Anomalies d'Origine Coronaire

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LETTER TO THE EDITOR

Anomalous origin of coronary arteries: When one sinus fits all

Antonios N. Pavlidis, George K. Karavolias, John S. Malakos, Eftihia Sbarouni, Panagiota Georgiadou

& Vasillis V. Voudris

Second Department of Cardiology, Onassis Cardiac Surgery Centre, Athens, Greece

lef anterior descending (LAD) coronary artery, while LM andlef circumex (LCX) coronary arteries were ree o obstructivedisease (Figure 1A). Right coronary artery (RCA) was ectopic,arising rom the lef sinus o Valsalva (LSOV), and was ree osignicant atherosclerotic disease (Figure 1B, Supplementary

Movie 1 to be ound online at http://www.inormahealthcare.com/abs/doi/10.3109/17482941.2012.683797). Further evalua-tion o the coronary anatomy with multislice computed tomog-raphy conrmed the anomalous origin o the RCA whicharoused rom the LSOV and coursed interarterially betweenthe pulmonary trunk and the aortic root into the right atrio-

ventricular groove (Figure 2). Photon emission myocardialperusion scan was negative. Patient was treated conservativelyand was discharged the ollowing day.

Patient 2

A 60-year-old man was reerred or coronary angiographyand possible revascularization therapy afer sustaining a

non-S elevation acute coronary syndrome. He was a heavysmoker and was under treatment or hypertension andhyperlipidemia. Echocardiography depicted lef ventricularhypertrophy with preserved lef ventricular ejection raction.Coronary angiography was perormed via the emoralapproach and multiple attempts with different diagnosticcatheters ailed to engage the lef coronary ostium. Cannula-tion o the right coronary system was achieved with an 6FAmplatz lef 1 (AL1) diagnostic catheter (Cordis) and con-trast injection demonstrated that the RCA and LM origi-nated rom two separate ostia within the right sinus oValsalva (RSOV) (Figure 3A). Afer a long course, the LMgave rise to the LCX and LAD (Supplementary Movie 2 to beound online at http://www.inormahealthcare.com/abs/doi/10.3109/17482941.2012.683797). A tight lesion was detectedin the distal part o the RCA, while the other coronary arter-ies were ree o signicant narrowing.

Te RCA was selectively cannulated with a 6F AL1 guid-ing catheter (Cordis) with side holes and the stenotic lesionwas successully crossed with a 0.014 inch BMW wire (AbbottVascular) and dilated with a 2.5 10 mm Falcon balloon

Correspondence: Antonios N. Pavlidis, 26 Phoenix Lodge Mansions, London W6 7BG, UK. Fax: 30 210 6205330. E-mail: [email protected]

(Received 13 February 2012; accepted 2 April 2012)

Acute Cardiac Care,September 2012; 14(3): 99102

Copyright 2012 Informa UK, Ltd

ISSN 1748-2941 print/ISSN 1748-295X online

DOI: 10.3109/17482941.2012.683797

A right coronary artery origin from the left coronary sinus and a

left coronary origin from the right sinus although rarely encoun-

tered during routine cardiac catheterization, they represent two

relatively common autopsy ndings in young patients suffering

sudden cardiac death. The interarterial course of the aberrant

artery, between the aortic root and the pulmonary artery hasbeen considered as a malignant variant, because of the higher

risk of myocardial ischemia and sudden death. We present two

rare cases of ectopic coronary origin from the opposite sinus of

Valsalva.

Keywords: Ectopic coronary, congenital coronary anomalies,

sudden death

Introduction

Coronary artery anomalies (CAA) are rare congenital abnor-malities that are usually seen in patients with other coexis-tent congenital cardiac malormations, such as a bicuspidaortic valve or transposition o the great vessels. Althoughusually asymptomatic, CAA are considered as the secondmost common cause o sudden cardiac death in young ath-letes (1). Te origin o both coronary arteries rom a singlesinus o Valsalva is an extremely rare abnormality in whichthe interarterial course o the ectopic arteries betweenthe great vessels has been linked to a high incidence ocardiovascular events and sudden death.

Cases presentation

Patient 1A 63-year-old man with atypical chest pains was reerred orcoronary angiography ollowing a positive exercise treadmilltest. He had a history o treated hypertension and hyperlipi-demia. Coronary angiogram was perormed via the emoralapproach and the lef main stem (LM) was cannulated witha 6F Judkins lef 4 (JL4) diagnostic catheter (Cordis). Terewas a moderate narrowing in the middle segment o the


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(Invatec). Afer predilatation o the narrowed segment, a3.018 mm Nobori stent (erumo) was deployed at 16atm. A nal diagnostic angiogram showed an excellentangiographic result (Figure 3B). Patient made an uneventulrecovery and he was discharged the ollowing day on dualantiplatelet therapy.

Discussion

CAA are ofen asymptomatic and are usually encountered ascoincidental ndings during coronary angiography or atautopsy with an estimated incidence o 0.9% and 0.3%respectively (1). However, it is speculated that 2031% opatients with CAA experience lie threatening cardiovascu-

lar complications such as angina, myocardial inarction,arrhythmias, syncope and sudden death (2). CAA accountor up to one third o sudden cardiac deaths in the youngpopulation (3). Anomalies in the origin and distribution othe coronary arteries are responsible or 9095% o CAA,while coronary stulae or the rest o the cases (2,4). Separateostia o the LCX and LAD within the LSOV in the absenceo LM has been described as the most common anatomic

var iant, with an incidence o 0.6% (5). Rigatel li et al.proposed a classiication o CAA based on angiographicappearance and clinical signiicance: benign (class I);relevant-associated with ixed myocardial ischemia (classII); severe-related to sudden death (class III); and critical-associated with CAD (class IV) (6). Lipton et al. classiied

Figure 1. Coronary angiography in anteroposterior (A) and right anterior oblique (B) projections demonstrating ectopic origin o the RCA arisingrom the lef sinus o Valsalva. Tere is a moderate narrowing in the middle segment o the LAD (arrow). RCA, right coronary artery; LAD, lefanterior descending; LCX, lef circumflex.

Figure 2. Reconstructed multislice cardiac computed tomography showing the interarterial course o the RCA between the aorta and the pulmonarytrunk. RCA, right coronary artery; LAD, lef anterior descending; LCX, lef circumflex; PA, pulmonary artery; Ao, aorta.


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coronary artery variations according to the origin and ana-tomical course related to the ascending aorta and the pul-monary artery (7).

Anomalous origin o RCA rom the LSOV is a rarecongenital abnormality ound in 0.030.17% o adultpatients undergoing coronary angiography (1). he courseo the aberrant RCA can be retro-aortic, interarterial(between the aorta and the pulmonary artery) or anteriorto the pulmonary trunk. he interarterial or malignantsubtype is the most common orm and has been linkedto a higher incidence o myocardial inarction, suddendeath and exercise induced angina pectoris (8). Potentialmechanisms o myocardial ischemia in those patients

include compression o the artery between the aorta andthe pulmonary artery, ostial obstruction due to slit-likecoronary oriice, acute take-o angle o the RCA, coro-nary stretching or angulation with distention o theascending aorta or the pulmonary trunk (9). Slit-like ori-ice structure and acute angle take-o are seen more re-quently in patients who suer sudden cardiac death (9).Surgical decompression (unrooing procedure), PCI, re-implantation o the anomalous artery and coronary arterybypass grating (CABG) are all acceptable as potentialtherapeutic approaches (10).

Te incidence o an anomalous origin o the LM romthe RSOV among patients who undergo angiography has

been estimated between 0.008 and 0.017% (2). Te LMeither arises independently to the ostium o the RCA, orless requently the two arteries share a common ostium(12). Four different types o this extremely rare coronaryabnormality have been described based on the course o theectopic LM. ype A: Anterior to the right ventricular out-ow tract, ype B: Between the aorta and the pulmonarytrunk, ype C: Cristal, through the supraventricular crestand interventricular septum, ype D: Dorsal or posterior tothe aorta (5,12). Te anomalous origin o the LCA rom theRSOV is the most requent and has been consistently related

to myocardial inarction and sudden cardiac death. Teincidence is signicantly higher in patients with interarte-rial course o the LM (13). Although ew cases o suddendeath and myocardial ischemia associated with a posteriorcourse o the LM have been described, this type o anomalyis considered mostly benign (14). Anginal symptoms areusually related to exercise and are caused by compression othe proximal part o the LM between the expanded aorticroot and the pulmonary trunk. Terapeutic approach mustbe individualized according to symptoms, age and the anat-omy o the aberrant LM. Surgical correction is generallyindicated in young symptomatic patients who are at highrisk o sudden death.

Multidetector computed tomography scan (MDC), car-diac magnetic resonance (CMR) and transesophagealechocardiography (EE) are commonly used or the diagno-sis and imaging o the origin and course o CAA. However,coronary angiography remains the gold standard or thediagnosis. MDC can provide various multiplanar imagereconstructions and valuable anatomic inormation that areusually diffi cult to assess during angiography. Tree dimen-sion (3D) multiplane EE is a minimally invasive imagingmodality that can portray directly the proximal and interar-terial course o the LM (15). Cannulation o the ectopiccoronary arteries during angiography can be extremely chal-lenging and success depends mostly on physicians experi-

ence. Intravascular ultrasound (IVUS) has also been used inorder to obtain cross-sectional luminal images.

In conclusion, anomalous origins o coronary arteriesrom the opposite coronary sinus are extremely rare entitiesduring angiography. Although most patients are usuallyasymptomatic, certain types o these congenital anomalieshave been linked to myocardial ischemia and sudden car-diac death. Cannulation o ectopic coronaries can re-quently be problematic and time consuming; thereore,other imaging modalities, such as MDC may have a com-plementary role.

Figure 3. (A) Coronary angiography in lef anterior oblique projection demonstrating ectopic origin o the LM arising rom the right sinus oValsalva. Tere is a significant distal lesion o the RCA (arrow). (B) Selective cannulation o the RCA afer successul treatment o the culpritlesion (arrow).


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Declaration of interest: Te authors report no conicts ointerest. Te authors alone are responsible or the contentand writing o the paper.References

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Supplementary material available online

Movies 1 & 2

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Anomalies d'Origine Coronaire