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C A R D I O L O G Y F O U N D A T I O N . T H I S I S A N O P E N A C C E S S A R T I C L E U N D E R T H E

C C B Y - N C - N D L I C E N S E ( h t t p : / / c r e a t i v e c o mm o n s . o r g / l i c e n s e s / b y - n c - n d / 4 . 0 / ) .

CASE REPORT

CLINICAL CASE

Giant Calcified Left Circumflex CoronaryArtery Aneurysm With Complex Coronary-to–Left Ventricular Communication

Rabih Touma, MD,a Mohan Palla, MD,b Khurshaid Alam, MD,c James F. Mastromatteo, MD,a

Aiden Abidov, MD, PHDa,b

ABSTRACT

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A 64-year-old asymptomatic man had an incidental finding of a giant left circumflex artery (LCX) aneurysm, with the

distal LCX draining into a confluence receiving terminal portions of all coronary arteries and communicating with the left

ventricle through a transmural fistulous tract. We believe that this is the first case reported with such a complex LCX

abnormality. (Level of Difficulty: Beginner.) (J Am Coll Cardiol Case Rep 2020;-:-–-) Published by Elsevier on behalf

of the American College of Cardiology Foundation. This is an open access article under the CC BY-NC-ND license

(http://creativecommons.org/licenses/by-nc-nd/4.0/).

HISTORY OF PRESENTATION

A 64-year-old asymptomatic man underwent acomputed tomography (CT) scan of the abdomen andpelvis for evaluation of hematuria. The scan revealedan incidental finding of left circumflex artery (LCX)aneurysm. His physical examination was unremark-able for any acute or chronic cardiovascular findings.Upon admission, his vital signs were: blood pressure,136/89 mm Hg; pulse, 74 beats/min and regular;respiratory rate, 18 breaths/min; temperature, 98.1�F;and pulse oximetry, 97% on room air.

PAST MEDICAL HISTORY

The patient had history of well-controlled hyperten-sion and no other modifiable or nonmodifiable riskfactors or clinical markers of coronary artery disease.

N 2666-0849

m the aDivision of Cardiology, John D. Dingell VA Medical Center, Detro

dicine, Wayne State University School of Medicine, Detroit, Michigan; an

e authors have reported that they have no relationships relevant to the c

e authors attest they are in compliance with human studies committe

titutions and Food and Drug Administration guidelines, including patien

it the JACC: Case Reports author instructions page.

nuscript received November 19, 2019; revised manuscript received April

DIFFERENTIAL DIAGNOSIS

Because of the incidental CT findings in thisasymptomatic patient, the possibility of congenitalversus acquired coronary aneurysm was raised.Given that the patient had not undergone any cor-onary interventions, the possibility of past iatro-genic causes or coronary manipulation wasexcluded.

INVESTIGATIONS

Subsequent coronary CT angiography (CTA) showeda giant (3.7-cm), calcified proximal LCX aneurysmwith a small thromboatheroma (Figure 1). An addi-tional smaller, noncalcified distal LCX aneurysmwas present. Furthermore, the distal LCX drainedinto a confluent structure receiving terminal por-

https://doi.org/10.1016/j.jaccas.2020.04.059

it, Michigan; bDivision of Cardiology, Department of

d the cHenry Ford Health System, Detroit, Michigan.

ontents of this paper to disclose.

es and animal welfare regulations of the authors’

t consent where appropriate. For more information,

21, 2020, accepted April 29, 2020.

LEARNING OBJECTIVES

� LCX aneurysm is an extremely rare clinicalcondition. Careful evaluation of the coronaryanatomy is needed to identify any additionalcoronary anomalies in these patients. Ourcase represents a unique anatomy, with agiant LCX aneurysm and the distal LCX

FIGURE 1 3D VR CTA Image

A 3-dimensional (3D) volume-re

shows a giant left circumflex (L

artery (LAD), obtuse marginal b

fistula. CTA ¼ computed tomog

ABBR EV I A T I ON S

AND ACRONYMS

CT = computed tomography

CTA = computed tomography

angiogram

LCX = left circumflex artery

LV = left ventricle

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tions of the remainder of the coronary ar-teries (Figures 2 and 3). This confluencecommunicated with the left ventricle (LV)through a moderate-caliber transmural fis-tulous tract (Figures 3 and 4). The leftanterior descending artery was markedlyenlarged and tortuous proximally, and itappeared to drain into the arterial conflu-

draining into a confluence receiving terminalportions of all coronary arteries andcommunicating with the LV through atransmural fistulous tract.

� Complex coronary anomalies require in-depth evaluation, including multimodalityimaging to assess the patients for presenceof myocardial ischemia and significant left-to-right shunt. Cardiac CTA is a very helpfuldiagnostic tool in establishing definitiveanatomy in these cases.

� The treatment should be individualized onthe basis of the patients’ symptoms, objec-tive prediction of risk (presence of ischemia,progression of the lesion), and the presenceof associated coronary and cardiacanomalies.

� With a careful work-up and follow-up, thiscoronary anomaly may have a benign short-term clinical outcome.

ence distally. The right coronary artery was domi-nant, with a normal caliber, and it terminateddistally into the confluence. Increased trabeculationwas present in the LV at the anterolateral segment.A transthoracic echocardiogram (Figure 5) showednormal left ventricular and right ventricular systolicfunction without any wall motion abnormalities orvalvular heart disease. Cardiac magnetic resonancerevealed normal resting myocardial perfusion,normal biventricular function, a normal pulmonary-to-systemic flow ratio, and no evidence of delayedgadolinium enhancement (Figures 6A and 6B). Anexercise single-photon emission computed tomog-raphy myocardial perfusion imaging study demon-strated no inducible ischemia, with normal leftventricular ejection fraction. We could not identifyany additional congenital abnormalities or signs ofinfection or vasculitis in the past medical history orduring the index evaluation.

ndered (VR) image of the lateral cardiac surface that

CX) coronary artery aneurysm, left anterior descending

ranch, and part of the left ventricular (LV) transmural

raphy angiography; LA ¼ left atrium.

MANAGEMENT

The patient was managed conservatively using dualantiplatelet therapy (aspirin and clopidogrel) and astatin because coronary artery thrombosis and pro-gressive stenosis within the aneurysm may causemyocardial ischemia, which increases the risk ofmyocardial infarction and sudden cardiac death inthese patients. The patient took warfarin for a month,but it was stopped because of hematuria and hema-tospermia. He also underwent yearly coronary CTAfor surveillance without significant change in the sizeof the aneurysm, and he has remained without car-diac complications to date for a total of 3 years. Atpresent, nonradiation modalities such as cardiacmagnetic resonance are not beneficial in this casebecause of the need in a 3-dimensional imagingmodality with excellent all-axis spatial resolution(isometric voxels), for depiction of such a complexcoronary anatomy.

DISCUSSION

The incidence of coronary artery aneurysms is 0.02%to 0.04%, and these aneurysms are usually seen inthe right coronary artery. To our knowledge, therehas been no case reported of a patient with an LCX

FIGURE 2 3D VR and MPR CTA Images

These 3D VR images of the inferior and lateral cardiac surface show a giant LCX coronary artery aneurysm, an LV transmural fistula, and

confluence receiving terminal portions of all the coronary arteries. Multiplanar reconstruction (MPR) images show a giant left circumflex

coronary artery aneurysm and confluence receiving terminal portions of all the coronary arteries. RA ¼ right atrium; RV ¼ right ventricle;

other abbreviations as in Figure 1.

FIGURE 3 3D VR and MPR CTA Images

These 3D VR images of the apical and lateral cardiac surface show the LAD, LCX, LV transmural fistula, and confluence receiving terminal

portions of all the coronary arteries. MPR images show an LV transmural fistula and confluence receiving terminal portions of all the coronary

arteries. Abbreviations as in Figures 1 and 2.

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FIGURE 4 3D VR and MPR CTA Images

These 3D VR and MPR images show a giant LCX coronary artery aneurysm, an LV transmural fistula, and the LCX. Abbreviations as in Figure 1.

FIGURE 5 Apical 4-Chamber Echocardiogram

This apical 4-chamber echocardiogram shows normal-size cardiac chambers, normal valves, and a calcified LCX aneurysm. Abbreviations as in

Figures 1 and 2.

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FIGURE 6 CMR SSFP Images in Systole and Diastole

Images obtained in (A) systole and (B) diastole demonstrate findings similar to those seen in the echocardiogram in Figure 5. A mild increase in the LCX aneurysm

dimension in diastole is noted. CMR ¼ cardiac magnetic resonance; SSFP ¼ steady-state free precession; other abbreviations as in Figures 1, 2, and 5.

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aneurysm communicating with a distal coronaryarterial confluence, and this confluence furtherconnected to the LV through a fistulous track. LCXaneurysms with fistulous communication have rarelybeen reported; among the reported aneurysms, mostcommunicated with the coronary sinus (1). Only 1case report described LCX aneurysm with a fistulouscommunication to the LV myocardium throughseveral small vessels (2).

Coronary artery aneurysms may represent apotentially life-threatening condition with importantcomplications including thrombosis or rupture. Ofnote, our patient was asymptomatic. The availabletreatment options for coronary artery aneurysm withfistula are surgical or endovascular interventions (1).However, treatment depends on the complexity ofthe anomalous anatomy. Furthermore, the follow-upduration and treatment with surgical or endovas-cular interventions in asymptomatic patients are un-known. A systematic approach with possiblemulticenter registries, development of a specificdiagnostic approach, and prognostic estimates in pa-tients with this complex anatomy, requires furtherresearch.

FOLLOW-UP

The patient underwent yearly coronary CTA for sur-veillance without significant change in the size of theLCX aneurysm, and he has remained without cardiaccomplications to date for a total of 3 years.

CONCLUSIONS

LCX aneurysm is an extremely rare clinical condition.Careful assessment of the coronary anatomy isneeded to identify any additional coronary anomaliesin these patients. Evaluation of such a uniqueanomaly as giant LCX aneurysm combined with distalLCX draining into a confluence receiving terminalportions of all coronary arteries and communicatingwith left ventricle through a transmural fistuloustract requires implementation of advanced3-dimensional imaging methods.

ADDRESS FOR CORRESPONDENCE: Dr. AidenAbidov, Division of Cardiology, John D. Dingell VAMedical Center, 4646 John R, Detroit, Michi-gan 48201. E-mail: aiden.abidov@wayne.edu.

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RE F E RENCE S

1. Gupta V, Truong QA, Okada DR, et al. Images incardiovascular medicine. Giant left circumflex cor-onary artery aneurysmwith arteriovenous fistula tothe coronary sinus. Circulation 2008;118:2304–7.

2. Suh SY, Kim JW, Yong HS, et al. Aneurysm ofcircumflex coronary artery caused by cardiac veinand fistulous connection to the left ventricleidentified on MDCT. Int J Cardiol 2007;114:e3–4.

KEY WORDS coronary artery aneurysm,coronary fistula, left ventricular fistula