© 2014, Wiley Periodicals, Inc. DOI: 10.1111/echo.12822
Echocardiography
CASE REPORTS Section Editor: Brain D. Hoit, M.D.
Infective Endocarditis Related to a Coronary Artery Fistula with an Unusual Localization and Ectatic Coronary Arteries Demet Menekse Gerede, M.D.,* Aynur Acibuca, M.D.,* Caglar Uzun, M.D.,† Huseyin Goksuluk, M.D.,* Aydan Ongun, M.D.,* Mustafa Kilickap, M.D.,* and Cetin Erol, M.D.* *Department of Cardiology, Ankara University School of Medicine, Ankara, Turkey; and †Department of Radiology, Ankara University School of Medicine, Ankara, Turkey
Coronary artery fistulas (CAF) are a rare cardiac anomaly that can be either congenital or acquired. CAFs have clinical significance because of complications such as dyspnea on exertion, congestive heart failure, and cardiac tamponade. The literature also contains case reports of CAF presenting as bacterial endocarditis. We describe a 31-year-old man who presented with native valve infective endocarditis related to an unusual form of a CAF between the circumflex coronary artery and left ventricle. He also had giant coronary arteries, which were imaged with computed tomography angiography and transesophageal echocardiography. The diameter of the circumflex coronary artery and left main coronary artery was measured as 19 mm. Surgical intervention for heart valves was performed because of vegetations resistant to continued antibiotic treatment. At the same time, the CAF was treated with surgery. (Echocardiography 2015;32:711–715) Key words: coronary artery fistula, giant coronary artery, infective endocarditis Coronary artery fistulas (CAF) are an uncommon heart anomaly, which consists of an abnormal communication between a coronary artery and a cardiac chamber or pulmonary vessel.1,2 The majority of CAFs occur in the right coronary artery and left anterior descending coronary artery; the circumflex coronary arteries are rarely involved.3 More than 90% of CAFs drain to the right side of the heart. Drainage to the left ventricle is least common, accounting for less than 3% of cases.4,5 The clinical presentation is related to the CAF size and patient age. Symptoms and complications of medium- or large-sized CAF are rare in childhood but become progressively apparent in patients diagnosed after 20 years old. The clinical presentation in adults includes angina, myocardial infarction, heart failure, arrhythmia, and endocarditis.4 Although there are case reports of associated CAF between infective endocarditis, the incidence of endocarditis in presence of CAF remains unknown.1,2 We describe the clinical status of a patient presenting with bacterial endocarditis and a rare fistula formed between the circumflex coronary artery Address for correspondence and reprint requests: Demet Menekse Gerede, M.D., Department of Cardiology, Ankara University School of Medicine, Ankara, Turkey 06340. Fax: +90 (32) 312 52 51; E-mail: [email protected]
and the left ventricle. In addition, he had unusually large coronary arteries. Case Presentation: A 31-year-old man was admitted to the hospital of Ankara University with fatigue and fever. While investigating infectious and rheumatic diseases, we suspected infective endocarditis. Then, a transthoracic echocardiography (TTE) (Vivid S5; GE, Horten, Norway) was performed and revealed vegetation on the mitral valve 11 9 4.5 mm in size. There was also Streptococcus spp. growth in the blood culture. Therefore, infective endocarditis was diagnosed, and antibiotic treatment was given. After 1 month of antibiotherapy, the size of vegetation was nearly the same. At the fifth weeks of treatment, the patient experienced a stomachache, and abdomen computed tomography (CT) (Aquilion 64, Toshiba Medical Systems, Tokyo, Japan) revealed a renal infarction associated with septic embolism. First, TTE was repeated and showed vegetation on the mitral and aortic valves. Simultaneously, an image near the mitral posterior leaflet suggesting an abscess cavity was found. Transesophageal echocardiography (TEE) immediately was performed and revealed vegetation on the mitral valve 19 9 6 mm in size (movie clips S1 and S2, 711
Gerede, et al.
Figure 1. Transesophageal echocardiogram, mid-esophageal modified long-axis view, revealing mobile vegetation (arrow) originating from mitral valve protruding into left atrium (LA). LV = left ventricle; AO = aorta.
Figure 4. Short-axis view on transesophageal echocardiography demonstrating dilated left main coronary artery. LMCA = left main coronary artery; Ao = aortic valve; LA = left atrium; LCC = left coronary cusp.
Figure 2. Mid-esophageal long-axis view on transesophageal echocardiography demonstrating echogenic mass suggestive of endocarditis on the aortic valve (arrow). LV = left ventricle; LA = left atrium; AO = aorta.
previously thought to be an abscess cavity was found to be dilated coronary arteries (Figs. 3 and 4). Considering the suspicion of coronary artery ectasia, a CT angiography was performed. It showed dilatation of the left main and circumflex coronary arteries up to 19 mm in diameter (Figs. 5 and 6). A CAF was also recognized between the circumflex coronary artery and the left ventricle near the mitral posterior leaflet on the CT images (Fig. 7). During clinical follow-up, valvular vegetations were unresponsive to antibiotic treatment, and a septic renal embolism occurred. Therefore, it was decided to perform mitral and aortic valve surgery. Surgical correction of the CAF at the same time was also planned. The coronary angiography (CAG) was performed and confirmed the dilatation of these two coronary arteries up to 16 mm diameter and
Figure 3. Pulsed-wave Doppler echocardiography displays diastolic coronary flow in dilated structure.
Fig. 1) and very small vegetation on the aortic valve (Fig. 2). When viewed with pulsed-wave Doppler at the 45° short axis of the TEE image, the structure 712
Figure 5. CT angiography reveals the dilation of left main and circumflex coronary arteries up to 19 mm in diameter. LMCA = left main coronary artery; AO = aorta.
Endocarditis, Coronary Ectasia, and Fistula Association
Figure 6. A, B. The fistulization of ectatic circumflex coronary artery to the left ventricle is showed on “volume rendered” (VR) three-dimensional view (arrow). Left anterior descending coronary artery (A) is marked as an arrowhead.
the CAF between the circumflex coronary artery and the left ventricle (Fig. 8). The vegetation on mitral posterior leaflet 15 9 4 mm in size and 2 9 3 mm in size small vegetation on the aortic valve were observed at the operation. Then, the mitral and aortic valves were replaced, and the CAF was corrected in surgery. Discussion: The view near the mitral posterior leaflet was considered to be an abscess pouch, or a pseudoaneurysm associated with infective endocarditis, because pseudoaneurysms of the left ventricle most commonly develop from abscesses in the mitral-aortic intervalvular fibrosa.6,7 However, CT angiography and TEE ruled out this diagnosis and showed diffuse dilatation of coronary arteries. This enlargement can be classified as coronary artery ectasia, which is diagnosed when a segment of the artery is 1.5 times larger than the diameter of the corresponding segment of the normal adjacent artery.8 The etiology and pathogenesis of this coronary enlargement are not well known. It can be either congenital or acquired. It can represent a physiologic response induced by
increased demand in a hypertrophied ventricle or by increased flow due to a coronary artery steal, as seen with a CAF or a coronary artery from the pulmonary artery with an anomalous origin. Coronary artery ectasia can also be acquired from a disease involving the coronary arteries, such as Kawasaki disease (KD). Extensive atherosclerotic changes and the destruction of the media of the coronary artery vessels seem to play a role in the pathogenesis of coronary artery ectasia.9 In our case, atherosclerotic changes were not observed in these vessels during the CAG. KD was thought to be in this patient. However, this disease is usually a clinical diagnosis. No laboratory studies are included among the diagnostic criteria for typical KD, but especially incomplete form of it should also be suspected when faced with coronary artery ectasia. For our patient, his clinical and laboratory signs were not compatible with KD, but we could not exclude the diagnosis of incomplete form of KD. Because of coexistence of CAF and coronary ectasia, we considered it to appear probably due to increased flow as a consequence of coronary artery steal, such as CAF. The patient’s ectatic coronary arteries likely are 713
Gerede, et al.
Figure 7. A, B. Two-dimensional double-oblique images; ectatic circumflex coronary artery (blue arrow), mitral valve posterior leaflet (yellow arrow), aortic valve (arrowheads). The fistulization of the dilated left circumflex coronary artery to left ventricle through inferior region of the posterior mitral leaflet is demonstrated (B).
Figure 8. Cardiac catheterization revealing coronary artery fistula connecting ectatic circumflex artery (CX) to left ventricle (white arrows).
congenital, or the coronary artery might have a predisposition to dilatation because of the media layer structure and exposure to increased flow due to CAF. However, we do not have conclusive evidence associated with the etiology of coronary artery ectasia in our case. 714
The other notable aspect of this case is infective endocarditis associated with a CAF between the circumflex coronary artery and the left ventricle. CAFs are rare, acquired or congenital coronary artery abnormalities. Congenital forms are more frequent.3 CAFs can also occur after chest trauma, cardiac surgery, or coronary interventions. Our patient did not have any of these predisposing conditions. The CAF likely was congenital, and this abnormality might have caused his native valve infective endocarditis because one of the most common CAF-related complications is infective endocarditis, along with dyspnea on exertion, palpitation, congestive heart failure, and death.10 The patient had no other predisposing factors, except for the vegetation which was assumed to occur because of the turbulent flow related with fistula near the mitral valve. When considering management of CAF, two factors which enter into the decision are complications likely related to the fistula and whether the patient has other indications to undergo an invasive procedure.4 Infective endocarditis in this patient was a CAF-related complication. At the same time, vegetation on mitral valve was persistent and unresponsive to antibiotic treatment, which posed an indication for surgery. The available therapeutic options for CAF include surgical correction and transcatheter embolization. In our case, surgical correction was found to be
Endocarditis, Coronary Ectasia, and Fistula Association
appropriate because the mitral and aortic valveassociated infective endocarditis required surgical intervention at the same time as the CAF. Conclusion: Transthoracic echocardiography, TEE, CAG, and CT angiography play an essential and complimentary role in the management of CAF and its complication. Vegetations on the valves should be considered as one of the complications of CAF. References
1. Kasravi B, Reid CL, Allen BJ: Coronary artery fistula presenting as bacterial endocarditis. J Am Soc Echocardiogr 2004;17:1315–1316. 2. Alkhulaifi AM, Horner SM, Pugsley WB, et al: Coronary artery fistulas presenting with bacterial endocarditis. Ann Thorac Surg 1995;60:202–204. 3. Gowda RM, Vasavada BC, Khan AI: Coronary artery fistulas: Clinical and therapeutic considerations. Int J Cardiol 2006;107:7–10. 4. Latson LA: Coronary artery fistulas: How to manage them. Catheter Cardiovasc Interv 2007;70:110–116.
5. Milici C, Bovelli D, Borghetti V, et al: A giant coronary artery aneurysm with coronary arteriovenous fistula in asymptomatic elderly patient. Case Rep Vasc Med 2013;2013:847972. 6. Baumgartner FJ, Omari BO, Robertson JM, et al: Annular abscesses in surgical endocarditis: Anatomic, clinical and operative features. Ann Thorac Surg 2000;70:442–447. 7. Kang N, Wan S, Ng CSH, et al: Periannular extension of infective endocarditis. Ann Thorac Cardiovasc Surg 2009;15:74–81. 8. Hartnell GG, Parnell BM, Pridie RB: Coronary artery ectasia. Its prevalence and clinical significance in 4993 patients. Br Heart J 1985;54:392–395. 9. Syed M, Lesch M: Coronary artery aneurysm: A review. Prog Cardiovasc Dis 1997;40:77. 10. Urrutia SCO, Falaschi G, Ott DA, et al: Surgical management of 56 patients with congenital coronary artery fistulas. Ann Thorac Surg 1983;35:300–307.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie Clips S1 and S2. Transgastric view of TEE reveals vegetations on mitral leaflets.
715
Copyright of Echocardiography is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Echocardiography
CASE REPORTS Section Editor: Brain D. Hoit, M.D.
Infective Endocarditis Related to a Coronary Artery Fistula with an Unusual Localization and Ectatic Coronary Arteries Demet Menekse Gerede, M.D.,* Aynur Acibuca, M.D.,* Caglar Uzun, M.D.,† Huseyin Goksuluk, M.D.,* Aydan Ongun, M.D.,* Mustafa Kilickap, M.D.,* and Cetin Erol, M.D.* *Department of Cardiology, Ankara University School of Medicine, Ankara, Turkey; and †Department of Radiology, Ankara University School of Medicine, Ankara, Turkey
Coronary artery fistulas (CAF) are a rare cardiac anomaly that can be either congenital or acquired. CAFs have clinical significance because of complications such as dyspnea on exertion, congestive heart failure, and cardiac tamponade. The literature also contains case reports of CAF presenting as bacterial endocarditis. We describe a 31-year-old man who presented with native valve infective endocarditis related to an unusual form of a CAF between the circumflex coronary artery and left ventricle. He also had giant coronary arteries, which were imaged with computed tomography angiography and transesophageal echocardiography. The diameter of the circumflex coronary artery and left main coronary artery was measured as 19 mm. Surgical intervention for heart valves was performed because of vegetations resistant to continued antibiotic treatment. At the same time, the CAF was treated with surgery. (Echocardiography 2015;32:711–715) Key words: coronary artery fistula, giant coronary artery, infective endocarditis Coronary artery fistulas (CAF) are an uncommon heart anomaly, which consists of an abnormal communication between a coronary artery and a cardiac chamber or pulmonary vessel.1,2 The majority of CAFs occur in the right coronary artery and left anterior descending coronary artery; the circumflex coronary arteries are rarely involved.3 More than 90% of CAFs drain to the right side of the heart. Drainage to the left ventricle is least common, accounting for less than 3% of cases.4,5 The clinical presentation is related to the CAF size and patient age. Symptoms and complications of medium- or large-sized CAF are rare in childhood but become progressively apparent in patients diagnosed after 20 years old. The clinical presentation in adults includes angina, myocardial infarction, heart failure, arrhythmia, and endocarditis.4 Although there are case reports of associated CAF between infective endocarditis, the incidence of endocarditis in presence of CAF remains unknown.1,2 We describe the clinical status of a patient presenting with bacterial endocarditis and a rare fistula formed between the circumflex coronary artery Address for correspondence and reprint requests: Demet Menekse Gerede, M.D., Department of Cardiology, Ankara University School of Medicine, Ankara, Turkey 06340. Fax: +90 (32) 312 52 51; E-mail: [email protected]
and the left ventricle. In addition, he had unusually large coronary arteries. Case Presentation: A 31-year-old man was admitted to the hospital of Ankara University with fatigue and fever. While investigating infectious and rheumatic diseases, we suspected infective endocarditis. Then, a transthoracic echocardiography (TTE) (Vivid S5; GE, Horten, Norway) was performed and revealed vegetation on the mitral valve 11 9 4.5 mm in size. There was also Streptococcus spp. growth in the blood culture. Therefore, infective endocarditis was diagnosed, and antibiotic treatment was given. After 1 month of antibiotherapy, the size of vegetation was nearly the same. At the fifth weeks of treatment, the patient experienced a stomachache, and abdomen computed tomography (CT) (Aquilion 64, Toshiba Medical Systems, Tokyo, Japan) revealed a renal infarction associated with septic embolism. First, TTE was repeated and showed vegetation on the mitral and aortic valves. Simultaneously, an image near the mitral posterior leaflet suggesting an abscess cavity was found. Transesophageal echocardiography (TEE) immediately was performed and revealed vegetation on the mitral valve 19 9 6 mm in size (movie clips S1 and S2, 711
Gerede, et al.
Figure 1. Transesophageal echocardiogram, mid-esophageal modified long-axis view, revealing mobile vegetation (arrow) originating from mitral valve protruding into left atrium (LA). LV = left ventricle; AO = aorta.
Figure 4. Short-axis view on transesophageal echocardiography demonstrating dilated left main coronary artery. LMCA = left main coronary artery; Ao = aortic valve; LA = left atrium; LCC = left coronary cusp.
Figure 2. Mid-esophageal long-axis view on transesophageal echocardiography demonstrating echogenic mass suggestive of endocarditis on the aortic valve (arrow). LV = left ventricle; LA = left atrium; AO = aorta.
previously thought to be an abscess cavity was found to be dilated coronary arteries (Figs. 3 and 4). Considering the suspicion of coronary artery ectasia, a CT angiography was performed. It showed dilatation of the left main and circumflex coronary arteries up to 19 mm in diameter (Figs. 5 and 6). A CAF was also recognized between the circumflex coronary artery and the left ventricle near the mitral posterior leaflet on the CT images (Fig. 7). During clinical follow-up, valvular vegetations were unresponsive to antibiotic treatment, and a septic renal embolism occurred. Therefore, it was decided to perform mitral and aortic valve surgery. Surgical correction of the CAF at the same time was also planned. The coronary angiography (CAG) was performed and confirmed the dilatation of these two coronary arteries up to 16 mm diameter and
Figure 3. Pulsed-wave Doppler echocardiography displays diastolic coronary flow in dilated structure.
Fig. 1) and very small vegetation on the aortic valve (Fig. 2). When viewed with pulsed-wave Doppler at the 45° short axis of the TEE image, the structure 712
Figure 5. CT angiography reveals the dilation of left main and circumflex coronary arteries up to 19 mm in diameter. LMCA = left main coronary artery; AO = aorta.
Endocarditis, Coronary Ectasia, and Fistula Association
Figure 6. A, B. The fistulization of ectatic circumflex coronary artery to the left ventricle is showed on “volume rendered” (VR) three-dimensional view (arrow). Left anterior descending coronary artery (A) is marked as an arrowhead.
the CAF between the circumflex coronary artery and the left ventricle (Fig. 8). The vegetation on mitral posterior leaflet 15 9 4 mm in size and 2 9 3 mm in size small vegetation on the aortic valve were observed at the operation. Then, the mitral and aortic valves were replaced, and the CAF was corrected in surgery. Discussion: The view near the mitral posterior leaflet was considered to be an abscess pouch, or a pseudoaneurysm associated with infective endocarditis, because pseudoaneurysms of the left ventricle most commonly develop from abscesses in the mitral-aortic intervalvular fibrosa.6,7 However, CT angiography and TEE ruled out this diagnosis and showed diffuse dilatation of coronary arteries. This enlargement can be classified as coronary artery ectasia, which is diagnosed when a segment of the artery is 1.5 times larger than the diameter of the corresponding segment of the normal adjacent artery.8 The etiology and pathogenesis of this coronary enlargement are not well known. It can be either congenital or acquired. It can represent a physiologic response induced by
increased demand in a hypertrophied ventricle or by increased flow due to a coronary artery steal, as seen with a CAF or a coronary artery from the pulmonary artery with an anomalous origin. Coronary artery ectasia can also be acquired from a disease involving the coronary arteries, such as Kawasaki disease (KD). Extensive atherosclerotic changes and the destruction of the media of the coronary artery vessels seem to play a role in the pathogenesis of coronary artery ectasia.9 In our case, atherosclerotic changes were not observed in these vessels during the CAG. KD was thought to be in this patient. However, this disease is usually a clinical diagnosis. No laboratory studies are included among the diagnostic criteria for typical KD, but especially incomplete form of it should also be suspected when faced with coronary artery ectasia. For our patient, his clinical and laboratory signs were not compatible with KD, but we could not exclude the diagnosis of incomplete form of KD. Because of coexistence of CAF and coronary ectasia, we considered it to appear probably due to increased flow as a consequence of coronary artery steal, such as CAF. The patient’s ectatic coronary arteries likely are 713
Gerede, et al.
Figure 7. A, B. Two-dimensional double-oblique images; ectatic circumflex coronary artery (blue arrow), mitral valve posterior leaflet (yellow arrow), aortic valve (arrowheads). The fistulization of the dilated left circumflex coronary artery to left ventricle through inferior region of the posterior mitral leaflet is demonstrated (B).
Figure 8. Cardiac catheterization revealing coronary artery fistula connecting ectatic circumflex artery (CX) to left ventricle (white arrows).
congenital, or the coronary artery might have a predisposition to dilatation because of the media layer structure and exposure to increased flow due to CAF. However, we do not have conclusive evidence associated with the etiology of coronary artery ectasia in our case. 714
The other notable aspect of this case is infective endocarditis associated with a CAF between the circumflex coronary artery and the left ventricle. CAFs are rare, acquired or congenital coronary artery abnormalities. Congenital forms are more frequent.3 CAFs can also occur after chest trauma, cardiac surgery, or coronary interventions. Our patient did not have any of these predisposing conditions. The CAF likely was congenital, and this abnormality might have caused his native valve infective endocarditis because one of the most common CAF-related complications is infective endocarditis, along with dyspnea on exertion, palpitation, congestive heart failure, and death.10 The patient had no other predisposing factors, except for the vegetation which was assumed to occur because of the turbulent flow related with fistula near the mitral valve. When considering management of CAF, two factors which enter into the decision are complications likely related to the fistula and whether the patient has other indications to undergo an invasive procedure.4 Infective endocarditis in this patient was a CAF-related complication. At the same time, vegetation on mitral valve was persistent and unresponsive to antibiotic treatment, which posed an indication for surgery. The available therapeutic options for CAF include surgical correction and transcatheter embolization. In our case, surgical correction was found to be
Endocarditis, Coronary Ectasia, and Fistula Association
appropriate because the mitral and aortic valveassociated infective endocarditis required surgical intervention at the same time as the CAF. Conclusion: Transthoracic echocardiography, TEE, CAG, and CT angiography play an essential and complimentary role in the management of CAF and its complication. Vegetations on the valves should be considered as one of the complications of CAF. References
1. Kasravi B, Reid CL, Allen BJ: Coronary artery fistula presenting as bacterial endocarditis. J Am Soc Echocardiogr 2004;17:1315–1316. 2. Alkhulaifi AM, Horner SM, Pugsley WB, et al: Coronary artery fistulas presenting with bacterial endocarditis. Ann Thorac Surg 1995;60:202–204. 3. Gowda RM, Vasavada BC, Khan AI: Coronary artery fistulas: Clinical and therapeutic considerations. Int J Cardiol 2006;107:7–10. 4. Latson LA: Coronary artery fistulas: How to manage them. Catheter Cardiovasc Interv 2007;70:110–116.
5. Milici C, Bovelli D, Borghetti V, et al: A giant coronary artery aneurysm with coronary arteriovenous fistula in asymptomatic elderly patient. Case Rep Vasc Med 2013;2013:847972. 6. Baumgartner FJ, Omari BO, Robertson JM, et al: Annular abscesses in surgical endocarditis: Anatomic, clinical and operative features. Ann Thorac Surg 2000;70:442–447. 7. Kang N, Wan S, Ng CSH, et al: Periannular extension of infective endocarditis. Ann Thorac Cardiovasc Surg 2009;15:74–81. 8. Hartnell GG, Parnell BM, Pridie RB: Coronary artery ectasia. Its prevalence and clinical significance in 4993 patients. Br Heart J 1985;54:392–395. 9. Syed M, Lesch M: Coronary artery aneurysm: A review. Prog Cardiovasc Dis 1997;40:77. 10. Urrutia SCO, Falaschi G, Ott DA, et al: Surgical management of 56 patients with congenital coronary artery fistulas. Ann Thorac Surg 1983;35:300–307.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie Clips S1 and S2. Transgastric view of TEE reveals vegetations on mitral leaflets.
715
Copyright of Echocardiography is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
