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Ann Thorac Surg 1996;61:984-986
© 1996 The Society of Thoracic Surgeons

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Case Report

Successful Repair of a Massive Coronary Arteriovenous Fistula in a 68-Year-Old Man

Division of Cardiovascular Surgery, Texas Heart Institute, Houston, Texas

Accepted for publication August 23, 1995.

	Abstract

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Successful ligation of a massive coronary arteriovenous fistula in a 68-year-old man is reported. The defect, which extended from the left coronary artery to the coronary sinus, had been diagnosed 17 years earlier. At the time of repair, the patient's age and fistula-related complications made him a relatively high-risk operative candidate. In light of this case, operative approaches for repairing coronary arteriovenous fistulas are discussed.

	Introduction

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Coronary arteriovenous fistula, first described by Krause in 1865 [1], is an uncommon lesion that is usually congenital and isolated [2]. Because of a lack of symptoms, it may remain undetected for many years. This report describes a massive coronary arteriovenous fistula that remained untreated for 17 years until the onset of symptoms prompted operative intervention.

A 68-year-old man with a congenital coronary arteriovenous fistula presented with a 2-week history of progressive shortness of breath, cough, and orthopnea. The fistula, which extended from the left coronary artery to the coronary sinus, had been diagnosed 17 years earlier but had remained asymptomatic. The patient's cardiologist had provided long-term observation only.

On admission to our hospital, the patient had atrial fibrillation, with a heart rate of 118 beats/min. He was tachypneic but comfortable. Signs of right-side heart failure included severe jugular venous distention and moderate hepatomegaly. Auscultation of the chest revealed bibasilar rales and a continuous heart murmur best heard along the left sternal border. Chest roentgenograms showed cardiomegaly and pulmonary congestion, and electrocardiography results revealed atrial fibrillation but no ischemia.

The patient was admitted to the intensive care unit, where his condition stabilized. Cardiac catheterization disclosed severe mitral and tricuspid valve regurgitation. Selective coronary angiography showed a massively dilated, tortuous, partially calcified fistula that extended from the left coronary artery to the coronary sinus (Fig 1). The right coronary artery was normal. The left anterior descending and diagonal branches were visualized but poorly opacified; this finding was attributed to a coronary steal phenomenon or Venturi effect. The circumflex system could not be visualized because of the size of the fistula. The cardiac ejection fraction was estimated to be 0.30.

View larger version (76K): [in this window] [in a new window]   Fig 1. . Selective coronary angiography before contrast injection (A) and after injection (B) showing the massively dilated, tortuous, partially calcified fistula, which extended from the left coronary artery to the coronary sinus.

Although the patient was weaned from CPB without difficulty, short-term intraaortic balloon counterpulsation was necessary during the immediate postoperative period because of low cardiac output syndrome. He recovered uneventfully and was discharged from the hospital 2 weeks later.

	Comment

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Although coronary arteriovenous fistulas usually occur between the right coronary artery and the right ventricle, they may involve any chamber or coronary artery [4]. Their hemodynamic importance varies, depending on their size and the structures involved. Fistulas to the right heart chambers may produce left to right shunting and volume overloading of the pulmonary circulation, whereas fistulas to the left heart chambers cause left ventricular volume overloading. In either case, myocardial ischemia may arise because of a coronary steal effect [5]. Although several cases of spontaneous closure have been reported [6], symptoms and complications tend to increase with age. Fifty percent of untreated patients experience symptoms by the second decade of life [7]. As these patients age, they become subject to fistula-related congestive heart failure, myocardial infarction, bacterial endocarditis, life-threatening dysrhythmias, and fistula rupture [7].

The diagnosis of coronary arteriovenous fistula should be considered whenever physical examination reveals a continuous heart murmur. If present, symptoms include angina, palpitations, and manifestations of congestive heart failure (fatigue, dyspnea on exertion, and orthopnea). The differential diagnosis includes patent ductus arteriosus, ruptured sinus of Valsalva, and aorticopulmonary window. Aortography and selective coronary angiography remain the diagnostic procedures of choice. Catheterization helps determine the shunt size, the precise features of the anomalous vessel, and the presence of coronary obstruction or coexisting abnormalities.

We agree with the majority of other experts [4, 8], who recommend that surgical intervention be undertaken at the time of diagnosis, especially in cases involving large shunts, regardless of the presence of symptoms. Our case illustrates the rationale for this strategy. The ruptured mitral chordae tendineae and the tricuspid regurgitation resulted from a left ventricular myocardial steal and volume overload of many years' duration; these conditions, together with the patient's age, placed him at relatively high operative risk.

Since 1947, when the first successful ligation of a coronary arteriovenous fistula was performed by Bjork and Crafoord [9], several techniques have been proposed for eliminating these defects while preserving coronary flow. The optimal surgical procedure and the need for CPB depend on the fistula's size, location, and drainage pattern, as well as the presence of associated cardiac lesions. Direct proximal and distal ligation may be performed without CPB and is most suitable for terminal fistulas arising from a main coronary trunk [4]. Nevertheless, this approach entails a risk of myocardial ischemia or infarction and, therefore, may necessitate revascularization. Intraoperative transesophageal echocardiography for continuous monitoring of ventricular function is useful in detecting myocardial compromise [10]. Another technique that avoids CPB is tangential arteriorrhaphy, which preserves antegrade flow through the normal vessel and is best used for lateral fistulas [4].

In most cases, the fistula's location precludes safe dissection for ligation or arteriorrhaphy, so CPB must be used. With this approach, the surgeon can explore the involved cardiac chamber and perform direct intracardiac closure of the fistula's distal end. Localization of the distal end is facilitated by briefly releasing the aortic cross-clamp or infusing cardioplegic solution. In our case, successful distal ligation of the fistula, without revascularization, was performed with CPB. Fistula drainage into the coronary sinus was an obvious obstacle to direct intracardiac ligation.

In selected cases in which operative intervention is deemed too risky, the fistula is closed by means of transcatheter embolization [11]. Because this method is relatively new, however, its safety and long-term benefits are unproven. Unless contraindicated, operative intervention remains the treatment of choice and, when performed electively, produces low mortality rates.

	Footnotes

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Address reprint requests to Dr Cooley, Texas Heart Institute, PO Box 20345, Houston, TX 77225.

	References

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1. Krause W. Über den Ursprung einer access orischen a. coronaria aus der a. pulmonaris. Z Ratl Med 1865;24:225–7.
2. Lowe JE, Oldham HN Jr, Sabiston DC Jr. Surgical management of congenital coronary artery fistulas. Ann Surg 1981;194:373–80.[Medline]
3. De Vega NG. La anuloplastia selectiva, regulable y permanente. Rev Esp Cardiol 1972;25:555–6.[Medline]
4. Fernandes ED, Kadivar H, Hallman GL, et al. Congenital malformations of the coronary arteries: the Texas Heart Institute experience. Ann Thorac Surg 1992;54:732–40.[Abstract/Free Full Text]
5. Theman TE, Crosby DR. Coronary artery steal secondary to coronary arteriovenous fistula. Can J Surg 1981;24:231–3, 236.[Medline]
6. Davis JT, Allen HD, Wheller JJ, et al. Coronary artery fistula in the pediatric age group: a 19-year institutional experience. Ann Thorac Surg 1994;58:760–3.[Abstract/Free Full Text]
7. Liberthson RR, Sagar K, Berkoben JP, Weintraub RM, Levine FH. Congenital coronary arteriovenous fistula: report of 13 patients, review of the literature and delineation of management. Circulation 1979;59:849–54.[Abstract/Free Full Text]
8. Blanche C, Chaux A. Long-term results of surgery for coronary artery fistulas. Int Surg 1990;75:238–9.[Medline]
9. Bjork G, Crafoord C. Arteriovenous aneurysm on the pulmonary artery simulating patent ductus arteriosus botalli. Thorax 1947;2:65–8.[Free Full Text]
10. Stevenson JG, Sorenson GK, Stamm SJ, McCloskey JP, Hall DG, Rittenhouse EA. Intraoperative transesophageal echocardiography of coronary artery fistulas. Ann Thorac Surg 1994;57:1217–21.[Abstract/Free Full Text]
11. Reidy JF, Anjos RT, Qureshi SA, Baker EJ, Tynam MJ. Transcatheter embolization in treatment of coronary artery fistulas. J Am Coll Cardiol 1991;18:187–92.[Abstract]

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This Article Abstract 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): Denton A. Cooley Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Phillips, W. S. Articles by Cooley, D. A. Search for Related Content PubMed PubMed Citation Articles by Phillips, W. S. Articles by Cooley, D. A.