Pediatr Cardiol 13:178-180, 1992
Pediatric Cardiology 9 Springer-Verlag New York Inc. I992
Echocardiographic Diagnosis of Fistula Between the Left Circumflex Coronary Artery and the Left Atrium E v a n M. Z a h n , Jeffrey F. S m a l l h o r n , G i a n Egger, Patricia E. B u r r o w s , I v a n M. R e b e c c a , a n d R o b e r t M. F r e e d o m Department of Pediatrics, Division of Cardiology and Cardiovascular Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada S U M M A R Y . This report describes a case of fistula between the left circumflex coronary artery and the left atrium, which was identified by color flow mapping. This finding was confirmed by selective coronary arteriography.
KEY WORDS: Coronary artery f i s t u l a - Color flow mapping
T w o - d i m e n s i o n a l D o p p l e r , or color flow m a p p i n g has b e c o m e a w e l l - d e s c r i b e d t e c h n i q u e for the identification o f c o r o n a r y a r t e r y fistulas [I, 3, 4, 6, 7, 9, 10]. T h e m a j o r i t y o f t h e s e cases h a v e i n v o l v e d fistulous c o m m u n i c a t i o n s with the right v e n t r i c l e or a t r i u m . C o n n e c t i o n with the left-sided c h a m b e r s is u n c o m m o n a n d , thus far, e c h o c a r d i o g r a p h i c identific a t i o n has o n l y b e e n d e s c r i b e d in the setting of a fistulous c o m m u n i c a t i o n b e t w e e n the right coron a r y a r t e r y a n d either the left v e n t r i c l e or left a t r i u m [6, 10]. This p r o s p e c t i v e case report describes the c o l o r flow D o p p l e r findings of a case of left c i r c u m f l e x c o r o n a r y a r t e r y to left atrial fistula.
Case Report A 6.5-year-old girl with Down's syndrome was referred to our institution for evaluation of a continuous murmur. At 2.5 years of age the child was diagnosed as having a ventricular septal defect, the clinical findings being confirmed by two-dimensional echocardiography. Following spontaneous closure of the ventricular septal defect, a continuous murmer was heard at a routine assessment. Physical examination revealed a healthy looking child with Down's syndrome. The weight and height were at the 75th and 10th percentile, respectively. The heart rate was 90, with a blood pressure of 90/62 mmHg in the right arm and no gradient between upper and lower extremities. Pulses in all four extremities were brisk. Examination of the chest revealed a modest left ventricular impulse. Auscultation revealed normal heart sounds, Address offprint requests to: Dr. J.F. Smallhorn, Division of
Cardiology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8.
with a grade 2.5/6 blowing continuous murmur at the left sternal border in the third interspace. Chest x-ray demonstrated mild cardiac enlargement, with increased pulmonary arterial vascular markings. The electrocardiogram revealed normal sinus rhythm, a frontal axis of +60~ with a QS pattern and upright T waves in leads V1 and V2. Two-dimensional and M-mode evaluation demonstrated normal intracardiac anatomy, with left ventricular and atrial measurements at the upper limits of normal. Color flow mapping in the precordial long-axis view revealed an area of turbulence that originated from the anterosuperior quadrant of the left atrium. Spectral analysis demonstrated low-velocity continuous flow, thus excluding mitral regurgitation as the source. In the precordial short-axis view, the left main and circumflex coronary arteries appeared dilated (Fig. 1). Color flow Doppler localized the source of turbulence to a fistulous connection between the circumflex coronary artery and the left atrium. Spectral analysis once again confirmed the presence of continuous flow. Interestingly, a ductal view demonstrated an associated small patent ductus arteriosus. Selective coronary angiography confirmed the presence of a left circumflex coronary artery fistula draining into the left atrium (Fig. 2). The small patent ductus arteriosus was likewise confirmed at angiography. Subsequent to this the child underwent ligation of the coronary artery fistula.
Discussion C o r o n a r y a r t e r y fistulas in c h i l d h o o d are rare, with an i n c i d e n c e of 0 , 2 6 - 0 . 4 0 % in selected series. T h e r e is a higher f r e q u e n c y of right c o r o n a r y a r t e r y i n v o l v e m e n t , with m o r e t h a n 90% of fistulas draining into the right side of the heart. D r a i n a g e to either the left a t r i u m or v e n t r i c l e is u n c o m m o n . T h u s far, t h e r e h a v e b e e n o n l y 12 r e p o r t e d cases of left
Zahn et al.: Echocardiography of Coronary Artery Fistula
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circumflex coronary artery to left atrial fistula [2, 5, l t]. The drainage sites within the left atrium are variable and have previously been classified [8]. Type 1 involves a short dilated branch from the left coronary artery, which terminates in the anterior wall or appendage. Type 2 involves the sinus node branch of either the right or left coronary artery, which courses posteriorly between the atria to terminate in the left atrium. Type 3, which can involve either coronary artery, includes all cases where termination is in the posterior wall of the left atrium. The echocardiographic diagnosis of coronary artery fistulas is based on the identification of a dilated coronary artery, with Doppler evidence of turbulent flow at the termination site [12]. The latter technique has been simplified since the advent of color flow mapping techniques. Our index case demonstrates the utility of this technique in identifying drainage to an unusual site. It also enables the echocardiographer to localize the coronary artery branch, which is involved in the fistulous communication. The treatment of choice, which is surgical ligation, is based on the identification of a fistulous communication, rather than on the magnitude of the left-to-right shunt. Although it is clear that this imaging modality provides an accurate means for the identification of these fistulous communications, at this time selective coronary arteriography is still required prior to surgical repair.
References 1. Barton CW, Snider RS, Rosenthal A (1986) Two-dimensional and Doppler echocardiographic features of left circumflex coronary artery to right ventricle fistula: case report and literature review. Pediatr Cardiol 7:167-17 2. Gasul BM, Fell EH, Moreano M, Weinberg M (1959) Congenital coronary arteriovenous aneurysm. Arch Surg 78:203-205 3. Lloyd TR, Mahoney LT, Marvin WJ, Knoedel D (1988) Identification of coronary artery to right ventricular fistulae by color flow mapping. Echocardiography 5:115-120 4. Ludomirsky A, Danford DA, Glasow PF, Blumenschein SD, Murphy DJ, Huhta JC (1987) Evaluation of coronary artery fistula by color-flow Doppler echocardiography. Echocardiography 4:383-387 5. McNamara JJ, Gross RE (1969) Congenital coronary artery fistula. Surgery 65:59-69
4-
Fig. 1. (A) A precordial short-axis view demonstrating a dilated left main coronary artery. (B) A slightly lower short-axis cut showing the entry site of the left circumflex to left atrial fistula. (C) The spectral Doppler trace obtained at the entry site of the fistulous communication.
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Fig. 2. Ascending aortography [lateral projection (A)] and selective left coronary angiograplay [left anterior oblique projection (B)] demonstrate high origin of an enlarged left coronary artery and a fistula between an atrial branch (arrow) of the circumflex artery and the left atrium.
6. Nishikimi T, Hirota K, Kawarabayashi T, Yoshiyami M, Teragaki M, Takenuchi K (1987) Right and left coronary artery to ]eft ventricle fistula detected by color Doppler flow mapping. Am Heart J 4:890-894 7. Ogden JA, Stansel HC (1974) The anatomic variability of coronary fistulae termination in the right and left atria. Chest 65:76-81 8. Oliver JM, Lopez de Sa E, Dominguez F, Sobrino JA, Munoz JE, Iglesias A (1987) Congenital right coronary artery-to-left atrium fistula detected by two-dimensional and Doppler echocardiography. Am Heart J 114:t65-166 9. Pickoff AS, Wolff GS, Bennett VL, Kaiser G, Ferrer PL
(1982) Pulsed Doppler echocardiographic detection of coronary artery to right ventricle fistula. Pediatr Cardiol 2:145149 10. Satomi G, Endo M, Takao A, Nakamura K (1983) A case of right coronary artery to left ventricle fistula: two-dimensional echocardiograpbic study. Pediatr Cardiol 4:229-232 11. Sloman G, MacPhee A, Fairley K (1967) An unusual coronary arteriocameral fistula. Am J Cardiol 15:856-859 12. Velvis H, Schmidt KG, Silverman NH, Turley K (1989) Diagnosis of coronary artery fistula by two-dimensional echocardiography, pulsed Doppler ultrasound and color flow imaging. JACC 14:968-976
Pediatric Cardiology 9 Springer-Verlag New York Inc. I992
Echocardiographic Diagnosis of Fistula Between the Left Circumflex Coronary Artery and the Left Atrium E v a n M. Z a h n , Jeffrey F. S m a l l h o r n , G i a n Egger, Patricia E. B u r r o w s , I v a n M. R e b e c c a , a n d R o b e r t M. F r e e d o m Department of Pediatrics, Division of Cardiology and Cardiovascular Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada S U M M A R Y . This report describes a case of fistula between the left circumflex coronary artery and the left atrium, which was identified by color flow mapping. This finding was confirmed by selective coronary arteriography.
KEY WORDS: Coronary artery f i s t u l a - Color flow mapping
T w o - d i m e n s i o n a l D o p p l e r , or color flow m a p p i n g has b e c o m e a w e l l - d e s c r i b e d t e c h n i q u e for the identification o f c o r o n a r y a r t e r y fistulas [I, 3, 4, 6, 7, 9, 10]. T h e m a j o r i t y o f t h e s e cases h a v e i n v o l v e d fistulous c o m m u n i c a t i o n s with the right v e n t r i c l e or a t r i u m . C o n n e c t i o n with the left-sided c h a m b e r s is u n c o m m o n a n d , thus far, e c h o c a r d i o g r a p h i c identific a t i o n has o n l y b e e n d e s c r i b e d in the setting of a fistulous c o m m u n i c a t i o n b e t w e e n the right coron a r y a r t e r y a n d either the left v e n t r i c l e or left a t r i u m [6, 10]. This p r o s p e c t i v e case report describes the c o l o r flow D o p p l e r findings of a case of left c i r c u m f l e x c o r o n a r y a r t e r y to left atrial fistula.
Case Report A 6.5-year-old girl with Down's syndrome was referred to our institution for evaluation of a continuous murmur. At 2.5 years of age the child was diagnosed as having a ventricular septal defect, the clinical findings being confirmed by two-dimensional echocardiography. Following spontaneous closure of the ventricular septal defect, a continuous murmer was heard at a routine assessment. Physical examination revealed a healthy looking child with Down's syndrome. The weight and height were at the 75th and 10th percentile, respectively. The heart rate was 90, with a blood pressure of 90/62 mmHg in the right arm and no gradient between upper and lower extremities. Pulses in all four extremities were brisk. Examination of the chest revealed a modest left ventricular impulse. Auscultation revealed normal heart sounds, Address offprint requests to: Dr. J.F. Smallhorn, Division of
Cardiology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8.
with a grade 2.5/6 blowing continuous murmur at the left sternal border in the third interspace. Chest x-ray demonstrated mild cardiac enlargement, with increased pulmonary arterial vascular markings. The electrocardiogram revealed normal sinus rhythm, a frontal axis of +60~ with a QS pattern and upright T waves in leads V1 and V2. Two-dimensional and M-mode evaluation demonstrated normal intracardiac anatomy, with left ventricular and atrial measurements at the upper limits of normal. Color flow mapping in the precordial long-axis view revealed an area of turbulence that originated from the anterosuperior quadrant of the left atrium. Spectral analysis demonstrated low-velocity continuous flow, thus excluding mitral regurgitation as the source. In the precordial short-axis view, the left main and circumflex coronary arteries appeared dilated (Fig. 1). Color flow Doppler localized the source of turbulence to a fistulous connection between the circumflex coronary artery and the left atrium. Spectral analysis once again confirmed the presence of continuous flow. Interestingly, a ductal view demonstrated an associated small patent ductus arteriosus. Selective coronary angiography confirmed the presence of a left circumflex coronary artery fistula draining into the left atrium (Fig. 2). The small patent ductus arteriosus was likewise confirmed at angiography. Subsequent to this the child underwent ligation of the coronary artery fistula.
Discussion C o r o n a r y a r t e r y fistulas in c h i l d h o o d are rare, with an i n c i d e n c e of 0 , 2 6 - 0 . 4 0 % in selected series. T h e r e is a higher f r e q u e n c y of right c o r o n a r y a r t e r y i n v o l v e m e n t , with m o r e t h a n 90% of fistulas draining into the right side of the heart. D r a i n a g e to either the left a t r i u m or v e n t r i c l e is u n c o m m o n . T h u s far, t h e r e h a v e b e e n o n l y 12 r e p o r t e d cases of left
Zahn et al.: Echocardiography of Coronary Artery Fistula
179
circumflex coronary artery to left atrial fistula [2, 5, l t]. The drainage sites within the left atrium are variable and have previously been classified [8]. Type 1 involves a short dilated branch from the left coronary artery, which terminates in the anterior wall or appendage. Type 2 involves the sinus node branch of either the right or left coronary artery, which courses posteriorly between the atria to terminate in the left atrium. Type 3, which can involve either coronary artery, includes all cases where termination is in the posterior wall of the left atrium. The echocardiographic diagnosis of coronary artery fistulas is based on the identification of a dilated coronary artery, with Doppler evidence of turbulent flow at the termination site [12]. The latter technique has been simplified since the advent of color flow mapping techniques. Our index case demonstrates the utility of this technique in identifying drainage to an unusual site. It also enables the echocardiographer to localize the coronary artery branch, which is involved in the fistulous communication. The treatment of choice, which is surgical ligation, is based on the identification of a fistulous communication, rather than on the magnitude of the left-to-right shunt. Although it is clear that this imaging modality provides an accurate means for the identification of these fistulous communications, at this time selective coronary arteriography is still required prior to surgical repair.
References 1. Barton CW, Snider RS, Rosenthal A (1986) Two-dimensional and Doppler echocardiographic features of left circumflex coronary artery to right ventricle fistula: case report and literature review. Pediatr Cardiol 7:167-17 2. Gasul BM, Fell EH, Moreano M, Weinberg M (1959) Congenital coronary arteriovenous aneurysm. Arch Surg 78:203-205 3. Lloyd TR, Mahoney LT, Marvin WJ, Knoedel D (1988) Identification of coronary artery to right ventricular fistulae by color flow mapping. Echocardiography 5:115-120 4. Ludomirsky A, Danford DA, Glasow PF, Blumenschein SD, Murphy DJ, Huhta JC (1987) Evaluation of coronary artery fistula by color-flow Doppler echocardiography. Echocardiography 4:383-387 5. McNamara JJ, Gross RE (1969) Congenital coronary artery fistula. Surgery 65:59-69
4-
Fig. 1. (A) A precordial short-axis view demonstrating a dilated left main coronary artery. (B) A slightly lower short-axis cut showing the entry site of the left circumflex to left atrial fistula. (C) The spectral Doppler trace obtained at the entry site of the fistulous communication.
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Fig. 2. Ascending aortography [lateral projection (A)] and selective left coronary angiograplay [left anterior oblique projection (B)] demonstrate high origin of an enlarged left coronary artery and a fistula between an atrial branch (arrow) of the circumflex artery and the left atrium.
6. Nishikimi T, Hirota K, Kawarabayashi T, Yoshiyami M, Teragaki M, Takenuchi K (1987) Right and left coronary artery to ]eft ventricle fistula detected by color Doppler flow mapping. Am Heart J 4:890-894 7. Ogden JA, Stansel HC (1974) The anatomic variability of coronary fistulae termination in the right and left atria. Chest 65:76-81 8. Oliver JM, Lopez de Sa E, Dominguez F, Sobrino JA, Munoz JE, Iglesias A (1987) Congenital right coronary artery-to-left atrium fistula detected by two-dimensional and Doppler echocardiography. Am Heart J 114:t65-166 9. Pickoff AS, Wolff GS, Bennett VL, Kaiser G, Ferrer PL
(1982) Pulsed Doppler echocardiographic detection of coronary artery to right ventricle fistula. Pediatr Cardiol 2:145149 10. Satomi G, Endo M, Takao A, Nakamura K (1983) A case of right coronary artery to left ventricle fistula: two-dimensional echocardiograpbic study. Pediatr Cardiol 4:229-232 11. Sloman G, MacPhee A, Fairley K (1967) An unusual coronary arteriocameral fistula. Am J Cardiol 15:856-859 12. Velvis H, Schmidt KG, Silverman NH, Turley K (1989) Diagnosis of coronary artery fistula by two-dimensional echocardiography, pulsed Doppler ultrasound and color flow imaging. JACC 14:968-976
