Catheterization and Cardiovascular Diagnosis 26:19-25 (1992)
Coronary Arteriovenous Fistula Presenting as Congestive Heart Failure Aaron D. Kugelmass, MD, Warren J. Manning, MD, Robert N. Piana, MD, Ronald M. Weintraub, MD, Donald S. Baim, MD, FSCAI, and William Grossman, MD Coronary arteriovenous fistulae are rare, but can be of hemodynamic significance. We report a circumflex-coronary sinus fistula in an elderly man which was associated with a loud continuous murmur and congestive heart failure. Noninvasive evaluation, Including transesophageal echocardiography and magnetic resonance Imaging, suggested the diagnosis. Angiographlc diagnosis of concomitant atherosclerotic coronary disease may be difficult, and can require postoperative study. 8 iwz wiiey-uss, I ~ C . Key words: coronary sinus fistula, transesophageal echocardiography, magnetic resonance imaging, atherosclerosis, postoperative coronary angiography
INTRODUCTION Coronary arteriovenous fistulae, precapillary communications between a coronary artery and a cardiac vein, chamber or great vessel, represent less than 1% of congenital cardiac anomalies [ I]. Of the more than 400 cases reported in the literature, almost all are congenital, with coronary arteriovenous fistulae resulting from trauma having been noted rarely [2,3]. Comprising 14% of anomalous coronary circulation, these fistulae represent the most common hemodynamically significant coronary anomaly [ 1,4]. Overall, coronary arteriovenous fistulae are quite rare and recent retrospective analyses of large coronary angiographic series have established an incidence of 0. I-0.2% in patients undergoing coronary angiography [33 1 . In this report we describe a case of a coronary arteriovenous fistula with a large left-to-right shunt presenting with congestive heart failure and a loud continuous murmur. Current diagnostic and therapeutic considerations are discussed.
CASE HISTORY A.S., a 72-year-old, physically active and healthy white man presented with progressive exertional dyspnea, chest pressure, and palpitations. These symptoms would abate with rest. Marked escalation of these symptoms, along with light headedness caused the patient to seek medical attention. The diagnosis of new onset atrial fibrillation prompted hospitalization. The past medical history included hypertension and hypercholesterolemia. A systolic murmur had been detected 14 years earlier, and reportedly was unchanged. The patient’s identical 0 1992 Wiley-Liss, Inc.
twin brother had undergone coronary artery bypass grafting (CABG) for severe three vessel atherosclerotic coronary artery disease 6 months earlier. There was no family history of congenital heart disease. Physical exam disclosed jugular venous distention and lateral displacement of the cardiac impulse. A continuous III/VI blowing murmur with systolic accentuation was appreciated across the precordium, but was loudest over the left infrascapular region. The electrocardiogram showed atrial fibrillation, right bundle branch block, and left ventricular hypertrophy. Cardiomegaly , with normal heart borders and perivascular haziness, was apparent on chest X-ray. Transthoracic echocardiography was notable for mild left ventricular hypertrophy with right ventricular and bilateral atrial dilatation. The coronary sinus was markedly enlarged, with Doppler study revealing turbulent flow. The left main coronary artery was markedly dilated to 1 cm in diameter, with continuous flow by color Doppler. From various views, single plane transesophageal echocardiography (Fig. 1) also showed aneurysmal dilatation of the left main and left circumflex coronary arteries and demonstrated high velocity flow into a 4 cm
From the Charles A. Dana Research Institute and the HarvardThorndike Laboratory of the Departments of Medicine (Cardiovascular Division), and Surgery, Beth Israel Hospital and Harvard Medical School, Boston, Massachusetts.
Received August 24, 1991; revision accepted December 18, 1991. Address reprint requests to William Grossman, MD, Cardiovascular Division, Beth Israel Hospital, 330 Brookline Avenue, Boston, MA 02215.
Fig. 1.
Coronary Arteriovenous Fistula
21
Fig. 2. Magnetic resonance imaging. (a) Demonstrates aneurysmal left main (LM)artery at the ostium in relation to the descending aorta (DA). (b) The open arrow defines the dilated circumflex artery (CX) in relation to the main pulmonary artery (PA). (c) The dilated venous lake (VL) is evident. (d) The closed arrow Identifies the dilated coronary sinus.
venous lake at the level of the AV groove, which drained into the dilated coronary sinus. Magnetic resonance imaging (Fig. 2) was performed with a 1.5 Tesla clinical system (Siemen’s Magnetom, Iselin, NJ). ECG-gated, multiplane, multiphase spinecho imaging was accomplished using TR of 47 msec and TE of 8 msec. A slice thickness of 5 mm with an interslice distance of 6 mm and a field-of-view of 350 X 350 mm were used. This confirmed right ventricular enlargement and also identified a dilated left main coronary artery and aneurysmal left circumflex coronary artery
which communicated directly to a posterior cavity that drained into the coronary sinus. Cardiac catheterization revealed elevated right and left heart filling pressures and a significant 0, step-up in the right atrium with calculated pulmonary (Q,)and systemic (Q,) flows of 7.3 and 3.8 litedmin, respectively, with a QJQ, = 1.9/1. Hemodynamic findings are summarized in Table I. Left ventriculography showed normal wall motion with a left ventricular ejection fraction of 52%. Manual coronary angiography revealed an atretic, nondominant right coronary artery. The left coronary artery was markedly dilated with poor visualization because of extremely high coronary flow. Accordingly, power injection of 40 ml at 10 ml/sec (Fig. 3) was perFig. 1. Transesophageal echocardiography. (a) Demonstrates formed through an 8F angioplasty guiding catheter and aneurysmal dilatation of the left main (LM) and circumflex (CX) demonstrated markedly dilated left main and circumflex arteries at the aortic ostlum (AO). (b) The closed arrow indicates arteries with brisk flow from the distal circumflex artery the fistula’s venous lake in relation to the enlarged left atrium into a smooth, dilated venous lake that subsequently (LA). (c) The closed arrow points to the dilated coronary sinus drained into a markedly enlarged coronary sinus. High within the right atrium (RA) at the level of the tricuspid valve fistulous flow limited full visualization of the distal epi(Tv) and right ventricle (RV).
22
Kugelmass et al. TABLE 1. Hemodynamic Findings at Cardiac Catheterization A . Oximetry and Pressure Measurement
O2saturation(%) Superior vena cava Inferior vena cava Right atrium Right ventricle Pulmonary artery Pulmonary capillary wedge Left ventricle Aorta
Preop
Postop
52 69 81 74 73
61 66 61 62 63
-
-
92
96
Pressures (mm Hg) a,v,(mean) Preop -/I74 14) 54/ 13 54/26/(37) 4 2 2 42 I ) I15/17 I I5/75/(92)
Postop -
I1/10/(8) 401 I2 40/19/(27) 14/ 14/(14) -
206/93/(122)
B . Oxygen Consumption and Blood Flow
Oxygen consumption index (liters/min/m') Pulmonary blood flow (literslmin) Systemic blood flow (liters/min)
QdQ,
Preop
Postop
I26 7.3 3.8 I .9
I25 4.6 4.6
1 .o
Fig. 3. Coronary angiography. In (a) (RAO caudal projection) the open arrows indicate the dilated left main and circumflex arteries, and the closed arrow identifies the proximal left anterior descending artery, which fills faintly. In (b) (lateral projection) the closed arrow demonstrates an aneurysmal proximal circumflex artery, while the open arrow indicates the left anterior descending artery, which is poorly visualized.
cardial coronary branches. We considered, but rejected, sinus. A large tortuous circumflex artery drained into a the option of repeating coronary injections during bal- large, partially calcified venous lake. On inspection, the loon occlusion of the circumflex fistula in lieu of repeat- remaining coronary arteries appeared normal. Upon opening the venous lake, the entrance of the circumflex ing coronary angiography postoperatively. Five days following cardiac catheterization the patient artery and exit to the coronary sinus were identified and underwent surgical correction of the fistula. Under car- each was closed with Teflon-pledget bolstered interdiopulmonary bypass, a small persistent left superior rupted sutures. The venous lake was then trimmed and vena cava was noted to drain into the dilated coronary obliterated with a running suture. Intraoperative trans-
Coronary Arteriovenous Fistula
23
stenoses of the proximal and middle left anterior descending artery.
DISCUSSION
Fig. 4. Coronary angiogram following fistula ligation (lateral projection). The open arrow indicates serial stenoses of the left anterior descending artery, which can now be visualized. Closed arrow delineates the total occlusion of the middle left circumflex. The arrowhead demonstrates the residual dilatation of the proximal circumflex.
esophageal Doppler echocardiography , immediately following cessation of cardiopulmonary bypass, showed no further fistulous flow. Upon return from the operating room, the patient’s continuous murmur had disappeared. He converted to sinus rhythm spontaneously on the second postoperative day. Perioperatively there was an elevation of CPK MB isoenzymes, 1063 IU/liter, 13% MB. A radionuclide ventriculogram revealed new posterolateral hypokinesis and inferobasal akinesis, consistent with a myocardial infarction. However, the patient felt markedly improved, with increased exercise tolerance. One month following surgery the patient underwent repeat cardiac catheterization to confirm fistula correction and better evaluate his coronary anatomy in a state of physiologic coronary flow. Hemodynamic evaluation indicated that right and left heart filling pressures were now nearly normal (Table I). A right heart oximetry study documented no persistent shunt. Coronary angiography (Fig. 4), which now allowed opacification of distal vessels, revealed persistent aneurysmal dilatation of the left main and proximal circumflex arteries. The proximal circumflex contained small amounts of intraluminal thrombus, and there was interim total occlusion of the circumflex distal to a large obtuse marginal. Distal marginal vessels received collateral flow from the proximal vessel. Additionally, there were sequential, high grade
Most coronary arteriovenous fistulae are believed to arise from the incomplete obliteration of primitive myocardial sinusoids [6,7]. This developmental arrest results in retained continuity between the mature coronary artery and cardiac vein or chamber. A slight majority of coronary arteriovenous fistulae arise from the right coronary artery [ 1,4,8-lo]. Origins from single or bilateral coronary arteries are rare. Most fistulae terminate in the right ventricle or atrium. Coronary arteriovenous fistulae ending in the pulmonary artery, left atrium or ventricle are less common [1,4,8-lo]. Fifty seven previous cases of coronary artery-coronary sinus fistulae have been reported [2,9,11-191. Coronary arteriovenous fistula may be detected at any age, with the reported age of diagnosis spanning 10 days to 84 years [4]. There is an equal sex distribution. Concurrent congenital cardiac anomalies, including patent ductus arteriosus, ventricular septal defect, atrial septal defect, and persistent superior left vena cava are present in approximately twenty percent of cases [2,20,21]. At diagnosis, one-half of patients are symptomatic, commonly children with large systemic to pulmonary shunts and congestive heart failure, or patients older than 40 years. Most frequent symptoms include dyspnea on exertion, fatigue, angina, as well as recurrent respiratory infections and endocarditis. Angina is often seen in older patients, occuring in up to 80% of patients over 50 years in one series [22]. This is attributed to the natural concurrence of atherosclerotic coronary artery disease. Additionally, myocardial ischemia due to coronary stenoses is believed to be exacerbated by coronary steal that results from high fistulous flow, as has been demonstrated by intracoronary flow measurements during surgical correction of an arteriovenous fistula [23]. Congestive heart failure presumably results from chronic ventricular volume overload in patients with substantial left-to-right shunts, which can be as large as Q,,/Q,=3.1/1 [24]. Atrial arrythmias, primarily fibrillation, are common in coronary arteriovenous fistulae with increased atrial flow. Infectious endocarditis is not uncommon, with a reported incidence of up to 12% [2]. Aneurysmal coronary dilatation is commonplace in symptomatic coronary arteriovenous fistulae. Vessel fibroelastosis has resulted in aneurysm diameters as large as 12 cm [ 141. Postoperative catheterization in adults has revealed little resolution of dilated vessels, and thrombosis may occur [25]. Despite thin aneurysm walls, only one case of spontaneous rupture has been reported [ 161.
24
Kugelmass et al.
Spontaneous closure is rare, having been reported in four cases [25-281. Diagnosis hinges on consideration of the entity. The continuous murmur, as in the present case, is suggestive of coronary arteriovenous fistulae. Chest X-ray and electrocardiogram are nonspecific. Noninvasive imaging has proven valuable in the diagnosis of coronary arteriovenous fistulae. Two-dimensional echocardiography is useful in assessing aneurysmal fistulae [29]. Smaller fistulae may be appreciated by coronary turbulence identified by color Doppler [30]. To our knowledge, this is the first report of evaluation of a coronary arteriovenous fistula by transesophageal echocardiography. This modality allowed definition of the fistula course. Additionally, it was employed intraoperatively to confirm effective fistula ligation. Magnetic resonance imaging has been utilized in four reported cases, and also provides excellent definition of the fistula course [31-331. These modalities are complementary to cardiac catheterization and coronary angiography in the evaluation of coronary arteriovenous fistulae and may assist in the detection of associated congenital anomalies. Selective coronary angiography establishes the fistula course and represents the standard for diagnosis. As this case illustrates, a large coronary arteriovenous fistula may limit visualization of coronary anatomy, which in the adult may obscure atherosclerotic stenoses. Compromised flow to other vessels because of coronary steal may potentiate this problem. Conversely, overestimation of the severity of concomitant atherosclerotic obstructions has resulted from coronary steal due to a coronary arteriovenous fistula [34]. Definitive therapy consists of surgical closure of the fistula, which may be accomplished without extracorporeal support in uncomplicated cases. Cardiopulmonary bypass is employed in situations with difficult access, intramuscular correction, or associated coronary artery bypass graft. Traditionally, operative treatment has been reserved for symptomatic patients. However, some authors argue that elective correction of fistulae in younger, asymptomatic patients can prevent late sequelae and reduce the increased surgical morbidity and mortality observed in older, symptomatic patients [ 12,351. SUMMARY
Coronary arteriovenous fistula is a rare, but clinically important coronary anomaly. Previous reports have documented the efficacy of transthoracic echocardiography and magnetic resonance imaging in diagnosing coronary arteriovenous fistulae. In this communication we have also described the diagnostic and intraoperative utility of transesophageal echocardiography in evaluating this condition. Cardiac catheterization with selective coronary
angiography is crucial for diagnosis and planning surgical management. This case further illustrates that in adult cases of coronary arteriovenous fistulae with a large coronary aneurysm or high fistulous flow, adequate visualization of myocardial branches may be difficult. Postoperative coronary angiography may be needed to assess the presence and extent of coronary atherosclerosis.
REFERENCES 1. Levin DC,Fellows KE, Abrams HL: Hemodynamically significant primary anomalies of the coronary arteries. Circulation 58: 25-34. 1978. 2. Ogden JA, Stansel HC: Coronary arterial fistulas terminating in the coronary venous system. J Thorac Cardiovasc Surg 63:172182, 1972. 3. Gillebert C, Van Hoof R, Van De Wed F, Piessens J , De Geest H: Coronary artery fistulas in the adult population. Eur Heart J 7:437-443, 1986. 4. Wilde P, Watt 1: Congenital coronary artery fistulae: Six new cases with a collective review. Clin Radio1 31:301-311, 1980. 5 . Yamanaka 0, Hobbs, RE: Coronary artery anaomalies in 126,595 patients undergoing coronary arteriography . Cathet Cardiovasc Diagn 21:28-40, 1990. 6. Grant RT: Development of the cardiac coronary vessels in the rabbit. Heart 13:261-271, 1926. 7. Grant RT: An unusual anomoly of the coronary vessels in the malformed heart of a child. Heart 13:273-283, 1926. 8. Sakakibara S, Yokoyama M, Takao A, Nogi M, Gomi H: Coronary arteriovenous fistula. Am Heart J 72:307-313, 1966. 9. Horiuchi T, Abe T, Tanaka S , Koyamada K: Congenital coronary arteriovenous fistulas. Ann Thorac Surg 11:102-112, 1971. 10. McNamara JJ, Gross RE: Congenital coronary artery fistula. Surgery 65:59-69, 1969. I I . Kimbiris D, Kasparian H, Knibbe P, Brest AN: Coronary arterycoronary sinus fistula. Am J Cardiol 26532-539. 1970. 12. Wellens F, Deuvaert F, Leclerc JL, Primo G: Coronary artery fistula: an absolute surgical indication. Acta Chir Belg 84:339344, 1984. 13. Come PC.Riley MF, Fortuin NJ: Echocardiographic mimicry of pericardial effusion. Am J Cardiol 47:365-370, 1981. 14. Kourouclis C, Athanasiadas D, Georgiou V, Andritsakis G, Avgoutstakis D: Right coronary artery to coronary sinus communication simulating pericardial effusion. Cardiology 63:47-52, 1978. 15. Gasul BM, Arcilla RA, Fell EH, Lynfield J , Bicoff JP, Luan LL: Congenital coronary arteriovenous fistula. Pediatrics 2553 1-560, 1960. 16. Habermann JH, Howard MI, Johnson ES: Rupture of the coronary sinus with hemopericardium. Circulation 28:1143-1144, 1963. 17. Croom RD, Wilcox BR, Abney RL: Right coronary artery-coronary sinus arteriovenous fistula. Ann Thorac Surg 4:182-188, 1967. 18. Hams A, Jefferson K, Chatterjee K: Coronary arteriovenous fistula with aneurysm of the coronary sinus. Br Heart J 3 1 :400-403. 1969. 19. Case Records of the Massachusetts General Hospital: N Engl J Med 315:1533-1543, 1986. 20, Didichen H, Skallberg L, Cappelen CH: Congenital coronary artery fistula. Thorax 21:121-128. 1966. 21, Oldham HN, Ebert PA, Young WG, Sabiston DC: Surgical man-
Coronary Arteriovenous Fistula
22.
23. 24. 25.
26. 27.
28. 29.
agement of coronary artery fistula. Ann Thorac Surg 12503-513, 1971. Effler DB, Sheldon WC, Turner JJ, Groves LK: Coronary arteriovenous fistulas: Diagnosis and surgical management. Surgery 61:41-50, 1967. Hudspeth AS, Linder JH: Congenital coronary arteriovenous fistula. Arch Surg 96:832-835, 1968. Ludomirsky A , O’Laughlin MP, Reul GJ, Mullins CE: Congenital aneurysm of the right coronary artery with fistulous connection to the right atrium. Am Heart 1 119:672-675. 1990. Jaffe RB, Glancy DL, Epstein SE, Brown BG, Morrow AG: Coronary arterial-right hean fistulae. Circulation 47: 133-143, 1973. Morgan JR. Forker AD, O’Sullivan MI. Fosburg RG: Coronary artrial fistulas. Am J Cardiol 30:432-436, 1972. Griffiths SP. Ellis K, Hordof AJ, Martin E , Levine OR: Spontaneous complete closure of a congenital coronary artery fistula. J Am Coll Cardiol 2:1169-1173. 1983. Hackett D, Hallidie-Smith KA: Spontaneous closure of coronary artery fistula. Br Heart J 52:477-479, 1984. Reeder GS, Tajik AJ, Smith HC: Visualization of coronary artery fistula by two-dimensional echocardiography. Mayo Clin Proc 55:185-189, 1980.
25
30. Fukuda K. Handa S, Ohnishi S, Nakamura Y:Noninvasive evaluation of right coronary artery-right atrial fistula using two-dimensional echocardiography , pulsed Doppler echocardiography and color flow mapping. Cardiology 75:375-380, 1988. 31. Boxer RA, LaCorte MA, Singh S, Ishmael R, Cooper R, Stein H: Noninvasive diagnosis of congenital left coronary artery to right ventricle fistula by nuclear magnetic resonance imaging. Pediatr Cardiol 10:45-47. 1989. 32. Pucillo AL, Schecter AG, Moggio RA, Kay RH. Baum SJ, Herman MV: MR imaging of coronary artery anomalies. J Comput Assist Tomogr 14(2):171-174, 1990. 33. Zhang CC, Hu LX, Guo DW, Ling ML: Two giant congenital aneurysms of the left anterior descending coronary artery with right ventricle fistula: Diagnosis based on two-dimensional echocardiography and magnetic resonance imaging. J C U 18: 601-605, 1990. 34. Deglin SM, Hurst VW, Hammond G, Kelley MJ: Coronary arteriovenous fistula associated with atherosclerotic disease: a problem in angiographic interpretation. Conn Med:768-769, 1978. 35. Liberthson RR, Sagar K, Berkoben JP, Weintraub RM, Levine FH: Congenital coronary artery fistula. Circulation 59:849-854, 1979.
Coronary Arteriovenous Fistula Presenting as Congestive Heart Failure Aaron D. Kugelmass, MD, Warren J. Manning, MD, Robert N. Piana, MD, Ronald M. Weintraub, MD, Donald S. Baim, MD, FSCAI, and William Grossman, MD Coronary arteriovenous fistulae are rare, but can be of hemodynamic significance. We report a circumflex-coronary sinus fistula in an elderly man which was associated with a loud continuous murmur and congestive heart failure. Noninvasive evaluation, Including transesophageal echocardiography and magnetic resonance Imaging, suggested the diagnosis. Angiographlc diagnosis of concomitant atherosclerotic coronary disease may be difficult, and can require postoperative study. 8 iwz wiiey-uss, I ~ C . Key words: coronary sinus fistula, transesophageal echocardiography, magnetic resonance imaging, atherosclerosis, postoperative coronary angiography
INTRODUCTION Coronary arteriovenous fistulae, precapillary communications between a coronary artery and a cardiac vein, chamber or great vessel, represent less than 1% of congenital cardiac anomalies [ I]. Of the more than 400 cases reported in the literature, almost all are congenital, with coronary arteriovenous fistulae resulting from trauma having been noted rarely [2,3]. Comprising 14% of anomalous coronary circulation, these fistulae represent the most common hemodynamically significant coronary anomaly [ 1,4]. Overall, coronary arteriovenous fistulae are quite rare and recent retrospective analyses of large coronary angiographic series have established an incidence of 0. I-0.2% in patients undergoing coronary angiography [33 1 . In this report we describe a case of a coronary arteriovenous fistula with a large left-to-right shunt presenting with congestive heart failure and a loud continuous murmur. Current diagnostic and therapeutic considerations are discussed.
CASE HISTORY A.S., a 72-year-old, physically active and healthy white man presented with progressive exertional dyspnea, chest pressure, and palpitations. These symptoms would abate with rest. Marked escalation of these symptoms, along with light headedness caused the patient to seek medical attention. The diagnosis of new onset atrial fibrillation prompted hospitalization. The past medical history included hypertension and hypercholesterolemia. A systolic murmur had been detected 14 years earlier, and reportedly was unchanged. The patient’s identical 0 1992 Wiley-Liss, Inc.
twin brother had undergone coronary artery bypass grafting (CABG) for severe three vessel atherosclerotic coronary artery disease 6 months earlier. There was no family history of congenital heart disease. Physical exam disclosed jugular venous distention and lateral displacement of the cardiac impulse. A continuous III/VI blowing murmur with systolic accentuation was appreciated across the precordium, but was loudest over the left infrascapular region. The electrocardiogram showed atrial fibrillation, right bundle branch block, and left ventricular hypertrophy. Cardiomegaly , with normal heart borders and perivascular haziness, was apparent on chest X-ray. Transthoracic echocardiography was notable for mild left ventricular hypertrophy with right ventricular and bilateral atrial dilatation. The coronary sinus was markedly enlarged, with Doppler study revealing turbulent flow. The left main coronary artery was markedly dilated to 1 cm in diameter, with continuous flow by color Doppler. From various views, single plane transesophageal echocardiography (Fig. 1) also showed aneurysmal dilatation of the left main and left circumflex coronary arteries and demonstrated high velocity flow into a 4 cm
From the Charles A. Dana Research Institute and the HarvardThorndike Laboratory of the Departments of Medicine (Cardiovascular Division), and Surgery, Beth Israel Hospital and Harvard Medical School, Boston, Massachusetts.
Received August 24, 1991; revision accepted December 18, 1991. Address reprint requests to William Grossman, MD, Cardiovascular Division, Beth Israel Hospital, 330 Brookline Avenue, Boston, MA 02215.
Fig. 1.
Coronary Arteriovenous Fistula
21
Fig. 2. Magnetic resonance imaging. (a) Demonstrates aneurysmal left main (LM)artery at the ostium in relation to the descending aorta (DA). (b) The open arrow defines the dilated circumflex artery (CX) in relation to the main pulmonary artery (PA). (c) The dilated venous lake (VL) is evident. (d) The closed arrow Identifies the dilated coronary sinus.
venous lake at the level of the AV groove, which drained into the dilated coronary sinus. Magnetic resonance imaging (Fig. 2) was performed with a 1.5 Tesla clinical system (Siemen’s Magnetom, Iselin, NJ). ECG-gated, multiplane, multiphase spinecho imaging was accomplished using TR of 47 msec and TE of 8 msec. A slice thickness of 5 mm with an interslice distance of 6 mm and a field-of-view of 350 X 350 mm were used. This confirmed right ventricular enlargement and also identified a dilated left main coronary artery and aneurysmal left circumflex coronary artery
which communicated directly to a posterior cavity that drained into the coronary sinus. Cardiac catheterization revealed elevated right and left heart filling pressures and a significant 0, step-up in the right atrium with calculated pulmonary (Q,)and systemic (Q,) flows of 7.3 and 3.8 litedmin, respectively, with a QJQ, = 1.9/1. Hemodynamic findings are summarized in Table I. Left ventriculography showed normal wall motion with a left ventricular ejection fraction of 52%. Manual coronary angiography revealed an atretic, nondominant right coronary artery. The left coronary artery was markedly dilated with poor visualization because of extremely high coronary flow. Accordingly, power injection of 40 ml at 10 ml/sec (Fig. 3) was perFig. 1. Transesophageal echocardiography. (a) Demonstrates formed through an 8F angioplasty guiding catheter and aneurysmal dilatation of the left main (LM) and circumflex (CX) demonstrated markedly dilated left main and circumflex arteries at the aortic ostlum (AO). (b) The closed arrow indicates arteries with brisk flow from the distal circumflex artery the fistula’s venous lake in relation to the enlarged left atrium into a smooth, dilated venous lake that subsequently (LA). (c) The closed arrow points to the dilated coronary sinus drained into a markedly enlarged coronary sinus. High within the right atrium (RA) at the level of the tricuspid valve fistulous flow limited full visualization of the distal epi(Tv) and right ventricle (RV).
22
Kugelmass et al. TABLE 1. Hemodynamic Findings at Cardiac Catheterization A . Oximetry and Pressure Measurement
O2saturation(%) Superior vena cava Inferior vena cava Right atrium Right ventricle Pulmonary artery Pulmonary capillary wedge Left ventricle Aorta
Preop
Postop
52 69 81 74 73
61 66 61 62 63
-
-
92
96
Pressures (mm Hg) a,v,(mean) Preop -/I74 14) 54/ 13 54/26/(37) 4 2 2 42 I ) I15/17 I I5/75/(92)
Postop -
I1/10/(8) 401 I2 40/19/(27) 14/ 14/(14) -
206/93/(122)
B . Oxygen Consumption and Blood Flow
Oxygen consumption index (liters/min/m') Pulmonary blood flow (literslmin) Systemic blood flow (liters/min)
QdQ,
Preop
Postop
I26 7.3 3.8 I .9
I25 4.6 4.6
1 .o
Fig. 3. Coronary angiography. In (a) (RAO caudal projection) the open arrows indicate the dilated left main and circumflex arteries, and the closed arrow identifies the proximal left anterior descending artery, which fills faintly. In (b) (lateral projection) the closed arrow demonstrates an aneurysmal proximal circumflex artery, while the open arrow indicates the left anterior descending artery, which is poorly visualized.
cardial coronary branches. We considered, but rejected, sinus. A large tortuous circumflex artery drained into a the option of repeating coronary injections during bal- large, partially calcified venous lake. On inspection, the loon occlusion of the circumflex fistula in lieu of repeat- remaining coronary arteries appeared normal. Upon opening the venous lake, the entrance of the circumflex ing coronary angiography postoperatively. Five days following cardiac catheterization the patient artery and exit to the coronary sinus were identified and underwent surgical correction of the fistula. Under car- each was closed with Teflon-pledget bolstered interdiopulmonary bypass, a small persistent left superior rupted sutures. The venous lake was then trimmed and vena cava was noted to drain into the dilated coronary obliterated with a running suture. Intraoperative trans-
Coronary Arteriovenous Fistula
23
stenoses of the proximal and middle left anterior descending artery.
DISCUSSION
Fig. 4. Coronary angiogram following fistula ligation (lateral projection). The open arrow indicates serial stenoses of the left anterior descending artery, which can now be visualized. Closed arrow delineates the total occlusion of the middle left circumflex. The arrowhead demonstrates the residual dilatation of the proximal circumflex.
esophageal Doppler echocardiography , immediately following cessation of cardiopulmonary bypass, showed no further fistulous flow. Upon return from the operating room, the patient’s continuous murmur had disappeared. He converted to sinus rhythm spontaneously on the second postoperative day. Perioperatively there was an elevation of CPK MB isoenzymes, 1063 IU/liter, 13% MB. A radionuclide ventriculogram revealed new posterolateral hypokinesis and inferobasal akinesis, consistent with a myocardial infarction. However, the patient felt markedly improved, with increased exercise tolerance. One month following surgery the patient underwent repeat cardiac catheterization to confirm fistula correction and better evaluate his coronary anatomy in a state of physiologic coronary flow. Hemodynamic evaluation indicated that right and left heart filling pressures were now nearly normal (Table I). A right heart oximetry study documented no persistent shunt. Coronary angiography (Fig. 4), which now allowed opacification of distal vessels, revealed persistent aneurysmal dilatation of the left main and proximal circumflex arteries. The proximal circumflex contained small amounts of intraluminal thrombus, and there was interim total occlusion of the circumflex distal to a large obtuse marginal. Distal marginal vessels received collateral flow from the proximal vessel. Additionally, there were sequential, high grade
Most coronary arteriovenous fistulae are believed to arise from the incomplete obliteration of primitive myocardial sinusoids [6,7]. This developmental arrest results in retained continuity between the mature coronary artery and cardiac vein or chamber. A slight majority of coronary arteriovenous fistulae arise from the right coronary artery [ 1,4,8-lo]. Origins from single or bilateral coronary arteries are rare. Most fistulae terminate in the right ventricle or atrium. Coronary arteriovenous fistulae ending in the pulmonary artery, left atrium or ventricle are less common [1,4,8-lo]. Fifty seven previous cases of coronary artery-coronary sinus fistulae have been reported [2,9,11-191. Coronary arteriovenous fistula may be detected at any age, with the reported age of diagnosis spanning 10 days to 84 years [4]. There is an equal sex distribution. Concurrent congenital cardiac anomalies, including patent ductus arteriosus, ventricular septal defect, atrial septal defect, and persistent superior left vena cava are present in approximately twenty percent of cases [2,20,21]. At diagnosis, one-half of patients are symptomatic, commonly children with large systemic to pulmonary shunts and congestive heart failure, or patients older than 40 years. Most frequent symptoms include dyspnea on exertion, fatigue, angina, as well as recurrent respiratory infections and endocarditis. Angina is often seen in older patients, occuring in up to 80% of patients over 50 years in one series [22]. This is attributed to the natural concurrence of atherosclerotic coronary artery disease. Additionally, myocardial ischemia due to coronary stenoses is believed to be exacerbated by coronary steal that results from high fistulous flow, as has been demonstrated by intracoronary flow measurements during surgical correction of an arteriovenous fistula [23]. Congestive heart failure presumably results from chronic ventricular volume overload in patients with substantial left-to-right shunts, which can be as large as Q,,/Q,=3.1/1 [24]. Atrial arrythmias, primarily fibrillation, are common in coronary arteriovenous fistulae with increased atrial flow. Infectious endocarditis is not uncommon, with a reported incidence of up to 12% [2]. Aneurysmal coronary dilatation is commonplace in symptomatic coronary arteriovenous fistulae. Vessel fibroelastosis has resulted in aneurysm diameters as large as 12 cm [ 141. Postoperative catheterization in adults has revealed little resolution of dilated vessels, and thrombosis may occur [25]. Despite thin aneurysm walls, only one case of spontaneous rupture has been reported [ 161.
24
Kugelmass et al.
Spontaneous closure is rare, having been reported in four cases [25-281. Diagnosis hinges on consideration of the entity. The continuous murmur, as in the present case, is suggestive of coronary arteriovenous fistulae. Chest X-ray and electrocardiogram are nonspecific. Noninvasive imaging has proven valuable in the diagnosis of coronary arteriovenous fistulae. Two-dimensional echocardiography is useful in assessing aneurysmal fistulae [29]. Smaller fistulae may be appreciated by coronary turbulence identified by color Doppler [30]. To our knowledge, this is the first report of evaluation of a coronary arteriovenous fistula by transesophageal echocardiography. This modality allowed definition of the fistula course. Additionally, it was employed intraoperatively to confirm effective fistula ligation. Magnetic resonance imaging has been utilized in four reported cases, and also provides excellent definition of the fistula course [31-331. These modalities are complementary to cardiac catheterization and coronary angiography in the evaluation of coronary arteriovenous fistulae and may assist in the detection of associated congenital anomalies. Selective coronary angiography establishes the fistula course and represents the standard for diagnosis. As this case illustrates, a large coronary arteriovenous fistula may limit visualization of coronary anatomy, which in the adult may obscure atherosclerotic stenoses. Compromised flow to other vessels because of coronary steal may potentiate this problem. Conversely, overestimation of the severity of concomitant atherosclerotic obstructions has resulted from coronary steal due to a coronary arteriovenous fistula [34]. Definitive therapy consists of surgical closure of the fistula, which may be accomplished without extracorporeal support in uncomplicated cases. Cardiopulmonary bypass is employed in situations with difficult access, intramuscular correction, or associated coronary artery bypass graft. Traditionally, operative treatment has been reserved for symptomatic patients. However, some authors argue that elective correction of fistulae in younger, asymptomatic patients can prevent late sequelae and reduce the increased surgical morbidity and mortality observed in older, symptomatic patients [ 12,351. SUMMARY
Coronary arteriovenous fistula is a rare, but clinically important coronary anomaly. Previous reports have documented the efficacy of transthoracic echocardiography and magnetic resonance imaging in diagnosing coronary arteriovenous fistulae. In this communication we have also described the diagnostic and intraoperative utility of transesophageal echocardiography in evaluating this condition. Cardiac catheterization with selective coronary
angiography is crucial for diagnosis and planning surgical management. This case further illustrates that in adult cases of coronary arteriovenous fistulae with a large coronary aneurysm or high fistulous flow, adequate visualization of myocardial branches may be difficult. Postoperative coronary angiography may be needed to assess the presence and extent of coronary atherosclerosis.
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