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Ann Thorac Surg 2004;77:1081-1083
© 2004 The Society of Thoracic Surgeons

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Case report

Left main coronary artery-to-pulmonary artery fistula with severe aneurysmal dilatation

^a Division of Cardiovascular Surgery, Duke University Medical Center, Durham, NC, USA
^b Division of Cardiology, Duke University Medical Center, Durham, North Carolina, USA

Accepted for publication May 2, 2003.

^* Address reprint requests to Dr Gandy, Department of Cardiothoracic Surgery, Falk Cardiovascular Research Center, 300 Pasteur Drive, Stanford, CA 94305-5406; , USA
e-mail: gandy001{at}mc.duke.edu

	Abstract

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Coronary artery fistulas (CAF) are rare abnormalities that can be symptomatic or asymptomatic. Most drain into the right ventricle or pulmonary artery, though a variety of other drainage sites have been reported. We report the results of the surgical closure of a symptomatic left coronary-to-pulmonary artery fistula associated with a giant 10-cm aneurysm and discuss the management of coronary artery fistulas.

	Introduction

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Coronary artery fistulas (CAF) are rare cardiovascular abnormalities characterized by a range of clinical presentations. Some CAF are asymptomatic; others have symptoms that range from mild shortness of breath to angina. The last two decades have seen advances in interventional catheterization procedures that have had implications for the treatment of CAF. We present a case of CAF that has been treated surgically and use this case as a forum to discuss the management of CAF in the current clinical environment.

A 62-year-old woman presented to her primary care physician with the new onset of a cough productive of yellow sputum. The patient was mildly short of breath and was limited in her ability to participate in her previously active lifestyle. She had no associated chest pain, palpitations, orthopnea, paroxysmal nocturnal dyspnea, or peripheral edema. She had been told since childhood that she had a heart murmur and had no history of previous thoracic surgical procedures.

A chest roentgenogram demonstrated a large mass on the left side of her cardiac silhouette (Fig 1A). A computed tomographic (CT) scan demonstrated a large coronary aneurysm that was about 8 cm at its largest diameter (Fig 1B). Transthoracic echocardiography demonstrated a fistula between the left coronary system and the main or left pulmonary artery (PA). A cardiac catheterization was performed in an effort to more carefully delineate the anatomy. This study confirmed the presence of the left coronary–PA fistula with a giant aneurysm (Fig 1C) and documented a Qp/Qs of 1.5:1. The remaining coronary anatomy was normal. It was unclear if the fistula originated from the left anterior descending (LAD) or the circumflex coronary artery.

View larger version (59K): [in this window] [in a new window]   Fig 1. (A) The aneurysm appears as a mediastinal mass (arrow) along the left cardiac border. (B) A chest computed tomographic scan shows an S-shaped fistulous tract originating from the left main coronary and ending in a large aneurysm (An). (Ao = aorta.) (C) A left ventriculogram shows the fistula (Fi) originating from the left main coronary artery. The aneurysm (An) is roughly the size of the left ventricle (LV) cavity.

	Comment

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Coronary artery fistulas are rare abnormalities with an estimated frequency of 0.27% to 0.4% of all congenital cardiac lesions [1]. The causative factors are unknown, but most are thought to originate as congenital anomalies or, less commonly, as a result of injury during coronary intervention or a surgical procedure. Most fistulas arise from the right coronary artery, with a smaller number originating from the left coronary artery. Most coronary fistulas drain into either the PA or RV, though RA, RV outflow tract, left atrium, and LV drainage sites have been reported [2]. CAF in adults are often accompanied by aneurysmal dilatation of the coronary artery, as was seen in this case.

Most coronary artery fistulas are asymptomatic. Symptoms are more likely to develop in older patients [1] or those with a larger CAF. Patients can, however, present with myocardial ischemia, angina, congestive heart failure, bacterial endocarditis, cardiac arrhythmia, or fistula rupture with or without associated chest pain and tamponade. Current treatment options include careful observation, surgical ligation with or without cardiopulmonary bypass, ligation with bypass of the involved coronary artery [3], and transcatheter embolization. Surgical ligation has been very successful, with 0% mortality in the three published series in the 1980s (reviewed in Mavroudis and associates [4]) and 1.4% mortality in the six published series in the 1990s (reviewed in Armsby and coworkers [2]). Closure rates have ranged from 50% (reviewed in Armsby and coworkers[2]) to 100% [4]. The most common complications of surgical therapy have included arrhythmias, transient ischemic attacks, and stroke. One operative death has been reported since 1979 [5].

Transcatheter closure of CAF began in 1983, and experience and results have improved since that time. Available devices for closure in the United States include coils, detachable balloons, and umbrella devices. In a recent report of 33 patients who underwent transcatheter closure, no mortality occurred and complete closure was established in 82% [2]. The most common complications included transient ST-T wave changes, transient arrhythmias, distal coronary spasm, fistula dissection, and unretrieved coil embolization. Fifteen percent of patients were not treated with catheter intervention secondary to anatomic restrictions that included multiple drainage sites and vessel tortuosity. Some series have reported that as little as 37% of fistulas will be amenable to catheter closure when anatomic suitability is considered [4].

There is general agreement that symptomatic patients should be treated. Patients with CAF and another complex cardiac lesion for which an operation would be indicated should undergo surgical intervention. The decision between transcatheter closure and surgical treatment should be based on the coronary anatomy and the expertise at the institution. There is disagreement in the treatment of asymptomatic patients, some of whom have tiny fistulas that are now being diagnosed incidentally by echocardiography. Some propose that these patients should be followed without intervention because cases of spontaneous closure have been reported. Others, however, favor the more aggressive approach of closure of CAF because of the significance of the potential complications [4]. Further study is necessary to determine the optimal management for these patients, but current management should be determined on a case-by-case basis. A proposed algorithm for the management of CAF is shown in Figure 2.

View larger version (30K): [in this window] [in a new window]   Fig 2. Recommended treatment algorithm of coronary artery fistulas (CAF). (Echo = echocardiography; SBE = subacute bacterial endocarditis scintigraphy; TCC = transcatheter closure.)

	References

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1. Liberthson R.R., Sagar K., Berkoben J.P., Weintraub R.M., Levine F.H. Congenital coronary arteriovenous fistula. Report of 13 patients, review of the literature and delineation of management. Circulation 1979;59:849-854.[Abstract/Free Full Text]
2. Armsby L.R., Keane J.F., Sherwood M.C., Forbess J.M., Perry S.B., Lock J.E. Management of coronary artery fistulae. Patient selection and results of transcatheter closure. J Am Coll Cardiol 2002;39:1026-1032.[Abstract/Free Full Text]
3. Lowe J.E., Oldham H.N., Jr, Sabiston D.C. Surgical management of congenital coronary artery fistulas. Ann Surg 1981;194:373-380.[Medline]
4. Mavroudis C., Backer C.L., Rocchini A.P., Muster A.J., Gevitz M. Coronary artery fistulas in infants and children: a surgical review and discussion of coil embolization. Ann Thorac Surg 1997;63:1235-1242.[Abstract/Free Full Text]
5. Schumacher G., Roithmaier A., Lorenz H.P., et al. Congenital coronary artery fistula in infancy and childhood: diagnostic and therapeutic aspects. Thorac Cardiovasc Surg 1997;45:287-294.[Medline]

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Kimberly L. Gandy James J. Jaggers Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Gandy, K. L. Articles by Jaggers, J. J. Search for Related Content PubMed PubMed Citation Articles by Gandy, K. L. Articles by Jaggers, J. J. Related Collections Great vessels