Ann Thorac Surg 2002;73:969-970
© 2002 The Society of Thoracic Surgeons
Case report
Right coronary artery and interatrial septal aneurysms with fistulous connection to the right atrium
Antonino G.M. Marullo, MD, PhDa,
Joseph F. Sabik, MD*a
a Department of Thoracic and Cardiovascular Surgery, The Cleveland Clinic Foundation, Cleveland, Ohio, USA
Accepted for publication July 30, 2001.
* Address reprint requests to Dr Sabik, Department of Thoracic and Cardiovascular Surgery/F25, The Cleveland Clinic Foundation, 9500 Euclid Ave, Cleveland, OH 44195, USA
e-mail: sabikj{at}ccf.org
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Abstract
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Coronary artery aneurysmal disease is a rare pathology occasionally associated with coronary artery to venous fistulous connection. We report a case of right coronary artery aneurysm with fistulous connection to the right atrium associated with an aneurysm of the interatrial septum.
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Introduction
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Coronary artery aneurysms as defined by the Coronary Artery Surgery Study (CASS) are coronary artery dilatations of more than 1.5 times the diameter of the patient’s largest coronary artery [1]. The incidence of coronary artery aneurysms among patients undergoing coronary angiography is 0.2% to 4.9% and the most common complications of coronary artery aneurysms are rupture and myocardial infarction due to thromboembolism [2]. Coronary artery aneurysms occur most commonly in the right coronary artery, followed by the circumflex artery and the left anterior descending artery.
Coronary arteriovenous fistulas are more rare than coronary artery aneurysms, with a reported incidence of 0.1% to 0.2% [3]. The association of these two malformations usually involves the right coronary artery with a fistulous connection to the right ventricle, pulmonary artery, superior vena cava, right atrium, or coronary sinus. We report a case of successful repair of right coronary artery aneurysm with fistulous connection to the right atrium and aneurysmal dilatation of the fistula involving the interatrial septum.
A 45-year-old woman was referred for evaluation of a myocardial murmur first diagnosed at age 30 years as a sinus of Valsalva aneurysm. She described the recent gradual onset of fatigue with intermittent dry cough and dyspnea with mild exertion. On physical examination, she had a grade II/VI systolic ejection murmur along the left sternal border and a grade II/VI diastolic murmur along the right sternal border. Chest radiography showed mild cardiomegaly and the electrocardiogram was consistent with a prior inferior myocardial infarction. The preoperative echocardiograms showed images that were thought to be consistent with aneurysms of both the right and the noncoronary sinuses of Valsalva, with rupture of the noncoronary sinus into the right atrium. A cardiac catheterization was performed to confirm these findings. The left coronary artery system was normal and the right coronary artery was visualized only nonselectively from the aortogram and appeared to arise from an aneurysmal sinus of Valsalva. To help delineate the anatomy, magnetic resonance imaging was performed and revealed an elongation of the right Valsalva sinus with a suggestion of communication along its undersurface involving the right atrium.
The patient underwent surgery through a median sternotomy. An aortotomy was performed and no sinus of Valsalva aneurysms were identified. The right coronary artery ostium was markedly enlarged with a diameter of 2.0 cm, and the right coronary artery was aneurysmal measuring 3.0 x 2.0 cm. A right atriotomy was performed and a second aneurysm was identified in the atrial septum just below the aortic root. This second aneurysm communicated with both the right coronary artery aneurysm and the right atrium and there was a 1-cm to 1.5-cm fistulous connection between the right coronary artery aneurysm and the atrial septal aneurysm (Fig 1).
The fistulous tract and the aneurysm of the interatrial septum as well as the right coronary artery aneurysm were ligated, the right coronary ostium was closed with a Hemashield patch through the aortotomy, and the right coronary artery was distally bypassed with a saphenous vein graft. After closure of both the aorta and the right atrium, the procedure was completed and the patient was returned to the postoperative intensive care unit in stable condition. Specimen pathology was normal. The postoperative course was uncomplicated and the patient was discharged on postoperative day 5.
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Fig 1. Intraoperative photograph (A) and diagram (B) showing the right coronary and interatrial septum aneurysms and the fistulous connection of the two aneurysms with the right atrium (arrow).
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Comment
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Coronary artery aneurysms are rare [2]. The right coronary artery is the most commonly affected vessel and the most common etiology is arteriosclerotic disease followed by Kawasaki disease, periarterite nodosa, systemic lupus erythematosus, syphilis, rheumatic fever, congenital, and trauma including coronary angioplasty and stenting [4]. Coronary artery arterovenous fistulas are also rare and are occasionally associated with aneurysmal disease [3]. Fistulous connection to the cardiac chambers may result from rupture of the coronary artery aneurysm, which is a very rare event [5], or may represent the primary anomaly that resulted in the aneurysm [3]. Diagnosis is suspected on the basis of the clinical history and echocardiographic examination and is confirmed by angiographic study. In the case we describe, the diagnosis was missed preoperatively and confused with sinus of Valsalva aneurysms. The main problem in making the right diagnosis was that it was impossible to obtain a direct injection of the right coronary artery for the angiographic study. Only the patient’s history was suggestive of the true diagnosis. She had a history of cardiac murmur dating back 14 years, electrocardiographic changes suggestive of inferior myocardial infarction without evidence of coronary disease, and signs of communication to a right chamber with a significant left-to-right shunt.
Indication for surgery is considered on the basis of the grade of obstruction associated with this disease in its atherosclerotic variant, the occurrence of rupture or fistulization, and sometimes on the evidence of a saccular shape of the aneurysm, which is demonstrated to rupture more easily than the fusiform variant. In the case presented the indication for surgery was clear even if misdiagnosed. The patient had signs of left-to-right shunt and evidence of fistulization to the right atrium. The treatment we used has been described by Emmerich and colleagues [6]. Other surgical corrections described include resection with end-to-end interposition of a vein graft, bypassing with or without ligation, and reconstruction of the vessel with plication of the aneurysm.
The singularity of this case consists in the aneurysm of the interatrial septum associated with the fistula to the right atrium. The combination of aneurysm of the coronary artery with aneurysm of the interatrial septum and fistulous connection among these two and the right atrium, as well as the normal pathology findings and the history of the patient, suggest that the primary anomaly was a congenital fistula, which as described by Fernandes and colleagues [3] caused the development of the coronary artery aneurysm and the interatrial septum aneurysm.
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References
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Robertson T., Fisher L. Prognostic significance of coronary artery aneurysm and ectasia in the Coronary Artery Surgery Study (CASS) registry. Prog Clin Biol Res 1987;250:325-339.[Medline]
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Falsetti H.L., Carrol R.J. Coronary artery aneurysm. A review of the literature with a report of 11 new cases. Chest 1976;69:630-636.[Abstract/Free Full Text]
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Fernandes E.D., Kadivar H., Hallman G.L., Reul G.J., Ott D.A., Cooley D.A. Congenital malformations of coronary arteries: the Texas Heart Institute experience. Ann Thorac Surg 1992;54:732-740.[Abstract]
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Dagalp Z., Pamir G., Alpman A., Omurlu K., Erol C., Oral D. Coronary artery aneurysm. Report of two cases and review of the literature. Angiology 1996;47:197-201.
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Chapman R.W.G., Watkins J. Rupture of right coronary artery aneurysm into the right atrium. Br Heart J 1978;40:938-939.[Abstract/Free Full Text]
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Emmerich J., Thomas D., Drobinski G., et al. Diagnosis of coronary aneurysms in sibling. Treatment with a new surgical procedure. Eur Heart J 1989;10:91-99.[Abstract/Free Full Text]
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