Ann Thorac Surg 1996;61:1520-1523
© 1996 The Society of Thoracic Surgeons
Case Report
Successful Repair of Coronary Artery-Coronary Sinus Fistula With Aneurysm in an Adult
Tomio Abe, MD,
Koji Kamata, MD,
Katsuhiko Nakanishi, MD,
Kiyofumi Morishita, MD,
Sakuzo Komatsu, MD
Department of Thoracic and Cardiovascular Surgery, Sapporo Medical University, School of Medicine, Sapporo, Japan
Accepted for publication October 24, 1995.
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Abstract
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We report a very rare case of an adult with coronary artery fistula and aneurysm formation. This fistula was successfully closed with direct suture closures by opening the aneurysm under complete cardiopulmonary bypass. The distal terminated orifice of the fistula, which drained to the coronary sinus, was also closed. Finally, aneurysmorrhaphy with overlapping mattress sutures was performed. The postoperative angiographic study demonstrated normal coronary artery distribution, and the patient was asymptomatic without recurrence at 2 years after the operation.
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Introduction
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Communications between a coronary artery and a chamber of the heart are uncommon congenital diseases, and coronary artery fistula with coronary artery aneurysm drained to the coronary sinus in an adult is very rare [1]. We describe a congenital case of right coronary fistula with coronary artery aneurysm drained to the coronary sinus in a 52-year old woman, who was treated successfully. The cause and indications for operative repair are discussed.
The patient was a 52-year-old woman admitted with a history of palpitations and easy fatigue of increasing severity. She had not pointed out her cardiac murmur before admission and had been doing well. The chest roentgenogram and electrocardiogram were within normal limits. Cardiac catheterization studies confirmed the presence of a left-to-right shunt of 10% at the right atrial level, with a pulmonary-to-systemic flow ratio of 1.2:1. Selective right coronary angiography revealed a grossly dilated, tortuous right coronary artery with a large coronary artery aneurysm; the distal fistula entered the right atrium through the coronary sinus (Fig 1
). The remainder of the coronary arteries appeared normal. The left ventricle was constructed well.
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Fig 1. . Preoperative right coronary artery (RCA) injection showing a dilated coronary artery and a large aneurysm of the right coronary artery. Drainage was to the coronary sinus and right atrium (RA). The black arrow indicates the origin of the fistula in the right coronary artery.
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On August 27, 1993, the patient underwent operation. A vertical sternotomy was done. The right coronary artery with the large dilated coronary artery aneurysm was visible, with pulsation and a size of 30 x 25 x 25 mm in diameter (Fig 2). The dilated vessel ran from the atrioventricular groove to the diaphragmatic surface of the heart. Using complete cardiopulmonary bypass, we induced cardiac arrest with cold blood cardioplegia. The aneurysm then was opened to see the location and size of the fistula. The aneurysm measured 4 x 2 mm in diameter without inflammatory evidence, and was closed using 7-0 monofilament sutures. The orifice of the terminated fistula measured 5 x 4 mm in diameter; we recognized that it drained to the coronary sinus by inserting an instrument under the right atriotomy incision. This distal fistula was closed using a 4-0 monofilament sutures. Finally, the aneurysm was closed with interrupted sutures. The discontinuation of cardiopulmonary bypass was uneventful, and recovery was smooth with no complications. Postoperative selective coronary angiography before discharge showed no abnormal communications and good filling of the peripheral branches of the right coronary artery (Fig 3).
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Fig 2. . Posterior wall of the coronary aneurysm measures 30 x 25 x 25 mm in diameter (A). The arrow points to the termination in the coronary sinus, which measured 5 x 4 mm in diameter (B). The proximal entry or orifice of the aneurysm was a slightly dilated vessel of good quality and normal-appearing intima. The size of the orifice was 3 x 2 mm in diameter. The distal branches were of normal size (C).
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Fig 3. . Right coronary artery (RCA) injection 1 month after operation. There were no abnormal communications between the right coronary artery and the coronary sinus and no aneurysm formation on the right coronary artery.
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Comment
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Congenital anomalies of the coronary arterial system are uncommon lesions. One of the common congenital anomalies of the coronary circulation is a fistula between a coronary artery and one of the chambers of the heart. The incidence of this anomaly is reported as 1% to 2% of all congenital heart diseases [2]. Communication between a right coronary artery and a coronary sinus is rare [3, 4]. Fernandes and associates [4] reported 104 cases of congenital coronary artery fistula; only 2 cases (1.9%) had communication with the left coronary arteries and coronary sinus. Few reports are available in the literature [5] on pathologic arteriovenous communications with coronary aneurysms between distributed coronary arteries and the right side of the heart or coronary veins. Schultz [1] reported 14 cases of true arteriovenous coronary aneurysms, and most of these lesions were congenital anomalies. In embryonic life, the coronary arteries communicate with the veins through an ordinary capillary network. In addition, the arteries give off branches to the intratrabecular spaces, the sinusoids, which in turn communicate with the cavities of the ventricles. Later, the sinusoids shrink into a normally calibrated capillary network, and the communication with the cavities of the cavities of the heart is transformed into tubesian veins. The majority of reported aneurysms seen in childhood have been secondary to a coronary artery fistula with shunt, and the exact cause factor is still unclear.
In the present case, the entry or orifice of the coronary artery fistula was relatively small, and the shunt flow ratio from left to right was 10%. Therefore, the aneurysm size did not increase rapidly but had been developing gradually. There was also no evidence of inflammation, and the aneurysm was not located at an arterial bifurcation. The patient had no clinical evidence of atherosclerosis in other vessels and no history suggestive of collagen vascular disease or trauma. The clinical findings associated with this fistula are extremely variable, depending upon the chamber of the heart involved and the size of the ostium leading into the chamber. In this case, the left-to-right shunt was small, the patient was entirely asymptomatic, and a continuous murmur was the only finding suggesting the diagnosis. Liberthson and colleagues [6] noted that 80% of patients with coronary artery fistula at ages of 20 years or less were asymptomatic. However, Oldham and associates [3] reported that among 150 patients with isolated fistula, 55% had symptoms. Congestive heart failure was seen in 14%, most commonly presenting in either the first year of life or after the age of 20 years.
In addition to clinical manifestations of congestive heart failure, myocardial ischemia, and infective endocarditis, the occurrence of aneurysms of the involved coronary arteries [1, 5], rupture of an aneurysm [7], and pulmonary hypertension have been reported with this anomaly. Because of these serious complications, surgical treatment of the fistula is recommended. The first successful ligation of a congenital coronary artery fistula was performed in 1947 by Björk and Crafoord [8]. The procedure initially used was ligation of the involved coronary artery on both sides of the fistula without cardiopulmonary bypass; however, a high incidence of myocardial ishemia and infarction resulted. More recently, improved selective coronary angiographic techniques have made possible careful preoperative determination of the size, location, and extent of the fistula.
After precise diagnosis of the fistula preoperatively, we used cardiopulmonary bypass and myocardial protection with cold blood cardioplegia. In this case of coronary sinus communication, the site of entry was so far posterior that it was inaccessible without the use of bypass. Closure of the fistula at its entry and its termination into the coronary aneurysm with cardiopulmonary bypass was successful, with no evidence of myocardial ischemia in this case. The overall results after operative ligation of coronary artery-cardiac chamber fistula have been very good. The 3.6% incidence of postoperative myocardial infarction in the patients reviewed may be reduced by proper placement of sutures to close the fistula and to avoid vessels supplying the myocardium. Residual or recurrent fistulas have been mentioned in the literature, but the true incidence is unknown.
In conclusion, we report a very rare case of an adult with right coronary artery fistula with coronary aneurysm draining to the coronary sinus. The patient survived the operation without postoperative complications and with no recurrence at 2 years.
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Footnotes
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Address reprint requests to Dr Abe, Department of Thoracic and Cardiovascular Surgery, Sapporo Medical University, South 1, West 17, Chuo-ku, Sapporo, Hokkaida, 060 Japan.
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References
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- Schultz J. Coronary arteriovenous aneurysm: review of the literature. Am Heart J 1958:56:431–42.
- Engel HJ, Torres C, Page HL Jr. Major variations in anatomical origin of the coronary arteries. Angiographic observations in 4,250 patients without associated congenital heart disease. Cathet Cardiovasc Diagn 1975;1:157–69.[Medline]
- Oldham HN, Ebert PA, Young WG, Sabiston DC. Surgical management of congenital coronary artery fistula. Ann Thorac Surg 1971;12:503–11.
- 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]
- Harris A, Jefferson K, Chatterjee K. Coronary arterio-venous fistula with aneurysm of coronary sinus. Br Heart J 1969;31:400–3.[Free Full Text]
- Liberthson RR, Sagar K, Berkoben JP, et al. 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]
- Haberman JH, Howard ML, Johnson ES. Rupture of the coronary sinus with hemopericardium, a rare complication of coronary A-V fistula. Circulation 1963;28:1143.[Abstract/Free Full Text]
- Björk G, Crafoord C. Arteriovenous aneurysm in the pulmonary artery simulating patent ductus arteriosus Botalli. Thorax 1947;2:265–70.
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Abstract
Introduction
