Interact CardioVasc Thorac Surg 2008;7:360-361. doi:10.1510/icvts.2007.171629
© 2008 European Association of Cardio-Thoracic Surgery
Congenital bilateral coronary artery to pulmonary artery fistulas associated with left main trunk stenosis
Beltran G. Levy Praschker*,
Akhtar Rama,
Iradj Gandjbakhch and
Alain Pavie
Thoracic and Cardiovascular Surgery Department, University of Paris VI Pierre et Marie, Groupe Hospitalier Pitie-Salpêtrière, Assistance Publique-Hôpitaux de Paris, 47–83, boulevard de l'Hôpital, 75651 Paris Cedex 13, France
Received 12 November 2007;
received in revised form 11 January 2008;
accepted 14 January 2008
*Corresponding author. Tel.: +(33)-1 42 16 56 43; fax: +(33)-1 42 16 56 39.
E-mail address: beltranlevy{at}hotmail.com (B.G. Levy Praschker).
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Abstract
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A rare case of a 57-year-old patient who presented with an acute coronary syndrome with incidental discovery of bilateral coronary arteriovenous fistulas originating from both coronary arteries to the pulmonary artery trunk and coronary artery atherosclerosis.
Key Words: Coronary artery; Fistula
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1. Introduction
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Coronary arteriovenous fistulas (CAVF) were first described by Krauss in 1865 [1]. They are present in 0.002% of the general population [1] and they are the most common hemodynamically significant coronary lesion. In angiographic series they represent between 0.13 and 0.87% of all coronary anomalies [2, 3]. We report one case of bilateral CAVF discovered incidentally during a routine angiogram in a patient evaluated for acute coronary syndrome, who underwent transpulmonary closure of the fistulas and coronary artery bypass graft surgery.
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2. Case report
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A 57-year-old male presented with acute coronary syndrome. An urgent coronary angiogram was performed (Fig. 1, Videos 1 and 2) and revealed left main trunk stenosis and two fistulas arising from left anterior descending artery and right coronary artery to pulmonary artery, with coronary steal from right coronary fistula (Fig. 2). Under cardiopulmonary bypass (CPB), a double coronary artery bypass graft surgery with both left and right internal mammary arteries was undertaken. Closure of pulmonary end of both fistulas was performed through a pulmonary artery arteriotomy. Postoperative course was uneventful. The patient remains asymptomatic at eight postoperative months.
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Fig. 1. Coronary angiogram with (a) left anterior descending artery to pulmonary artery (PA) fistula and left main trunk stenosis (white arrow), and (b) right coronary artery (RCA) to pulmonary artery fistula. We may observe that almost all the contrast agent goes through the fistula.
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Video 1. Left coronary artery angiogram showing a left anterior descending artery to pulmonary artery fistula and left main trunk stenosis.
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Video 2. Right coronary artery angiogram showing the coronary artery-to-pulmonary artery fistula. We may observe almost all contrast agent going to the pulmonary artery through the fistula.
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3. Discussion
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Coronary artery fistulas are rare anomalies, more often originating from right coronary artery [4]. Bilateral fistulas, originating from both the coronary system, account for 5% of the total. These types of fistulas terminate more often into the pulmonary artery (56%) than unilateral fistulas (17%) [4, 5].
Clinical examination may reveal a continuous murmur and/or a thrill in the upper chest. Younger patients (under 20 years) are more often asymptomatic [6]; symptoms may develop later in life due to chronic left-to-right shunt. Liberthson et al. [6] in a review of 174 cases reported in the literature, found that 55% of the patients older than 20 years had symptoms at presentation, such as dyspnoea on exertion (35%), fatigue (8%) or angina (22%). Conversely, only 9% of those <20 years of age had had such symptoms. The incidence of death before operation also increased from 1% in those patients <20 years of age to 14% in those >20 years of age. The most common fistula related complication was cardiac heart failure (12–19%) and endocarditis represented only 3–4% accordingly to age. As seen in this case, these fistulas could be the cause of the coronary steel syndrome, and when they are associated with other cardiac diseases such as coronary atherosclerosis, they may potentiate myocardial ischemia.
In asymptomatic patients, observation with endocarditis prophylaxis is recommended. In this group of patients, closure of the fistula may be recommended in those with significant shunt [7]. General agreement exists that symptomatic patients should be treated. Current treatment options include: transcatheter embolization or surgical ligation. Transcatheter approach presents a closure rate of 85% [8]. Nevertheless, vessel tortuosity and multiple drainage sites may render CAVF unsuitable for this technique [8]. Various surgical techniques were described. They include: internal closure from within the cardiac chamber or pulmonary artery, distal ligation alone or proximal and distal ligation/division with or without closure from within the aneurysmal coronary artery [7, 9, 10]. Surgical ligation presents a very low mortality rate (1.4%) [8]. It could be achieved with or without CPB [7, 9, 10]. The main concern is the long-term results. Cheung et al. [9] in a series of 41 patients operated on for CAVF, showed that 80.5% of the patients were asymptomatic in the follow-up (range 5.08 months to 28.09 years). Despite this good clinical result, the authors showed a recurrence rate of 19%, the latter being more important in the external ligation/division group as compared to the intracardiac closure group (22% vs. 16%, respectively). When surgical correction of bilateral fistulas to the pulmonary artery is undertaken, as in the present case, we recommend routine exploration of the pulmonary artery under CPB to avoid the technical difficulty of approaching the friable vascular network and the omission of a communication in the vascular malformation, which can cause late recurrence.
In summary, bilateral coronary arteriovenous fistulas are rare congenital anomalies, when they coexist with other cardiopathies they should be treated surgically and the closure should be achieved from within the pulmonary artery.
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References
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- Dodge-Khatami A, Mavroudis C, Backer CL. Congenital heart surgery nomenclature and database project: anomalies of the coronary arteries. Ann Thorac Surg 2000;69:S270–297.[Abstract/Free Full Text]
- Gillebert C, Van Hoof R, Van de Werf F, Piessens J, De Geest H. Coronary artery fistulas in an adult population. Eur Heart J 1986;7:437–443.[Abstract/Free Full Text]
- Angelini P, Velasco JA, Flamm S. Coronary anomalies: incidence, pathophysiology, and clinical relevance. Circulation 2002;105:2449–2454.[Free Full Text]
- Levin DC, Fellows KE, Abrams HL. Hemodynamically significant primary anomalies of the coronary arteries. Angiographic aspects. Circulation 1978;58:25–34.[Abstract/Free Full Text]
- Baim DS, Kline H, Silverman JF. Bilateral coronary artery — pulmonary artery fistulas. Report of five cases and review of the literature. Circulation 1982;65:810–815.[Free Full Text]
- 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;59:849–854.[Abstract/Free Full Text]
- Lowe JE, Oldham HN Jr, Sabiston DC Jr. Surgical management of congenital coronary artery fistulas. Ann Surg 1981;194:373–380.[Medline]
- Armsby LR, Keane JF, Sherwood MC, Forbess JM, Perry SB, Lock JE. Management of coronary artery fistulae. Patient selection and results of transcatheter closure. J Am Coll Cardiol 2002;39:1026–1032.[Abstract/Free Full Text]
- Cheung DL, Au WK, Cheung HH, Chiu CS, Lee WT. Coronary artery fistulas: long-term results of surgical correction. Ann Thorac Surg 2001;71:190–195.[Abstract/Free Full Text]
- Olearchyk AS, Runk DM, Alavi M, Grosso MA. Congenital bilateral coronary-to-pulmonary artery fistulas. Ann Thorac Surg 1997;64:233–235.[Abstract/Free Full Text]
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Abstract
1. Introduction