcg/L) within 24 hours of presentation. He was managed as per acute coronary syndrome guidelines. Coronary angiography via the right radial approach revealed an anomalous origin of the left main coronary artery (LMCA) from the right sinus of Valsalva with a distal vessel 90% angiographic stenosis [Fig. 1-2]. The right coronary artery (RCA) was dominant and free of severe angiographic stenosis. Given the critical nature of the stenosis ad hoc percutaneous coronary intervention (PCI) to the LMCA was contemplated, however there were concerns of a
‘‘malignant’’ course anomaly (i.e. with an interarterial course between the pulmonary artery and ascending aorta) where surgical revascularisation would be more appropriate. A Swan-Ganz (SG) catheter was placed in the pulmonary artery (PA) in an attempt to define the course of the anomalous coronary artery as suggested by the literature [1]. The SG catheter and anomalous coronary crossed in all projections suggesting the course was likely to be anterior to the pulmonary artery [Fig. 3]. However we were not sufficiently confident of this to proceed without CT imaging which has been shown to be a more accurate way of defining the anatomy [2]. Hence coronary CT angiography (CCTA) was performed that demonstrated a non-malignant course of the LMCA [Fig. 4-5] and therefore he was referred for percutaneous revascularisation. Percutaneous coronary intervention to the LMCA was undertaken via the right femoral approach for better guide support. Various 6 French guiding catheters were tried including Judkins Right 4, Amplatz 1.0, Hockey Stick, Multipurpose and XB 3.0. All of these catheters failed to provide
*Corresponding author at: Department of Cardiology, Western Hospital, Footscray, 3011, Victoria, Australia. Tel.: +61 414 636 080; fax: +61 3 8345 7357, Email: [email protected] © 2015 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier Inc. All rights reserved.
e124
Figure 1 Anteroposterior cranial projection demonstrating anomalous left main coronary artery originating from the right sinus of Valsalva with a distal vessel severe stenosis (black arrow). There is non selective opacification of the right coronary artery (white arrow).
N. Shah et al.
Figure 3 Anteroposterior projection demonstrating crossing of Swan-Ganz catheter (white arrow) and anomalous left main coronary artery (black arrow).
sufficient support for stenting of the lesion. Surprisingly, the best guide support appeared to come from a Judkins Left 3.5. The stenosis was predilated with a Sprinter balloon and stented with a Xience Prime 3.0 x 15 mm drug eluting stent. He made an uncomplicated recovery and was discharged two days post procedure.
Figure 2 Right anterior oblique projection demonstrating anomalous left main coronary artery originating from the right sinus of Valsalva with a distal vessel severe stenosis (black arrow).
Figure 4 3D reconstruction of coronary CT angiography clearly demonstrating anomalous left main coronary artery with a distal vessel severe stenosis (black arrows). The course of the anomalous artery is anterior to the pulmonary artery (PA) and not between the PA and aorta (Ao). i.e. it is a benign form of anomalous coronary artery. The right coronary artery can also be seen (white arrow).
e125
PCI of anomalous coronary artery
Figure 5 A curve multiplanar reformat (MPR) image of Coronary CT angiography demonstrating anomalous left main coronary artery with a distal vessel severe stenosis (white arrow). The aorta (Ao) and pulmonary artery (PA) are also seen.
Discussion Anomalous origination of a coronary artery from the opposite sinus of Valsalva (ACAOS) can be found in up to 1% of all coronary angiographies undertaken [3]. It is six times more common for the RCA to originate from the left sinus of Valsalva than vice versa [3]. ACAOS is estimated to account for 33% of sudden cardiac death in military recruits [4] in the United States of America and 13% of athletes [5]. In autopsies of athletes who have succumbed to sudden cardiac death, presumably due to ACAOS, the affected coronary artery is found to have an interarterial or ‘‘malignant’’ course between
the aorta and the PA in 80% of cases [5] and only this particular anatomy is associated with severe prognosis [3]. Strenuous physical activity is recognised to be a particular risk factor for sudden death [5,6] which may explain why it is described in military recruits and athletes. Sudden death appears to be a particular concern in the young and the risk appears to reduce beyond the age of 30 years [7]. The American Heart Association (AHA) suggests identification of possible ACAOS for evaluation of aborted sudden cardiac death or life threatening arrhythmias with a class I recommendation [8]. ACAOS, however, is usually identified incidentally as most individuals are asymptomatic and it is commonly recognised during coronary angiography performed for atypical chest pain [3]. CCTA or magnetic resonance angiography is recommended by the AHA for initial evaluation of suspected ACAOS [8] but a focussed transthoracic echocardiogram is also described in the literature as the initial imaging modality of choice [9,10]. Invasive coronary angiography is usually required but is poor at excluding ‘‘malignant’’ course and CCTA is well described as more suitable for this purpose [2]. SG catheter had been used in the past but it does not exclude an interarterial course of ACAOS convincingly, as in this case. Avoidance of strenuous physical activity is recommended for reduction in the risk of sudden cardiac death but beta blockade use is described as a surrogate [3]. Surgical coronary revascularisation has a class I recommendation by the AHA for anomalous origination of the LMCA with a ‘‘malignant’’ course and also the RCA, if ischaemia is demonstrated [8]. This usually involves direct reimplantation of the ectopic artery, unroofing of intramural segment or osteoplasty [3]. There is paucity of data on PCI and it is limited to case reports or series (Table 1). PCI in ACAOS can be particularly challenging due to the course of the artery, by the angulation and takeoff of the ostia as in this case. It is also confounded by the fact, coronary guiding catheters used to perform PCI are specifically designed for normally originating coronary arteries and do not necessarily adjust for anomalous anatomy.
Table 1 Case reports describing percutaneous coronary intervention to anomalous left coronary arteries arising from the right coronary sinus. (LAD, left anterior descending artery; RCA, right coronary artery; LCx, left circumflex artery; LV, left ventricle; AV, atrioventricular; STEMI, ST elevation myocardial infarction; PCI, percutaneous coronary intervention.). Case
PCI vessel
Course
Access
Guide
Presentation
Survival to
Author
discharge 75M
LAD
Not noted
Femoral
JR4
STEMI – delayed PCI
Yes
Hershey J et. al.
81F
LAD and RCA
Interarterial
Femoral
JR4 & Hockey stick
Unstable angina
Yes
Kang et. al.
42F 48M
LMCA RCA
Interarterial LV free wall
Femoral Femoral
AL1 JR4
Positive sestamibi STEMI - delayed PCI
Yes Yes
Schrale et. al. Zhou et. al.
59M
LAD
Retro-aortic
Femoral
ECR 4
Stable angina
Yes
Vijayvergiya et. al.
75M
LAD/RCA/LCx
AV groove
Femoral
AR2
Stable angina
Yes
Gupta et. al.
54M
LMCA
Retro-aortic
Femoral
RJ - OKA Mach 1
Unstable angina
Yes
Routoulas et. al.
62M
LCx
Retro-aortic
Radial
JR4
STEMI
Yes
Constantinides et. al.
e126
Occasionally the guiding catheter of choice can be quite the opposite of the routine guiding catheter as in this case.
Summary This case demonstrates the complexity of approaching PCI to anomalous origination of a coronary artery from the opposite sinus of Valsalva with concomitant CAD. PCI should only be undertaken with prior planning to exclude a ‘‘malignant’’ course of the artery as well as to ensure adequate guide support.
Conflict of Interest The authors report no relationships that could be construed as a conflict of interest.
References [1] Shirani J, Brofferio A, WP W, Talavera F, Compton SJ, Suleman A, et al. Isolated Coronary Artery Anomalies. Medscape. 2014, http://emedicine. medscape.com/article/153512-overview. [2] Soon KH, Chaitowitz I, Selvanayagam JB, Kelly AM, Zakhem B, Nguyen M, et al. Comparison of fluoroscopic coronary angiography and multi-slice coronary angiography in the characterization of anomalous coronary artery. International Journal of Cardiology 2008;130:96–8.
N. Shah et al.
[3] Angelini P. Coronary artery anomalies: an entity in search of an identity. Circulation 2007;115:1296–305. [4] Eckart RE, Scoville SL, Campbell CL, Shry EA, Stajduhar KC, Potter RN, et al. Sudden death in young adults: a 25-year review of autopsies in military recruits. Annals of Internal Medicine 2004;141:829–34. [5] Maron BJ, Shirani J, Poliac LC, Mathenge R, Roberts WC, Mueller FO. Sudden death in young competitive athletes. Clinical, demographic, and pathological profiles. JAMA: the Journal of the American Medical Association 1996;276:199–204. [6] Drory Y, Turetz Y, Hiss Y, Lev B, Fisman EZ, Pines A, et al. Sudden unexpected death in persons less than 40 years of age. The American Journal of Cardiology 1991;68:1388–92. [7] Taylor AJ, Byers JP, Cheitlin MD, Virmani R. Anomalous right or left coronary artery from the contralateral coronary sinus: ‘‘high-risk’’ abnormalities in the initial coronary artery course and heterogeneous clinical outcomes. American Heart Journal 1997;133:428–35. [8] Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Journal of the American College of Cardiology 2008;52:e143–263. [9] Ropers D, Moshage W, Daniel WG, Jessl J, Gottwik M, Achenbach S. Visualization of coronary artery anomalies and their anatomic course by contrast-enhanced electron beam tomography and three-dimensional reconstruction. The American Journal of Cardiology 2001;87:193–7. [10] Post JC, van Rossum AC, Bronzwaer JG, de Cock CC, Hofman MB, Valk J, et al. Magnetic resonance angiography of anomalous coronary arteries. A new gold standard for delineating the proximal course? Circulation 1995;92:3163–71.
