Fig 3. (A) Intraoperative findings and a (B)schema are shown after semirigid ringannuloplasty and direct closure of mitralcleft. The aneurysm of the anterior leaflet wasclearly seen during the saline test (arrows).

Fig 2. (A) Intraoperative photograph of themitral leaflets and a (B) schema are shown.The arrows indicate a “collapsed” mitralvalve aneurysm. The arrowheads indicate amitral cleft between the A2 and A3 portions.A strut chorda of the A2 portion is hooked.

2240 CASE REPORT GUNDRE ET AL Ann Thorac SurgGIANT CORONARY ANEURYSM WITH FISTULA 2013;96:2240–3

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was made primarily by transthoracic echocardiography,but MVA was not found until TEE was performed. The3D-TEE modality is an important and reliable diagnosticoption compared with conventional 2D-TEE [7]. Theability of 3D-TEE to display a surgeon’s view from the leftatrial side provides valuable information preoperatively.

An MVA with a cleft is a good indication for mitralvalve repair. The precise preoperative diagnosis by TEEis useful for developing a strategy for mitral valve repair[1, 4]. However, it is sometimes difficult to repair, andvalve replacement is needed, such as in cases with theMVA occupying a larger part of the mitral leaflet [3, 8].Mitral valve repair should be considered, especially insuch a young and elective patients. Long-term results ofmitral valve repair for these entities are not clear becauseof its rarity, so careful follow-up is necessary.

In conclusion, we encountered a rare case of an MVAwith a cleft in an adult patient and successfully repairedthe lesion. The 3D-TEE modality provides excellentanatomic information preoperatively.

Accepted for publication April 8, 2013.

Address correspondence to Dr Mishra, Dr PK Sen Department of Car-diovascular & Thoracic Surgery, Rm 305, CVTC Bldg, King Edward Me-morial Hospital, Parel, Mumbai 400 012. Maharashtra, India; e-mail:drprashantmishra100@yahoo.co.in.

References

1. Halkos ME, Symbas JD, Felner JM, Symbas PN. Aneurysm ofthe mitral valve: a rare complication of aortic valve endo-carditis. Ann Thorac Surg 2004;78:e65–6.

2. Piper C, Hetzer R, Korfer R, et al. The importance of sec-ondary mitral valve involvement in primary aortic valveendocarditis; the mitral kissing vegetation. Eur Heart J2002;23:79–86.

3. Gajjar TP, Desai NB. True aneurysm of anterior mitral leaflet-A rare entity. J Thorac Cardiovasc Surg 2012;144:e93–5.

4. Piazza N, Marra S, Webb J, et al. Two cases of aneurysm of theanterior mitral valve leaflet associated with transcatheteraortic valve endocarditis: a mere coincidence? J Thorac Car-diovasc Surg 2010;140:36–8.

� 2013 by The Society of Thoracic SurgeonsPublished by Elsevier Inc

5. Imamura H, Sakamoto S, Maruyama Y, Ochi M, Shimizu K.Two-patch repair for atrioventricular septal defect with mitralaneurysm. Ann Thorac Surg 2009;88:1341–3.

6. Vilacosta I, San Roman JA, Sarria C, et al. Clinical, anatomic,and echocardiographic characteristics of aneurysms of themitral valve. Am J Cardiol 1999;84:110–3.

7. Peters S, EssopR. Congenital submitral aneurysmwith ruptureinto the left atrium: assessment by 2D and 3D transesophagealechocardiography. Echocardiography 2011;28:E121–4.

8. Gao C, Xiao C, Li B. Mitral valve aneurysm with infectiveendocarditis. Ann Thorac Surg 2004;78:2171–3.

Giant Aneurysm of the Left AtrialBranch of the Left CircumflexArtery With FistulaNitin P. Gundre, MS, Prashant Mishra, MCh,Balaji Aironi, MCh, Pradeep Vaideeswar, MD, andNandkishor Agrawal, MCh

Dr PK Sen Department of Cardiovascular & Thoracic Surgeryand Department of Pathology (Cardiovascular & ThoracicDivision), Seth GS Medical College and King Edward MemorialHospital, Mumbai, India

Giant coronary artery aneurysm with a fistula is a rarecondition. We present one of the largest aneurysms of leftcircumflex coronary artery territory, arising from the leftatrial branch of the left circumflex coronary artery. It had

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2241Ann Thorac Surg CASE REPORT GUNDRE ET AL2013;96:2240–3 GIANT CORONARY ANEURYSM WITH FISTULA

a maximum diameter of 10 cm, with a fistulous connec-tion to the right atrium. Total exclusion of the aneurysmalmass was achieved by ligation of the afferent artery,closure of the entry point from within the aneurysm, andclosure of the fistulous communication from within theright atrium. The patient’s postoperative course wasuneventful.

(Ann Thorac Surg 2013;96:2240–3)� 2013 by The Society of Thoracic Surgeons

oronary arterial aneurysm (CAA) is defined as a

Fig 1. Coronary angiography shows the giant coronary arteryaneurysm.

Ccoronary dilatation that exceeds by 1.5 times thediameter of the adjacent normal arterial segment or thepatient’s largest coronary artery. CAAs are uncommonlesions, noted in 0.2% to 4.9% of patients undergoingcoronary angiography [1]. Some CAAs may enlarge to adiameter exceeding 20 mm and are called “giant CAA”[1]. At times, CAAs (usually of the right coronary artery)can develop fistulous connections with adjoining cham-bers or vessels [2]. We report the successful repair of agiant CAA of the left atrial branch of the left circumflexcoronary artery with a fistulous connection to the rightatrium. This is one of the largest CAA of circumflex cor-onary artery territory to be reported to date in worldliterature.

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A 51-year-old woman presented with exertional dyspnea,chest pain, facial puffiness, difficulty in swallowing, andpedal edema. On physical examination, she had raisedjugular venous pressure, continuous thrill, and contin-uous grade V/VI murmur along the right sternal border.Stigmata of Marfan syndrome were not present.

A chest roentgenogram showed cardiomegaly and rightatrial enlargement. Echocardiography showed a dilatedleft circumflex coronary artery and a large cystic masscommunicating with the right atrium. Coronary angiog-raphy revealed a huge aneurysm from a branch of thecircumflex coronary artery (Fig 1). To help delineate theanatomy, cardiac computed tomography was performedand revealed a giant saccular aneurysm compressing thesuperior vena cava and both the atrial chambers (Fig 2).

During the surgical repair, femorofemoral bypass wasinstituted to avoid the risk of rupturing the large aneurysmthat was abutting undersurface of the sternum. Then,midline sternotomywas done using a oscillating saw. Afterpericardotomy, a 10- � 8-cm aneurysm was noted post-erosuperior to the right atrium (Fig 2) supplied by a leftatrial branch of the left circumflex coronary artery. Theafferent artery was looped, doubly ligated, and transfixed.

The aneurysm was dissected from the superior venacava and the right atrium. The aneurysm was opened,and part of its wall was excised and sent for histopatho-logic examination. The entry point of the afferent arteryinto the aneurysm was sutured from within the aneu-rysm. The fistulous connection of the aneurysm to theright atrium was closed from the right atrial aspectbecause the external margins were calcified.

Microscopic findings of the resected specimen wereconsistent with cystic medial change (Fig 2). There was nosign of atherosclerotic change in the specimen. The pa-tient’s postoperative course was uneventful, and she wasdischarged.

Comment

CAAs have been diagnosed with increasing frequencysince the advent of coronary angiography. However giantCAAs are rare, with a reported incidence of 0.2% to 4.9%in patients undergoing coronary angiography [2]. Thereported incidence of CAA and giant CAA in the cardiacsurgical population is approximately 0.04% and 0.02%,respectively [1]. The presence of CAAs with fistulizationinto a cardiac chamber is much more unusual. Proximaland middle segments of right coronary artery are themost common sites of CAA, followed by proximal leftanterior descending artery and left circumflex coronaryartery. The most common cause of CAA is atherosclerosis,and others include Kawasaki disease, congenital malfor-mations, infective or noninfective vasculitis, neoplasms,connective tissue disorders (Ehlers-Danlos syndrome,Marfan syndrome), and even iatrogenic (trauma, post-angioplasty) [3, 4].The natural history and standard treatment of giant

CAAs remain unclear because of its rarity. Most patientswith CAAs are asymptomatic. Surgical treatment isessential to avoid complications such as progressiveenlargement, compression of surrounding structures,rupture (leading to cardiac tamponade), superior venacava syndrome, thrombosis, and embolization into thedistal coronary circulation. Coronary steal syndrome,angina, myocardial infarction, and sudden deaths of un-certain cause have also been reported [5]. There is alsopossibility of mechanical interference of coronary flow,dissection of the coronary artery, and fistula formationcausing left-to-right shunt with right ventricular overloadand congestive heart failure.

Fig 2. (1) Computed tomography scan shows the giant coronary artery aneurysm (CAA). (2) A 3-dimensional reconstructed computed tomographyscan shows the CAA and its relationship to other structures. (3) CAA as viewed after sternotomy. (4) Excised flap of the aneurysm wall showssmooth, glistening, slightly undulant intima. (5) Histopathologic examination shows irregularly arranged smooth muscle separated by pools ofground substance (hematoxylin and eosin stain, original magnification x 400). (AO ¼ ascending aorta, LA ¼ left atrium, LV ¼ left ventricle,RA ¼ right atrium, RAA ¼ right atrial appendage.)

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Small aneurysms that produce no symptoms may bemanaged conservatively with regular follow-up, whereasCAAs that produce significant symptoms should beconsidered for surgical management. Nonsurgical treat-ment options include coil embolization and covered stents[3]. No typical surgical procedure has been established forgiant CAA. Principles of surgical treatment of CAA are toexclude the aneurysmal segment and maintain circulationto the involved artery with a saphenous vein graft or oneof the internal mammary arteries. Exclusion is necessaryto prevent complications of rupture, embolism into distalcoronary circulation, and competitive flow, which couldocclude the graft [4].

Various surgical strategies have been adopted, such asplication or resection of the aneurysm and reconstructionwith end-to-end interposition of a vein graft and coronarybypass, with or without ligation [1]. In patients with

coronary artery fistula, the fistula should be closed.The incidence of recurrence is higher in external plicationor division of the fistula compared with intracardiacclosure [6].The aneurysm in our patient was unusually large and

involved a rare location. Surgical correction seemed to benecessary because of her symptoms, the large size of theaneurysm (that would pose a risk of rupture or othercomplications), and fistulization into the right atrium.Although computed tomography imaging showed nomural thrombus in the aneurysmal sac, coil embolizationwas not considered because we feared that distal embo-lism might occur due to the presence of major outflowand the fistulous communication. Because the aneurysmwas arising from the distal-most portion of the coronaryartery and that too from the left atrial branch, coronarybypass was not a treatment option.

2243Ann Thorac Surg CASE REPORT EKEKE ET AL2013;96:2243–5 ANNULOAORTIC ECTASIA IN A PATIENT WITH CAP

We preferred to initiate femorofemoral bypass first,to avoid risk of rupture of the giant aneurysm duringsternotomy, because it was seen abutting the sternum onthe lateral chest roentgenogram. The opening of theafferent artery into the aneurysm and the fistulous open-ing into the right atrium were closed from within theaneurysmal sac and the right atrium, respectively, to avoidrisk of recurrence. On histopathologic examination, theexcised wall, surprisingly, revealed cystic medial change.

We conclude that in patients with giant CAAwith fistula,surgical repair is the treatment of choice and the surgicalstrategy should be carefully planned and individualized.

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References

1. Li D, Wu Q, Sun L, et al. Surgical treatment of giant coronaryartery aneurysm. J Thorac Cardiovasc Surg 2005;130:817–21.

2. Marullo AG, Sabik JF. Right coronary artery and interatrialseptal aneurysms with fistulous connection to the rightatrium. Ann Thorac Surg 2002;73:969–70.

3. Hiramori S, Hoshino K, Hiok h, et al. Spontaneous rupture ofa giant coronary artery aneurysm causing cardiac tamponade:a case report. J Cardiol Cases 2011;3:e119–22.

4. Abou Eid G, Lang-Lazdunski L, Hvass U, et al. Managementof giant coronary artery aneurysm with fistulization into theright atrium. Ann Thorac Surg 1993;56:372–4.

5. Onouchi Z, Hamaoka K, Kamiya Y, et al. Transformation ofcoronary artery aneurysm to obstructive lesion and the role ofcollateral vessels in myocardial perfusion in patients withKawasaki Disease. J Am Coll Cardiol 1993;21:158–62.

6. Sugiura T, Saito S, Kihara S, Sato W, Kurosawa H. Giantcoronary artery aneurysm associated with medial mucoiddegeneration. Ann Thorac Surg 2009;87:933–4.

Annuloaortic Ectasia in a PatientWith Congenital Absence of theLeft PericardiumChigozirim N. Ekeke, BS, Curt Daniels, MD,Subha V. Raman, MD, Charles Hitchcock, MD, PhD,Steven E. Katz, MD, and Juan A. Crestanello, MD

Division of Cardiac Surgery, Cardiovascular Medicine, andDepartments of Pathology, and Ophthalmology, The Ohio StateUniversity Wexner Medical Center, Columbus, Ohio

We report a patient with congenital absence of the leftpericardium with development of progressive annu-loaortic ectasia and aortic insufficiency during a 12-yearperiod. The patient was treated with a Bentall proce-dure. Pathologic examination of the aorta revealed cysticmedial necrosis. The surgical management and a possibleassociation between congenital absence of pericardiumand Marfan syndrome are discussed.

(Ann Thorac Surg 2013;96:2243–5)� 2013 by The Society of Thoracic Surgeons

Accepted for publication April 8, 2013.

Address correspondence to Dr Crestanello, Division of Cardiac Surgery,N-816 Doan Hall, 410 W 10th Ave, Columbus OH 43210; e-mail: juan.crestanello@osumc.edu.

� 2013 by The Society of Thoracic SurgeonsPublished by Elsevier Inc

ongenital absence of the left pericardium (CAP)

Cusually presents as an incidental finding duringevaluation for associated cardiac anomalies [1]. Theextreme cardiac displacement and abnormal location ofcardiac and mediastinum structures can make cardiacprocedures challenging [2]. Coronary artery bypassgrafting and valve operations have been describedin patients with CAP [2, 3]. CAP can be an isolateddefect or can be associated with intracardiac andextracardiac defects [1, 2]. The association of CAP withMarfan syndrome or with other connective disordersthat predispose patients to aortic aneurysms is not wellestablished.We present a case of CAP in a patient with marfanoid

features. Progressive annuloaortic ectasia and congestiveheart failure, secondary to aortic insufficiency, developedover a 12-year period.

A 48-year-old man diagnosed with congenital absence ofthe pericardium (CAP) 12 years earlier [4] presented withdyspnea upon exertion and increased neck pulsations. Atype A aortic dissection had occurred in the patient’sbrother when he was in his early 40s.The physical examination revealed an increased pulse

pressure and a diastolic murmur. Echocardiographydemonstrated severe aortic insufficiency and annu-loaortic ectasia. The sonographic windows were poor.Cardiac magnetic resonance imaging confirmed severeaortic insufficiency, with a regurgitant fraction of 50%.The aortic valve was trileaflet. The left ventricularejection fraction was 0.45, and the left ventricle di-mensions were 76 mm in end-diastole and 47 mmin end-systole. As expected, there was displacement ofthe heart into the left chest, with the apex pointingposteriorly.Computed tomography angiography showed that the

aortic annulus, the sinuses of Valsalva, and the sino-tubular junction were aneurysmal (Fig 1). The arch anddescending aorta were normal in diameter.We recommended surgical intervention based on the

presence of severe symptomatic aortic regurgitationwith decreased left ventricular ejection fraction anddilated left ventricle and the presence of annuloaorticectasia. Upon sternotomy, the heart was covered by theright lung. No adhesions were present. The left phrenicnerve was located anteriorly to the right atrioventric-ular groove. The aorta, pulmonary artery, and superiorand inferior vena cava were in normal position. Thelocation of the aortic sinuses and coronary ostium wasnormal. The ventricular cavities were displaced towardthe left and posteriorly. There was a free pericardialedge next to the right atrium covering the right phrenicnerve.Cardiopulmonary bypass was instituted by cannulating

the distal ascending aorta and the right atrium. Theascending aorta, sinuses of Valsalva, and aortic leafletswere excised. Buttons containing the coronary arterieswere fashioned. A Bentall procedure was performedwith a 32-mm valved conduit. Histologic examination ofthe ascending aortic wall demonstrated medial necrosis

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