Ann Thorac Surg 1997;64:233-235
© 1997 The Society of Thoracic Surgeons
Case Report
Congenital Bilateral Coronary-to-Pulmonary Artery Fistulas
Andrew S. Olearchyk, MD,
Dianne M. Runk, MD,
Mosen Alavi, MD,
Michael A. Grosso, MD
Sections of Cardiothoracic Surgery and Cardiology, Episcopal Hospital, Philadelphia, Pennsylvania
Accepted for publication February 11, 1997.
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Abstract
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Rare, congenital bilateral coronary-to-pulmonary artery fistulas in an older woman, one originating from the proximal right coronary artery and the other from a distal left main coronary artery and draining to the proximal main pulmonary artery, were successfully dissected, identified, and ligated proximally and also closed distally on a beating heart with cardiopulmonary bypass standby.
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Introduction
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In 1865, Krause [1] described a congenital coronary arteriovenous (AV) fistula, which is a part of congenital anomalies of coronary arteries, which occur in 1% to 2% of the population [2]. Such fistulas comprise 48.7% of all congenital anomalies of the coronary arteries. Of all such fistulas, the coronary-to-pulmonary artery fistula (CPAF) constitutes 15% to 30% [3]. Multiple fistulas occur in 10.7% to 16% of all coronary AV fistulas, whereas both coronary arteries are involved in about 5% [2–4]. The origin of the CPAF from the left main coronary artery (LMCA), and its bilaterality, as found in our patient, are very unusual.
A 60-year-old woman complained of a progressive dyspnea on effort, orthopnea, and paroxysmal nocturnal dyspnea of 1 year's duration. There was a continuous murmur over the upper anterior mediastinum. Chest roentgenography showed a cardiothoracic ratio of 0.55, electrocardiography showed normal sinus rhythm with left-axis deviation, and transthoracic echocardiography showed a continuing shunt between the ascending aorta and the main pulmonary artery (PA). Cardiac catheterization and coronary angiography on November 21, 1996 (Figs 1–3
) revealed bilateral CPAFs. One fistula originated from the very proximal right coronary artery and the other, extremely tortuous, from a distal portion of a markedly dilated entire LMCA, and formed an extensive malformation in front of the main PA with multiple entries into it. The right coronary artery, left anterior descending artery, and circumflex artery distal to the fistula were normal in caliber. There was a step-up of oxygen saturation between the right ventricle and the PA of 6%.
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Fig 1. . Cardiac catheterization and coronary angiography of the right coronary artery in the right anterior oblique projection. It shows one of two coronary-to-pulmonary artery fistulas: this one originated from the very proximal right coronary artery and ascended up toward the anterior aspect of the main pulmonary artery with an entry into it.
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On November 27, through a median sternotomy incision with cardiopulmonary bypass standby, and after a longitudinal opening of the pericardium, the bilateral CPAF was identified (Fig 4) and a continuous thrill was palpated over it. Both fistulas were dissected and identified at their origin from the proximal right coronary artery and distal LMCA, encircled with 2-0 silk, and temporarily occluded with tourniquets until the thrill disappeared for 10 minutes. There were no electrocardiographic changes, and cardiac hemodynamics were normal. Then, the fistulas were double ligated proximally with a 2-0 silk suture and their entries into the PA were closed with two pledgeted 4-0 Prolene (Ethicon, Somerville, NJ) sutures. The patient is doing well.
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Fig 4. . Operative photograph after a median sternotomy and longitudinal opening of the pericardium showing bilateral coronary-to- pulmonary artery fistulas. One fistula originated from the very proximal right coronary artery and the other, tortuous, from the distal left main coronary artery. Both fistulas ascended toward the anterior aspect of the main pulmonary artery and formed an extensive malformation with entries into that artery.
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Comment
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In the coronary AV fistula, the site of origin of the fistula is from the right coronary artery in 50% to 58%, from the left anterior descending artery in 25%, from the circumflex artery in 18.3%, from the diagonal branch in 1.9%, and from the LMCA or circumflex-marginal branch in 0.7% each [2, 3, 5]. The coronary AV fistula drainage site was the PA in 29.8% to 43%, the right ventricle in 14% to 40%, the right atrium in 19% to 20.2%, the left ventricle in 5.8% to 19%, and the left atrium in 5%. Twenty percent of patients with congenital coronary AV fistula have other congenital or acquired heart disease [2–4,6]. In adults, 60% complained of fatigue and dyspnea on effort. Angina pectoris and myocardial infarction, due to coronary artery steal, occur in 3% to 7%. Congestive heart failure develops in 19% of patients, and endocarditis in the fistula in 20%. It may rupture spontaneously, causing hemopericardium and cardiac tamponade [5]. Surgical techniques include internal closure of the fistula from within the PA or a cardiac chamber (46.1%), tangential arteriorrhaphy for lateral fistulas (28.8%), distal ligation alone (11.5%), proximal and distal ligations (6.7%), ligation and coronary artery bypass grafting (3.8%), and closure from within the aneurysmal coronary artery (2.9%) [2, 4, 5]. Complex fistulas require cardiopulmonary bypass and cardioplegic arrest [3].
In our patient the CPAF was complex and bilateral, and one of the fistulas originated from the LMCA. But we were able to dissect, identify, and ligate the origin of both fistulas from the coronary arteries, and close their entries into the PA on a beating heart.
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Fig 2. . Selective injection of dye into the left main coronary artery in the right anterior oblique projection shows a markedly enlarged left main coronary artery with a dilated and tortuous second fistula originating from its distal portion, passing toward the anterior aspect of the pulmonary artery, where it formed an extensive malformation with an entry into that artery. The left anterior descending artery and circumflex artery were of normal diameter and not diseased.
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Fig 3. . Selective injection of dye into the left main coronary artery in the left anterior oblique projection, showing a coronary-to-pulmonary artery fistula between the left main coronary artery and the anterior aspect of the main pulmonary artery with an extensive malformation entering that artery.
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Footnotes
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Address reprint requests to Dr Olearchyk, 129 Walt Whitman Blvd, Cherry Hill, NJ 08003.
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References
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- Krause W. Ueber den Ursprung einer akzessorischen A. coronaria aus der A. pulmonalis. Z Ratl Med 1865;24:225–9.
- Fernandes ED, Kadivar H, Hallmann GJ, et al. Congenital malformations of the coronary arteries: the Texas Heart Institute experience. Ann Thorac Surg 1992;54:732–40.[Abstract]
- Kirklin JW, Barratt-Boyes BG. Congenital anomalies of the coronary arteries. In: Kirklin JW, Barratt-Boyes BG, eds. Cardiac surgery. New York: Churchill Livingstone, 1993:1167–93.
- Bauer EP, Piepho A, Klovekorn WP. Coronary arteriovenous fistula: surgical correction of a rare form. Thorac Cardiovasc Surg 1994;42:237–9.[Medline]
- Bauer HH, Allmendinger PD, Flaherty J, et al. Congenital coronary arteriovenous fistula: spontaneous rupture and cardiac tamponade. Ann Thorac Surg 1996;62:1521–3.[Abstract/Free Full Text]
- Tai YT, Fong PC, Chow WH. Bilateral coronary-artery-to-pulmonary artery fistula coexistent with apical hypertrophic cardiomyopathy-a case report. Angiology 1992;43:72–5.[Abstract/Free Full Text]
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Abstract
Introduction
