The Japanese Journal of Surgery (1992) 22:176-179
SURGERYTODAY © Springer-Verlag 1992
Surgical Management of the Coronary Artery to Pulmonary Artery Fistulas; a Case of a Large Ruptured Aneurysm YOSHIYAISHIKURA,SHIGETOHODAGIRI,AKIRASHIMAZU,DAIGOHIRAO,HIROYUKIWATANABEand KOUICHIYANO The Department of Thoracic and Cardiovascular Surgery, School of Medicine, University of Occupational & Environmental Health, Fukuoka, Japan
patients were treated for coronary arterypulmonary artery fistula. Each was asymptomatic due to a coronary artery fistula. There was one instance each of myocardial infarction, mitral stenosis and a large closed ruptured aneurysm including a thrombus. All the fistulas were comprised of several small plexiform arranged vessels. The left to right shunt ratio was approximately 8 per cent or less. A surgical operation was performed to close the drainage orifice of the fistulas through pulmonary arteriotomy under cardiopulmonary bypass in two patients while one patient underwent a ligation of a large abnormal vessel to the aneurysm followed by a resection of the aneurysm without cardiopulmonary bypass. A large saccular aneurysm of such fistulas is rare and a rupture of such an aneurysm is even more rare. The surgical management of coronary artery fistulas is also discussed herein.
coronary artery fistula, plexiform arrangement of vessels, saccular large aneurysm, ruptured aneurysm
Introduction Congenital coronary artery fistulas are relatively rare, such as in the case of an abnormal origin of the coronary artery or a fistula to the low pressure cardiac chambers. 1'2 Recently fistulas in the form of plexiform vessels (one of the coronary artery fistulas) are being occasionaly found by routine coronary angiography. 3'4 This type fistula without other associated heart disease is usually asymptomatic, and difficult to find. A large saccular aneurysm of these fistulas is rare and rupture of the aneurysm is even more rare. This paper discusses Reprint requests to: Yoshiya Ishikura, MD, The Department of Thoracic and Cardiovascular Surgery, School of Medicine, University of Occupational & Environmental Health, Japan., 1-1 Iseigaoka Yahatanishi-ku Kit~kyushu city, Fukuoka, Japan (Received for publication on Sep. 22, 1990).
the surgical management of coronary artery to pulmonary artery fistulas of this type in three patients.
MaterialsandResults In three patients, a coronary artery to pulmonary artery fistulas was noted by diagnostic coronary arteriography. Their clinical data are presented in Table I. Patient No.1 was a 59 year old man admitted due to severe chest pain and was diagnosed as inferior myocardial infarction. H e had a Levine grade II continuous murmur in the left second intercostal space at the left sternal border. Patient No.2 was a 37 year old woman admitted with complaints of exertional dyspnea and palpitation according to mitral stenosis. Patient No.3 was a 62 year. old woman admitted owing to a mediastinal tumor of increasing size, but which was asymptomatic (Fig. 1). The E C G was normal in two patients (Patient No.2,3); while one patient showed inferior myocardial infarction (Patient No.l). Pulmonary wedge pressure was normal (less than 12 mmHg) in 2 patients (Patient No.l,3) and abnormal (18mmHg) in the one with mitral stenosis (Patient No.2). Cardiac catheterization in two patients indicated about an 8 per cent left to right shunt ratio at pulmonary artery (Patient No.l,2), while the o t h e r patient (No.3) showed an unclear shunt. By coronary angiography, one patient showed severe atherosclerotic stenosis at the right coronary and left anterior descending artery (Patient N o . l ) . The coronary artery to the pulmonary artery fistulas was managed for three fistulas, one originating from the left anterior descending artery (Patient N o . l ) , one from the left anterior descending artery and circumflex artery (Patient No.3) (Fig. 2), and one from the proximal right coronary artery and left anterior descending artery (Patient No.2). All the fistulas were composed of several small plexiform arranged vessels. There was no proximal dilatation of the
Y. Ishikura et al. : The Coronary Artery to Pulmonary Artery Fistulas Table 1. Patient profiles. Fistula Case
large aneurysm (closed rupture)
closure drainage via MPA, ACB, CPB. closure drainage via MPA, OMC, CPB. aneurysmectomy, division of afferent artery, non-CPB
L A D + RC
L A D + CX
LADi left anterior descending artery, RC: right coronary artery, CX: circumflex artery, MPA: main pulmonary artery, MI: myocardial infarction, MS: mitral stenosis, ACB: Aorto-coronary Bypass, OMC: open mitral commissurotomy, CPB: Cardiopulmonary bypass
Fig. 1. Case No.3; chest X-ray
Fig. 2. Same case; Left coronary arteriography showing a large saccular aneurysrn (large arrow) in the tumor shadow (small arrow)
Y. Ishikura et al. : The Coronary Artery to Pulmonary Artery Fistulas to the main pulmonary artery. The large tumor with the aneurysm, whose surface assumed cyanostic like appearance similar to hematoma, was resected from the main pulmonary artery and right ventricular outflow myocardium without a cardiopulmonary bypass (Fig. 4). The pathological findings of the tumor indicated mainly thrombus and aneurysm with wall destruction. Both hematoma and hemorrhage were found under the epicardium and outside the aneurysm. By postoperative angiography, two patients (No. 1,2) were judged to show no further signs of the original fistulas, but one patient (No.3) showed very small residual fistula into the pulmonary artery. For all patients, the postoperative course was uneventfull.
Fig, 3. Same case; CT showing a tumor (arrow) resembling a thrombus at the antero-left-lateral portion of main pulmonary artery
Fig. 4. Same case; The aneurysm was excised and opened. The tumor contained a large thrombus and a hematoma was noted outside the aneurysm and under the epicardium
donor vessels, and contrast material was clearly seen to penetrated the main pulmonary artery in all cases. In patient No.3 a saccular large aneurysm and thrombuslike-tumor was found in addition to the fistula by DSA, CT and MRI (Fig. 3). In patient No.l, the two orifices of the fistulas were closed via the pulmonary artery and A-C bypass grafting to the left anterior descending artery. In patient No.2, one orifice of the fistulas was closed via a pulmonary arteriotomy and an open mitral commisurotomy. In patient No.3, a left posterolateral thoracotomy was made in the 5th intercostal space, and a slightly bloody effusion was found moderately in the pericardial space. He underwent ligation of a large abnormal vessel to the tumor and two abnormal vessels
Coronary artery fistulas are relatively rare 1'2 and are of the following three types; the first is of abnormal origin in the coronary artery, with a common origin of the pulmonary artery, the second is a fistula in the low pressure right cardiac chambers, and the third is a plexiform arrangement of fistulas in the pulmonary artery. I-4 The incidence of the third type of fistula numbered only two cases out of the 15 patients of all coronary artery fistulas by Effter's report, 4 and two in 12 patients by Iskandrian's report. 3 But this type of coronary artery fistula may well be more common than would be indicated from the reports. Coronary fistulas may exist without clinical manifestations and can be detected during diagnostic coronary arteriography. 1-4 The present cases were associated with either significant obstructive coronary artery disease, mitral stenosis or a large saccular aneurysm such as a mediastinal tumor. Many patients with only coronary artery fistulas are asymptomatic, and diagnosis can be made based on a continuous cardiac murmur. ~-s Two of our patients had a slightly continuous murmur, while the other was asymptomatic with only an increasing tumor shadow of the left mediastinum in the chest X-ray. The left to right shunt flow of the third type fistulas is usually low. However, in the case of a large shunt flow (QP/QS > 2.0) there are sometimes symptoms of heart failure. 5'6'8 Pulmonary hypertension has been described but is quite unusual. 6 Some patients have chest pain which is often interpreted as angina pectoris by coronary steal phenomenon. 3-5' 7-9 One of the present patients with myocardial infarction was found to have severe coronary artery stenosis at the periphery portio n of the fistel vessels, which possibly indicated coronary steal phenomenon. 4'1°'11 Subacute bacterial endocarditis has been reported in approximately 3.3 per cent of patients with coronary artery-cardiac chamber fistulas. 5'7'8 An abnormal coronary artery with fistula is usually quite
Y. Ishikura et al. : The Coronary Artery to Pulmonary Artery Fistulas dilated, but a large saccular a n e u r y s m of the a b n o r m a l c o r o n a r y artery is rare, 5'6'12'13 and a rupture of the a n e u r y s m prior to operation is even m o r e rare.14'15 T h e a n e u r y s m of our patient showed a large thrombus, and there was a h e m o r r h a g e outside the aneurysmal wall and u n d e r the epicardium. It appears reasonable to consider that the a n e u r y s m was caused by a closed rupture. Fistulas originate most c o m m o n l y f r o m the right c o r o n a r y artery. 6'7'9 In our patients, two fistulas originated from the left c o r o n a r y artery, and one f r o m both c o r o n a r y arteries. T h e c o r o n a r y arteries originate as an endothelial growth at the base of the aorta and c o m m u n i c a t e with the capillary n e t w o r k on the surface of the heart. Indication for surgical operation are as follows; a large shunt flow and s y m p t o m s of heart failure, subacute bacterial endocarditis, angina pectoris by c o r o n a r y steal p h e n o m e n o n , myocardial infarction and p u l m o n a r y hypertension as well as c o r o n a r y a n e u r y s m f o r m a t i o n with subsequent rupture or embolization. 2-9 T h e m e t h o d of operation for small fistula is generally direct ligation without a cardiop u l m o n a r y bypass or, at least, with a c a r d i o p u l m o n a r y bypass on stand-by. 2'4-9 H o w e v e r , multiple fistulas such as seen in this study involved a difficult ligation of the involved c o r o n a r y artery on both sides of the fistula. T h e drainage orifices of the fistulas were closed via a p u l m o n a r y a r t e r i o t o m y utilizing a cardiop u l m o n a r y bypass in two patients. While, in the other patient with a large aneurysm, we ligated the a b n o r m a l vessel into the a n e u r y s m and resected the a n e u r y s m without a c a r d i o p u l m o n a r y bypass, yet with the bypass on stand-by. O u r experience indicates that a diffusely dilated vessel can return to n o r m a l size after a successful closure of the fistula. T h e wall of the a b n o r m a l vessels is dilated and friable. Thus, surgical t r e a t m e n t can be c o n d u c t e d in patients without adverse symptoms to prevent a r u p t u r e of the a n e u r y s m or dilated vessels.
References 1. Ogden JA (1970) Congenital anomalies of the coronary artery. Am J Cardiol 25:474-9 2. MacNamara JJ, Gross RE (1969) Congenital coronary artery fistula. Surgery 65:59-69 3. Iskandrian AS, Kimbiris D, Bemis CE, Segal BL (1978) Coronary artery to pulmonary artery fistulas. Am Heart J 96: 605-9 4. Effler DB, Sheldon WC, Turner JJ, Groves LK (1967) Coronary arteriovenous fistulas: Diagnosis and surgical management. Report of fifteen cases. Surgery 61:41-50 5. Rittenhouse EA, Doty DB, Ehrenhaft JL (1975) Congenital coronary artery-cardiac chamber fistula. Ann Thoracic Surg 20:468-485 6. Oldham HN, Jr., Ebert PA, Young WG, Sabiston DC, Jr (1971) Surgical management of congenital coronary artery fistula. Ann Thoracic Surg 12:503-513 7. Lowe JE, Oldham HN, Jr., Sabiston DC, Jr (1981) Surgical management of congenital coronary artery fistula. Ann Surg 194:373-380 8. Liberthson RR, Sagar K, Berkoben JP, Weintraub RM, Levine FH (1979) Congenital coronary arteriovenous fistula. Report of 13 patients, review of the literature and delineation of management. Circulation 59:849-854 9. Urrutia-S CO, Falaschi G, Ott DA, Cooley DA (1983) Surgical management of 56 patients with congenital coronary artery fistulas. Ann Thoracic Surg 35:300-307 10. Phillips PA, Libanoff AJ (1974) Arteriovenous communication associated with obstructive arteriosclerotic coronary artery disease and myocardial infarction. Chest 65:106-108 11. Zalman F, Andia AM, Wu K, Moores WY, Hoit B, Maisel AS (1987) Atherosclerotic coronary artery aneurysm progressing to coronary artery fistula: Presentation as myocardial infarction with continuous murmur. Am Heart J 114:427-429 12. Kalke B, Edwards JE (1969) Localized aneurysms of the coronary arteries. Angiology 19:460-470 13. Watanabe Y, Ohteki H, Minato N, Sakurai J, Natsuaki M, Itoh T (1989) A successful surgical treatment for left coronary arterypulmonary artery fistula with giant saccular aneurysm. JJATS 37:1206-1211 14. Soma Y, Higashi S, Suzuki S, lwai F, Kawada K, Inoue T (1985) A ruptured aneurysm of circumflex artery accompanying coronary artery fistula. JJATS 33:2245-2248 15. Habermann JH, Howard ML, Johnson ES (1963) Rupture of the coronary sinus with hemopericardium. A rare complication of coronary arteriovenous fistula. Circulation 28:1143-1144