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ase Report
rigin of all three coronaries separately from right sinus of valsalva – A rarenomaly
urender Deora (MD, DM) ∗, Sanjay Shah (MD, DM), Tejas Patel (DM)epartment of Cardiovascular Sciences, Sheth V.S. General Hospital, Smt. N.H.L. Municipal Medical College, Gujarat University, Ahmedabad, Gujarat 380006, India
r t i c l e i n f o
rticle history:eceived 25 June 2012eceived in revised form 31 August 2012ccepted 10 September 2012
a b s t r a c t
Anomalous coronary artery origin from opposite sinus is uncommon and separate origin of all threecoronary arteries from right sinus of valsalva is exceptionally rare. The anomaly may cause ischemia dueto atherosclerosis or due to altered ostial configuration, its exit angulations from the aorta, route of theartery, and intussuception. Various imaging modalities such as echocardiography, coronary angiography,
eywords:oronary anomalyight coronary sinus
schemia
computed tomography, and magnetic resonance imaging have been used to diagnose origin and courseof anomalous coronary arteries. Management includes medical treatment and percutaneous or surgicalrevascularization. Percutaneous coronary intervention is technically challenging and needs judiciousselection of guide catheters. We report a patient who presented with inferior wall myocardial infarctionand separate origin of all three coronaries from right sinus of valsalva with successful percutaneousrevascularization.
Anomalous coronary artery origin from opposite sinus is a rareoronary anomaly and may result in myocardial ischemia andudden cardiac death. Left anterior descending (LAD) and left cir-umflex (LCx) coronary artery originating separately from rightinus of valsalva is exceptionally rare and very few cases have beeneported in the literature. Here, we report an interesting case ofeparate origin of all three coronaries from right coronary sinusresenting with inferior wall myocardial infarction and discuss thearious causes of angina and technical difficulties in percutaneousoronary intervention.
ase history
A 46-year-old male smoker, dyslipidemic, nondiabetic, nor-otensive was admitted to the emergency department with
etrosternal chest pain radiating to left arm of 6 h duration. 12-leadlectrocardiogram revealed ST segment elevation in II, III, aVF, RV3,nd RV4. The patient was diagnosed as a case of acute coronary syn-rome – inferior wall and right ventricle myocardial infarction andas thrombolyzed with 1.5 MU streptokinase. On day 2 of admis-
ion, due to ongoing chest pain, coronary angiography was donehrough right femoral artery. Selective coronary artery cannula-ion was attempted with 5F JL 3.5 diagnostic catheter; however, no
anese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
coronary artery was seen arising from left coronary cusp. Rightcoronary artery cannulation with 5F JR3.5 diagnostic catheterrevealed all three coronaries arising separately from right coronarycusp (Fig. 1 and S Video 1). Right coronary artery (RCA) revealedsignificant lesion in mid segment. Percutaneous coronary interven-tion (PCI) to RCA was done with drug eluting stent. Initially, RCAwas cannulated with 6F JR 3.5 but due to poor back up support 6FAL 2 was taken to complete the procedure. Computed tomographycoronary angiography confirmed the anomalous separate origin ofLAD and LCx from right coronary sinus (Fig. 2) and delineated thecourse of LAD which traversed between aorta and pulmonary artery(Fig. 3). LCx was retroaortic in its course. The patient was dischargedwithout any complication and was asymptomatic at one monthfollow up.
Discussion
Coronary artery anomalies are rare with an incidence varyingfrom 0.3% in autopsy series to 1.3% in angiographic series [1,2] andmay be seen either as an isolated anomaly or in association withother congenital cardiac defects. The most common form of coro-nary anomaly is anomalous origin of LCX from either right coronarysinus or proximal RCA. Left main coronary artery from right sinusof valsalva is a well-recognized coronary anomaly and is associatedwith sudden cardiac death, but separate origin of both LAD and LCx
from opposite sinus is very rare. The incidence of such anomaly isreported to be 3.1% in a coronary angiographic and magnetic res-onance imaging study [3]. Engel et al. [4] reported four cases andClick et al. [5] reported three cases in their studies.
ig. 1. Coronary angiogram in left anterior oblique view revealing separate origin ofll three coronaries from right coronary sinus. LAD, left anterior descending artery;CA, right coronary artery; LCX, left circumflex artery.
Various imaging modalities such as echocardiography, coro-ary angiography, computed tomography, and magnetic resonance
maging have been used to diagnose origin and course of anoma-ous coronary arteries. The most common course of the proximalnomalous LAD is anterior to pulmonary trunk and anomalous LCXosterior to aorta [5]. But in our case, LAD after anomalous ori-in traversed between the great vessels. The patients with thesenomalous coronaries may have ischemia due to atheroscleroticisease or due to abnormal acute angle of takeoff, slit-like or nar-owed ostium, intramural course, or compression between the
ortic root and pulmonary trunk. Intravascular ultrasound studieslso revealed intussuception with coronary segmental hypoplasiand lateral luminal compression as an important pathophysiolo-ical mechanism of ischemia [6]. An analysis from the CASS study
ig. 2. Reconstructed computed tomography coronary angiography showing sep-rate origin of all three coronaries from right coronary sinus. Ao, aorta; LAD, leftnterior descending artery; RCA, right coronary artery; LCX, left circumflex artery.
graphic coronary angiography in left saggital view showing the course of left anteriordescending artery (LAD) between pulmonary artery (PA) and aorta.
showed that anomalous coronary arteries are not at increased riskfor atherosclerosis as compared to non anomalous arteries in age-and gender-matched control patients except for the anomalous cir-cumflex coronary artery [7]. Increased risk for atherosclerosis inanomalous LCx may be due to increased stress on the coronaryarterial wall by the expanding aorta [8].
The management of patients with anomalous coronariesincludes medical management, PCI, or surgical repair. Asymp-tomatic patients with interarterial course of the LAD should becarefully evaluated for signs of ischemia and should be advised toavoid strenuous activities. PCI in these anomalous coronary arteriesis technically challenging because of altered ostial configuration,its exit angulations from the aorta, route of the artery, and thelocation of the atherosclerotic lesion. The guide catheter must beadapted both to the angle of origin from the aorta and to the widthof the ascending aorta. For RCA with a horizontal take-off, standardJudkins right is generally the best choice whereas with an inferiortake-off Judkins right is the first choice and if the ascending aortais dilated multipurpose curve is better. For the RCA with a supe-rior orientation of the ostium (Shepard’s Crook RCA), left Amplatzconfiguration is preferred. When additional back-up is required forcurved and calcified mid RCA lesions either deep engagement ofthe guide catheter or an Amplatz left 1 or 2 is extremely helpfulwhether it is transradial as in our case or transfemoral approach. PCIto RCA which originates from the left coronary sinus the preferredguide catheters are large Judkins left, Amplatz left, or multipur-pose [9]. PCI of the anomalous circumflex artery arising separatelyfrom right coronary sinus and retroaortic in course Amplatz left 1,Amplatz right 1, or Judkins right 4 may be helpful. In cases with thecircumflex arising from the RCA, JR 4 is the preferred guide catheter.Similarly for the LAD it depends on orifice configuration whichdecides the guide catheter. Therefore, the guide catheter selec-tion is important for successful PCI and needs to be changed as perthe lesion morphology where adequate support helps in crossingthe lesion with guidewires and balloon catheters and significantlyreduces the procedure time [10].
In conclusion, absence of left coronary artery origin from leftcoronary sinus should make suspicion of anomalous origin ofleft coronaries. All three coronary arteries arising separately from
right coronary sinus is an exceedingly rare congenital abnormal-ity. Patients with these anomalous coronary arteries may haveangina either due to atherosclerosis or due to abnormal anatomical
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actors. PCI is technically challenging and requires judicious selec-ion of guide catheters.
unding source
None.
onflict of interest
None.
ppendix A. Supplementary data
Supplementary data associated with this article can beound, in the online version, at http://dx.doi.org/10.1016/j.jccase.012.09.005.
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