Ann Thorac Surg 1995;60:202-204
© 1995 The Society of Thoracic Surgeons
Case Reports
Coronary Artery Fistulas Presenting With Bacterial Endocarditis
Abdul M. Alkhulaifi, FRCS,
Simon M. Horner, MRCP,
Wilf B. Pugsley, FRCS,
R. Howard Swanton, FRCP
Departments of Cardiothoracic Surgery and Cardiology, The Middlesex Hospital, London, United Kingdom
Accepted for publication December 8, 1994.
 |
Abstract
|
|---|
Coronary artery fistulas are rare congenital malformations. Two cases presenting with bacterial endocarditis are described. Both were treated successfully by grafting of the coronary artery and ligation of the fistula.
|
Introduction
|
|---|
Coronary artery fistulas are rare congenital malformations, and in the majority of instances the patients are asymptomatic. Presentation with bacterial endocarditis has been reported previously in the literature [1]. We report on 2 patients with coronary artery fistula who presented with bacterial endocarditis.
|
Case Reports
|
|---|
Patient 1
A 41-year-old man presented with tiredness and an enlarging cardiac shadow. He had had infective endocarditis, caused by Streptococcus viridans, 13 years previously, which was assumed to be on his mitral valve. He was treated with intravenous antibiotics for 8 weeks.
He remained asymptomatic but his heart size increased on chest roentgenograms from 16.9/34 cm to 19.7/34 cm over a 2-year period, and he was admitted for cardiac catheterization.
He was taking amiodarone, 200 mg on alternate days for atrial fibrillation, amiloride, 5 mg, and hydrochlorthiazide, 50 mg. On examination he appeared well; there were signs of mild mitral regurgitation and a continuous murmur at the right sternal edge.
Cardiac catheterization revealed a large right coronary artery fistula communicating with the coronary sinus (Fig 1
) with a shunt ratio of 1.4:1. The left ventricle was dilated with good systolic function and a minor degree of mitral regurgitation. He was treated for bacterial endocarditis with intravenous antibiotics, and antifailure therapy was commenced.
View larger version (111K):
[in this window]
[in a new window]
|
Fig 1. . (A) Right anterior oblique view of the coronary artery fistula with an explanatory line drawing below. (B) Left anterior oblique view of the coronary artery fistula with an explanatory line drawing below.
|
|
At operation a grossly enlarged and aneurysmal right coronary artery was found to occupy the region between the ascending aorta, the right atrium, and the right ventricle. Cardiopulmonary bypass was instituted, the aorta and the fistulous right coronary artery were clamped, and cold cardioplegia solution (St. Thomas') was injected into the aortic root. The fistula was occluded to prevent cardioplegia solution from escaping into the coronary sinus. Cardioplegia also was injected into the distal right coronary artery. The fistula was found to extend for 4 cm, after which it expanded into an aneurysmal sac 5 cm in diameter. From the inferior aspect of the sac arose the distal segment of a normal right coronary artery. From the superior aspect of the sac the fistula continued upward toward the roof of the right atrium and then backward and inferiorly between the right atrium and aorta to open into the coronary sinus. The right coronary artery was grafted using a saphenous vein graft. The proximal end of the fistula was traced into the aorta and closed. The fistula and aneurysmal sac were laid open and secured in that position.
Patient 2
A 72-year-old woman presented with nocturnal sweats, malaise, and loss of weight associated with pyrexia of 38°C. Laboratory investigations revealed anemia (hemoglobin level, 9.6 g/dL) and an erythrocyte sedimentation rate of 95 mm/h. On examination she had a regular pulse rate of 80 beats/min and a blood pressure of 140/80 mm Hg. She also had a loud diastolic murmur in the aortic area and evidence of cardiomegaly. Blood cultures (six cultures) confirmed positive growth of Streptococcus viridans. Chest radiograph showed a cardiothoracic ratio of 16/24.5 cm. Cardiac catheterization revealed a dilated left ventricle with a normal-sized aortic root, no aortic regurgitation, normal cardiac valves, and a giant coronary fistula arising at the origin of the circumflex coronary artery draining into the left atrium. The left to right shunt was only 1.2:1. The left anterior descending coronary artery was normal. The patient was treated with intravenous antibiotics and her infective endocarditis was cured.
At operation, with cardiopulmonary bypass and cardioplegic (St. Thomas') cardiac arrest, there was a dilated and tortuous circumflex coronary artery that drained directly into the coronary sinus. Two normal-looking obtuse marginal branches were seen to arise from the fistulous circumflex coronary artery. The fistula was oversewn and the obtuse marginal branches were grafted using saphenous vein conduit.
|
Comment
|
|---|
Congenital coronary artery fistulas are rare arterial malformations, the first case being described in 1865 [2]. The majority of cases (55%) are asymptomatic at the time of presentation [1]. Because the underlying pathophysiology is that of a left to a right shunt, it is not surprising that the most common clinical presentation is that of congestive cardiac failure [3]. Although bacterial endocarditis of the fistulous tract has been reported in the literature [1], this mode of clinical presentation remains rare. In the 2 patients we present here, bacterial endocarditis was the diagnosis at the time of presentation; however, symptoms and signs of congestive cardiac failure were evident in the first case. The development of bacterial endocarditis has been shown to be enhanced by the presence of congenital cardiac malformation [4]. Predisposing factors to bacterial colonization of either defective valve or, as in these 2 patients, a coronary artery fistula include high pressure flow across the defect and the presence of roughened endothelium. We have noted during operation that the endothelial lining of the fistulous tract in patient 1 was grossly roughened with evidence of atheromatous disease. Whether this represents a secondary change due to bacterial infection or a change resulting from flow turbulence inside the fistula, therefore unrelated to infection, remains unknown.
We have isolated Streptococcus viridans as the organism responsible for infection in the 2 patients we have studied. Streptococci account for the majority of cases of endocarditis, with Streptococcus viridans being the most common, particularly when congenital malformations are the underlying cause for bacterial endocarditis [5, 6].
The usual form of correction is direct ligation, with a mortality of 2% [7]; it is recommended that ligation should be performed at the point of entry into the cardiac chamber [7] to avoid the development of myocardial ischemia. Direct ligation of the fistulous tract in the 2 patients we operated on was not possible without compromising distal coronary blood flow to the myocardium. We, therefore, elected to graft the distal coronary vessels using saphenous vein conduit to ensure optimal coronary blood flow.
We report here 2 cases of coronary artery fistula presenting with a rare complication, namely, bacterial endocarditis. After antibiotic therapy to eliminate the infection both cases were treated successfully by closure of the fistula and bypass grafting to the affected coronary vessels.
|
Footnotes
|
|---|
Address reprint requests to Dr Alkhulaifi, Department of Cardiothoracic Surgery, The Middlesex Hospital, Mortimer St, London W1N 8AA, United Kingdom.
|
References
|
|---|
- Lowe JE, Oldham HN, Sabiston DC. Surgical management of congenital coronary artery fistulas. Ann Surg 1981;194:373–80.[Medline]
- Krause W. Ztschr Rationelle Med 24, 1865.
- Liberthson RR, Saga K, Berkohen JP, Weintraub RM, Levine FH. Congenital coronary artery fistula. Circulation 1979;59:849–54.[Abstract/Free Full Text]
- McKinsey DS, Ratts TE, Bismo AL. Underlying cardiac lesions in adults with infective endocarditis: the changing spectrum. Am J Med 1987;82:681–6.[Medline]
- Schollin J, Bjarke B, Wesstrom G. Infective endocarditis in Swedish children. I. Incidence, etiology, underlying factors and port of entery of infection. II. Location, major complications, laboratory findings, delay of treatment, treatment and outcome. 1986;75:993–9.
- Geva T, Frand M. Infective endocarditis in children with congenital heart disease: the changing spectrum 1965–1985. Eur Heart J 1988;9:1244–7.[Abstract/Free Full Text]
- Rittenhouse EA, Doty DB, Ehrenhaft JL. Congenital coronary artery–cardiac chamber fistula. Ann Thorac Surg 1975;20: 468–85.[Abstract]
This article has been cited by other articles:
|
|
|
|
 
J-M Schleich, C Rey, M Gewillig, and A Bozio
Spontaneous closure of congenital coronary artery fistulas
Heart,
April 1, 2001;
85(4):
6e - 6.
[Abstract]
[Full Text]
|
|
|
Top
Abstract
Introduction
