Brian Buxton, M.D., Rogelio Ventimeglia, M.D.,. George J. Reul, M.D., and Denton A. Cooley, M.D.. The widespread use of coronary arteriography has led to the ...
CONGENITAL CORONARY-TO-PULMONARY ARTERY PISTULA: DIAGNOSIS AND TREATMENT Brian Buxton, M.D., Rogelio Ventimeglia, M.D., George J. Reul, M.D., and Denton A. Cooley, M.D.
The widespread use of coronary arteriography has led to the discovery of increasing numbers of simple coronary artery fistulae, as well as fistulae associated with coronary artery occlusive disease.' Because coronary artery-to-pulmonary artery fistulae are so rare, accounting for only 20 percent of all coronary artery fistulae,2 our recent experiences with the surgical treatment of six patients is presented. (Patients with an anomalous origin of a coronary artery from the pulmonary artery were excluded, although they may have had flow into the pulmonary artery from the coronary system.)
MATERIALS AND METHODS Six patients with a coronary-to-pulmonary artery fistula underwent surgical treatment in this institution between January 1973 and August 1975. The mean age of the four females and two males was 54.5 years (see Table). Four patients presented primarily with chest pain, one with paroxysmal atrial tachycardia and one with cardiac failure. In the four patients with an uncomplicated fistula, the electrocardiogram was normal or nearly normal. In two patients who had coronary artery occlusive disease in addition to the coronary-to-pulmonary artery fistula, the electrocardiogram showed evidence of a previous infarction. Results of plain chest roentgenography were normal in all patients. Cardiac catheterization, performed in three patients, revealed normal left and right heart pressures; and in none could a left-to-right shunt be demonstrated by oximetry. Coronary angiography revealed diagnostic features in all patients. In three, the fistula arose from the left coronary system and in one from the right coronary system, while in two the fistula originated from both coronary arteries (Fig. 1.). Extensive coronary artery occlusive disease was present in two patients. After surgical correction, one of the patients was found to have hemiplegia, presumably the result of embolism at the time of operation. The remaining five did not experience any serious complications. Follow-up data were gathered for a mean of 16 months (range three-to-28 months). The condition of four patients who had a simple coronary-to-pulmonary artery fistula was improved after surgery. One was free of symptoms, two were almost free from chest pain, and the one who had presented with cardiac failure experienced less fatigue and greater exercise tolerance. From the Division of Surgery of the Texas Heart Institute, St. Luke's Episcopal and Texas Children's Hospitals, Houston, Texas. Address for reprints: Denton A. Cooley, M.D.. Texas Heart Institute, P.O. Box 20345, Houston, Texas 77025. 202
Cardiovascular Diseases, Bulletin of the Texas Heart Institute, Vol. 3, Number 2, 1976
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The two patients who had concomitant coronary-to-pulmonary artery fistula and coronary artery occlusive disease were completely free of symptoms after surgical treatment of the double lesion. OPERATIVE TREATMENT
All patients were treated surgically using cardiopulmonary bypass. The opening in the pulmonary artery was identified by incising the main pulmonary artery longitudinally along the anterior surface (Fig. 2). A single ostium was identified in five patients, and two accessory openings were present in the pulmonary artery of one. The position of the pulmonary ostium was constant and was located approximately 1-2 cm above the left anterior leaflet of the pulmonary valve; the appearance and position were similar to a normal coronary artery orifice. The termination of the fistula could be easily identified by releasing the aortic clamp, thus permitting blood to emerge from the orifice in the pulmonary artery, which was then oversewn with Dacron sutures. When possible, the abnormal vessels were ligated on the surface of the main pulmonary artery. However, identifying
Anomalous artery excised Fig. 2. Diagram showing stages of obliteration of a left coronary-to-main pulmonary artery fistula: a - Identification of fistula on surface of main pulmonary artery. b- Oversewing of internal opening. c- Closure of pulmonary artery with excision of fistulous connection between the main pulmonary artery and the left coronary artery vein bypass. 205
the vessel on the surface of the pulmonary artery was usually more difficult than finding the internal aperture, particularly when there was a cirsoid malformation. Also, some fistulae had multiple connections with the coronary circulation, thus making simple ligation on the surface of the pulmonary artery difficult and possibly unreliable.
DICUSSION
Coronary-to-pulmonary artery fistulae are usually congenital in origin. Edwards3 classified them into two types: primary fistulae, in which the fistula is the main pathological lesion; and secondary fistulae, which occur as a consequence of other cardiac malformations, e.g., aortic or pulmonary atresia. The secondary type occurs less frequently, and for the purpose of this report, only primary fistulae have been considered. The fistula may originate from either the right or left coronary artery, both coronary vessels, or a single coronary artery.4 The abnormal vessel may arise from the coronary artery at any level, although our findings are that most originate proximally. The fistula may be terminal or arise as a lateral branch from a coronary artery. In this series of coronary-topulmonary artery fistulae, the left coronary artery was the site of origin more frequently than the right, a finding similar to that of McNamara et al.2 and Sakakibara et al.5 However, when a fistula terminated in the right ventricle or atrium, the right coronary artery was involved more often than the left.6 The fistula may be a single vessel, while in other instances it is plexiform, consisting of tortuous vessels which terminate either singly or in multiple sites in the pulmonary artery.2 The coronary artery from which the fistula originates is sometimes tortuous, dilated or aneurysmal.7'8 No associated congenital abnormalities were observed in our six patients, although two had acquired coronary artery occlusive vascular disease. The hemodynamic effect of the left-to-right shunt is related to the size of the shunt and the pulmonary vascular resistance. In the three patients who underwent cardiac catheterization, no shunt could be detected from the catheterization data. A mild-to-moderate increase in pulmonary pressure has been reported in patients with coronary fistulae terminating at the right side of the heart, but severe pulmonary hypertension is rare.2 The presence of the fistula may lead to a decreased amount of blood perfusing the myocardium which may result in angina, myocardial infarction, or papillary muscle dysfunction.1'9-1' Clinically, patients with a coronary artery-to-pulmonary artery fistula may be asymptomatic, or present with angina pectoris or cardiac failure. In this series, pain was the most common complaint. In the two patients who had a fistula and coronary artery occlusive disease, difficulty was encountered in determining which lesion was responsible for the symptoms. A continuous murmur generally is present, although this was detected in only three of six patients and its location was along the left sternal border.5 The location of the murmur at the apex and accentuation of the diastolic component are important characteristics of coronary artery fistulae.'2 The diastolic murmur and systolic click in one patient (see Table) may have represented mitral valvular dysfunction secondary to 206
papillary muscle damage. Differential diagnoses should include aorticopulmonary window, patent ductus arteriosus, ventricular septal defect with aortic insufficiency and a ruptured aneurysm of the sinus of Valsalva. Chest roentgenograms and electrocardiographic findings are nonspecific determinants. Selective coronary angiography is the most accurate diagnostic tool and will usually demonstrate evidence of the origin, course, size and termination of the fistula. Also, selective angiography is valuable in detecting associated arteriosclerotic lesions of the coronary arteries (present in two patients). The widespread use of coronary angiography will probably result in the recognition of more patients with simple coronaryto-pulmonary fistula as well as one complicated by coronary occlusive disease. A ventriculogram may be useful if left ventricular or papillary muscle dysfunction is suspected. Surgical treatment is usually indicated in patients with severe symptoms, uncontrollable by medical treatment. However, the method of treating asymptomatic patients with coronary artery fistulae is more controversial. Some physicians think that these patients should be treated surgically because there is a risk of developing congestive cardiac failure, myocardial ischemia, bacterial endocarditis, or rupture of the fistula.5 Others think that a conservative approach should be adopted when the shunt is trivial, and that operation should be undertaken only for patients who have developed a complication."3"14 When a fistula is associated with coronary artery occlusive disease, correction of both lesions would seem appropriate in patients with significant symptoms or complications. The goal of surgical treatment of a coronary-to-pulmonary artery fistula is permanent closure of the fistula with preservation of the myocardial circulation.'5 The surgical approach depends upon the anatomical configuration, e.g., whether the fistula is a single vessel or is comprised of multiple vessels, the accessibility of the abnormal vessel, and the origin. Knowledge of the number of openings in the pulmonary artery and the anatomy of the coronary vessels is also desirable. Perhaps of even greater importance in planning the surgical procedure is the discovery of coronary artery occlusive disease associated with a congenital coronary-to-pulmonary artery fistula. Under these circumstances, we believed it best to correct both lesions concomitantly. A fistula between a coronary artery and a pulmonary artery was first ligated on the surface of the pulmonary artery by Biorck and Crafoord in 1947.1' The technique reported was unreliable7 because the fistula was not always single and the vessel was not always easily identified.'2 Swan et al.18 first described the surgical technique of closing the internal orifice of a coronary artery-to-right ventricular fistula under direct vision with the aid of circulatory arrest. A modification of this procedure using cardiopulmonary bypass has the advantage of allowing positive identification of the fistula by opening the pulmonary artery. We prefer this technique when multiple sites of origin exist, when there is a cirsoid malformation on the surface of the pulmonary artery, or when there is more than one internal opening in the pulmonary artery. In patients who have coronary artery occlusive disease and a fistula, the addition of a coronary artery vein bypass permits correction of associated coronary artery occlusive disease.4"9'20 207
SUMMARY Six patients with a coronary-to-pulmonary artery fistula underwent surgical treatment at this institution between January 1973 and August 1975. Four patients presented with angina pectoris, one with paroxysmal atrial fibrillation and one with congestive cardiac failure. Results of coronary angiography revealed that the fistula arose from the left coronary system in three patients, from the right coronary system in one, and from both coronary systems in two. All fistulae terminated in the main pulmonary artery just distal to the pulmonary valve. Two patients had severe coronary artery disease associated with a fistula. In all patients the fistula was oversewn from within the pulmonary artery in addition to ligation of the fistulous vessel on the surface of the pulmonary artery or heart, employing cardiopulmonary bypass. Two patients underwent concomitant aortocoronary artery saphenous vein bypass. Follow-up data revealed that two patients were improved and the remaining four were free of symptoms.
ADDENDUM Two additional patients with a coronary-to-pulmonary artery fistula have been operated on at this institution. One, a 57-year-old woman had experienced three episodes of severe chest pain. The results of electrocardiography were normal. Coronary angiography revealed a fistula from the left anterior descending coronary artery to the main pulmonary artery. The patient underwent surgical correction using cardiopulmonary bypass in August, 1975; however, she died after operation from a neurological complication. The second patient, a 45-year-old man, presented with chest pain, a normal electrocardiogram, and a fistula from the left anterior descending coronary artery to the main pulmonary artery. He underwent successful surgical correction on January, 1976, and was relieved of all symptoms. REFERENCES 1. Delaye J, Janin A, Clermont A, Dupont J, Amiel M, Perrin A, Gonin A: Shunt, Gauchedroite par des branches des arteres coronaries droite et gauche communiquant avec l'artere pulmonaire. Coeur et Medicine Int 12:659-667, 1973 2. McNamara JJ, Gross RE: Congenital coronary fistula. Surgery 65:59-69, 1969 3. Edwards JE: Editorial. Anomalous coronary arteries with special reference to arterio-venous like communications. Circulation 17: 1001, 1958 4. Hallman GL, Cooley DA, McNamara DG, Latson JR: Single leift coronary artery with fistula to right ventricle. Circulation 32: 293-297, 1965 5. Sakakibara S, Yokoyama M, Takao A, Nogi M, Gomi H: Coronary arteriovenous fistulanine operated cases. Am Heart J, 72:307-314, 1966 6. de Nef JJE, Varghese PJ, Losekoot G: Congenital artery fistula. Br Heart J 33:857-862, 1971 7. Neufeld HN, Lester RG, Adams P Jr., Anderson RC, Lillehei CW, Edwards JE: Congenital communication of a coronary artery with a cardiac chamber on the pulmonary trunk. Circula-
tion 24:171-179, 1961 8. Cooley DA, Ellis PR: Surgical consideration of coronary arterial fistulae. Am J Cardiol
10:467-494, 1962
9. Effler DB, Sheldon WC, Turner JJ, Groves LK: Coronary arteriovenous fistulas: Diagnosis and surgical management. Report of 15 cases. Surgery 61:41-50, 1967
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10. Morgan JR, Forker AD, O'Sullivan MJ, Fosburg RG: Coronary arterial fistulas. Seven cases with unusual features. Am J Cardiol 30:432-436, 1972 11. Lee GB, Gobel FL, Lillehei CW, Neff WS, Eliot RS: Correction of shunt from right conal coronary artery to pulmonary trunk with relief of symptoms. Circulation 37:244-248, 1968 12. Roos JP, Hartman H, Van der Schaar H, Brom AG: Diagnosis and surgical treatment of coronary artery fistula. Thorax 25:259-266, 1970 13. Humblet L, Delvign J, Kilbertus H, Colligan P, Jorns H: Arteriovenous fistula involving both coronary arteries and main pulmonary trunk. Br. Heart J 31:136-139, 1969 14. Sasse F: Surgery for coronary artery fistula. Chest 68:848-849, 1975 15. Li,tta D, Hallman GL, Cooley DA: Surgical treatment of congenital coronary artery fistula. Surgery 70, 856-864, 1971 16. Biorck G, Crafoord C: Arteriovenous aneurysm on the pulmonary artery simulating ductus arteriosus Botalli. Thorax 2:65-74, 1947 17. El Said GM, Dawson JT, Sandiford FM, Mullens CE, Hallman GL, Cooley DA, McNamara DG: Coronary artery anomalies: Diagnosis, indications, and results of surgical management. European J Cardiol 1:63-70, 1973 18. Swan H, Wilson JN, Woodwark G, Blount SG: Surgical obliteration of a coronary artery fistula to right ventricle. Arch Surg 79:820, 1959 19. FavaloTo RG: Saphenous vein graft in the surgical treatment of coronary artery disease: Operative technique. J Thorac Cardiovasc Surg 58:178-185, 1969 20. Cooley DA, Hallman GL, Bloodwell RD: Definitive surgical treatment of anomalous origin of left coronary artery from pulmonary artery: Indications and results. J Thorac Cardiovasc Surg 52:798-808, 1966
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