Catheterization and Cardiovascular Interventions 85:620–624 (2015)
An Unusual Case of Infective Endocarditis Involving a Right Coronary Artery to Superior Vena Cava Fistula Ujjval Jariwala,1,2 MD, Rani K. Hasan,1 MD, MHS, Eric M. Thorn,3 MD, and Sammy Zakaria,1* MD, MPH Coronary artery fistulas (CAFs) are rare and mostly congenital anomalous connections between a coronary artery and a cardiac chamber or great vessel. Most CAFs are small, asymptomatic, and found incidentally during cardiac imaging. However, they can lead to serious complications including myocardial infarction, congestive heart failure, arrhythmias, or fistula rupture. CAFs have been associated with infective endocarditis, but to our knowledge, this complication has never been reported involving an isolated CAF to an otherwise anatomically normal great vessel. We report the first case of this complication in a 49-year-old man with a presumed streptococcus vegetation found within an isolated large, tortuous CAF connecting the right coronary artery to the superior vena cava. After completing antibiotic treatment, transcatheter closure of the CAF was performed. Since then, the patient has remained symptom-free. This case demonstrates that CAF closure is feasible following CAF-associated endocarditis, and that closure may represent a viable strategy for reducing risk of recurrent infection. C 2014 Wiley Periodicals, Inc. V
Key words: angiography; coronary; anomalous coronaries; closure; vascular access; fistula/shunts
INTRODUCTION
A coronary artery fistula (CAF) is an abnormal connection between a coronary artery and a cardiac chamber or great vessel. While uncommon, CAFs can lead to clinically significant complications such as myocardial ischemia due to coronary steal, congestive heart failure from volume overload, arrhythmias, or fistula rupture [1]. Rarely, CAFs are associated with infective endocarditis. However, to our knowledge, there are no reports of endocarditis occurring in a patient with an isolated coronary fistula to an otherwise anatomically normal great vessel. We report the first description of this complication and subsequent transcatheter closure (TCC) of the CAF, which connected the right coronary artery (RCA) to the superior vena cava (SVC).
CASE REPORT
A 49-year-old man with diabetes mellitus complained of intermittent fever, fatigue, headache, cough, occasional dizziness, and anorexia. He had a normal chest radiograph and a negative tuberculin sensitivity skin test. He was empirically treated with seven days of clarithromycin followed by seven days of doxycycline. After six weeks, he continued to have symptoms and was hospitalized for further evaluation. On admisC 2014 Wiley Periodicals, Inc. V
sion, he had a temperature of 38.6 C (101.6 F), a blood pressure of 170/92 mm Hg, a heart rate of 129/min, and a respiratory rate of 16/min. His physical examination revealed a moderate intensity continuous murmur over the left lower sternal border, which was not previously noted. Otherwise, his physical examination was unremarkable, and he did not have any signs of heart failure. He had a normal electrocardiogram. His laboratory studies were remarkable for a white blood cell count of 11.9/lL (76% neutrophils and 14% Additional Supporting Information may be found in the online version of this article. 1
Johns Hopkins University School of Medicine, Baltimore, Maryland 2 Greater Baltimore Medical Center, Towson, Maryland 3 Virginia Cardiovascular Associates, Manassas, Virginia Conflict of interest: Nothing to report. *Correspondence to: Sammy Zakaria, MD, MPH, 4940 Eastern Avenue, Building 301, Suite 2400, Baltimore, MD 21224. E-mail: [email protected] Received 15 July 2013; Revision accepted 6 July 2014 DOI: 10.1002/ccd.25597 Published online 10 July (wileyonlinelibrary.com)
2014
in
Wiley
Online
Library
Coronary Artery Fistula Endocarditis
Fig. 1. A high esophageal transesophageal echocardiogram view demonstrating a vegetation (*) presumably near the ostium of the RCA to SVC fistula. RPA: right pulmonary artery, LA: left atrium, RA: right atrium, SVC: superior vena cava. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
Fig. 2. 3D cardiac computed tomography image highlighting a CAF originating from the RCA, following a tortuous course, and ending within the SVC.
lymphocytes), erythrocyte sedimentation rate of 101 mm/hr, and C-reactive protein of 10.4 mg/dL. Blood cultures obtained at admission grew viridans streptococci. Since endocarditis was suspected, transesophageal echocardiography (TEE) was performed. The TEE demonstrated evidence of a large CAF connecting the RCA with the SVC, coursing between the
621
Fig. 3. Contrast injection into the right coronary artery demonstrates a dilated and tortuous coronary artery fistula connecting into the superior vena cava.
aorta and the right atrium, with blood flow shunting toward the SVC based on color flow Doppler imaging. In addition, a vegetation was visualized at the junction between the fistula and the SVC (Fig. 1, Supporting Information Movie 1). The patient was then successfully treated with six weeks of intravenous ceftriaxone and vancomycin. Antibiotics were then stopped, and subsequent repeat blood cultures were negative. Since the fistula was considered a nidus for recurrent infection, the patient was referred for CAF closure. A cardiac computed tomographic angiogram (CTA) was first performed to further delineate CAF anatomy, and showed dilation and tortuosity of the proximal RCA, with fistulous communication to the anterior aspect of the SVC just above the insertion of the SVC to the right atrium (Fig. 2). The maximum diameter of fistula was 13 mm. There was a left to right shunt via the fistula with mild enlargement of the right heart chambers. Guided by the findings on CTA, the patient then underwent TCC of fistula. Initially, diagnostic angiography was performed which confirmed the RCA to SVC fistula (Fig. 3, Supporting Information Movie 2). After attempts to access the fistula from a venous approach failed, a coronary guide catheter was then successfully advanced from the right femoral artery into the fistulous tract via the RCA. Based on the angiography images, a number of closure approaches were then considered, including deploying multiple coils or earlier generation Amplatzer devices. Ultimately, the Amplatzer Vascular Plug
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
622
Jariwala et al.
Fig. 4. Coronary cine imagining during initial deployment of an Amplatzer Vascular Plug II.
II (Fig. 4, Supporting Information Movie 3) was selected despite being harder to deploy, because its three relatively large mesh disk layers increased the probability of fistula closure using a single device. Subsequent RCA angiography demonstrated closure of the fistula with minimal residual flow, and markedly improved filling of the distal RCA (Fig. 5, Supporting Information Movie 4). One month later, the patient had no cardiovascular complaints and his heart murmur resolved. Since then, he has not needed any further therapies, except for prophylactic antibiotics prescribed prior to dental procedures.
DISCUSSION
CAFs are very rare, found in 0.002% of the general population and 0.2%–0.6% of those undergoing coronary angiography [1]. They are most often congenital, but can also be acquired as a complication of invasive cardiac procedures such as coronary artery catheterization, cardiac surgery, pacemaker insertion, or endomyocardial biopsy. Most CAFs are single (90%) and arise most frequently from the RCA (50–60%), followed by the left anterior descending (25–42%), and left circumflex arteries [2]. In most cases, a CAF connects into right heart structures (57–93%), including the pulmonary artery, right atrium, or ventricle [2]. Less commonly, a CAF leads into the left heart and rarely (1%) connects directly into the SVC, as in the present case [2].
Fig. 5. Contrast angiography within the right coronary artery 10 min after initial deployment, subsequent repositioning and advancement of the Amplatzer Vascular Plug II, and withdrawal of the deployment system. There is near-closure of the coronary artery fistula with minimal residual flow and improved filling of the distal artery.
The physiologic derangements and clinical manifestations associated with CAFs depend on fistula size, tortuosity, and site. Most CAFs are small and asymptomatic and found incidentally during imaging or angiography [3]. While the natural history is highly variable, CAFs tend to grow larger with age, and can result in symptoms. In a series of 187 patients with CAF, exertional dyspnea, fatigue, or chest pain were only present in 19% of patients less than 20 years of age but in 63% of adults older than 20 years [3]. Symptoms are generally a consequence of shunting, leading to progressive right or left ventricular volume overload, heart failure, pulmonary hypertension, and/or atrial or ventricular tachyarrhythmia [3]. In addition, CAFs can lead to coronary steal—shunting of blood away from the myocardium, with subsequent myocardial ischemia or infarction [3]. Also, the coronary artery proximal to CAF or the fistula itself can become enlarged and aneurysmal, potentially leading to rupture with resultant hemopericardium and pericardial tamponade [3]. Finally, infective endocarditis can occur as in this case of our patient. However, this complication is very rare and has only been reported to occur in those with other significant cardiovascular congenital abnormalities [3,4]. To our knowledge, our report is the first case of infective endocarditis in a patient with an isolated coronary fistula leading to a structure outside of the cardiac chambers without any other associated cardiac anomalies.
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Coronary Artery Fistula Endocarditis
As demonstrated by our case, the diagnosis of a CAF complication can be difficult and delayed. Physical examination may disclose a continuous murmur over the left sternal border. Electrocardiography may show changes in QRS axis and signs of hypertrophy, and chest radiography may demonstrate cardiomegaly or pulmonary edema [3]. Echocardiography is useful, as this modality can detect significantly enlarged coronary arteries and other structural abnormalities, especially with transesophageal imaging [5]. In addition, CAF location, size, and function can be further defined with administration of echocardiographic-contrast microbubbles and the use of color flow Doppler imaging [5]. Traditionally, the gold standard for CAF detection has been invasive coronary angiography. However, while cardiac catheterization allows for hemodynamic assessment, angiography often cannot elucidate the relationship between a CAF and other cardiac structures. Therefore, complementary non-invasive coronary imaging, such as cardiac computed tomography angiography or magnetic resonance angiography, is often used to determine the origin, course, and anatomy of the CAF [5]. Spontaneous closure of CAFs due to thrombus has been reported, but it is rare [3]. Contemporary treatment guidelines recommend the closure of symptomatic CAFs. In addition, asymptomatic large CAFs (i.e., those that have a diameter three times greater than the proximal normal coronary artery) are also generally closed [1]. For small CAFs with a diameter less than two times the size of the proximal artery, treatment is more controversial, since there are no well-established criteria for closure [1]. If treatment is considered, potential options include surgical ligation with or without cardiopulmonary bypass. Alternatively, TCC can be considered using devices such as coils, double umbrella devices, vascular plugs, balloons, or covered stents [1]. In general, TCC is preferred over surgery, because the procedure is less invasive and allows for a faster recovery and a shorter hospitalization. However, some patients are not TCC candidates, especially those with CAFs that are extremely tortuous, have multiple drainage sites, involve coronary branches, or are associated with additional cardiac abnormalities that require surgical repair [6]. If TCC is performed, the rate of complete closure found on long-term follow up ranges from 81% to 91%, which is comparable to surgical intervention [6]. Procedural complications of TCC include improper device implantation or migration (7%), coronary spasm (3%), myocardial ischemia, transient arrhythmias, coronary dissection or thrombosis (3%), and death (2.2%) [1,6].
623
Post-intervention, the optimal duration of antiplatelet or anticoagulation therapy is unclear; current expert consensus suggests at least four weeks of therapy [1]. Also, infective endocarditis prophylaxis is needed with dental procedures within the first 6 months after device implantation, with life-long antibiotic prophylaxis with dental procedures for those patients with a history of endocarditis [7]. Prognosis after successful closure of CAF is excellent and most patients remain symptom free, but long-term followup is essential, especially for TCC-treated patients, as up to 20% of these patients may require repeat procedures [1].
CONCLUSION
CAFs are rare and can lead to adverse clinical outcomes, including myocardial infarction, congestive heart failure, arrhythmia, fistula rupture. Seldom, infective endocarditis can occur, but it has not previously been described to occur in patients with otherwise normal cardiovascular anatomy. Our case suggests that CAF closure may be an important part of management in such patients, since such a CAF may be a nidus for infection and lead to recurrent infective endocarditis. This case report should motivate future prospective studies of CAF patients to determine whether closure or antibiotic treatment alone should be the preferred treatment strategy in such patients. REFERENCES 1. Jama A, Barsoum M, Bjarnason H, Holmes DR Jr, Rihal CS. Percutaneous closure of congenital coronary artery fistulae: Results and angiographic follow-up. JACC Cardiovasc Interv 2011;4:814–821. 2. Dodge-Khatami A, Mavroudis C, Backer CL. Congenital heart surgery nomenclature and database project: Anomalies of the coronary arteries. Ann Thorac Surg 2000;69(4 Suppl):S270– S297. 3. Liberthson RR, Sagar K, Berkoben JP, Weintraub RM, Levine FH. Congenital coronary arteriovenous fistula. Report of 13 patients, review of the literature and delineation of management. Circulation 1979;59:849–854. 4. Stansel HC Jr, Fenn JE. Coronary arteriovenous fistula between the left coronary artery and persistent left superior vena cava complicated by bacterial endocarditis. Ann Surg 1964;160:292– 296. 5. Joshi JK, Beache GM, Slaughter MS, Sobieski MA, Schneider W, Stoddard MF. Coronary artery fistula: 64-slice computed tomographic delineation and correlation with multiplane transesophageal echocardiography and surgical findings. Echocardiography 2012;29:E69–E71. 6. Armsby LR, Keane JF, Sherwood MC, Forbess JM, Perry SB, Lock JE. Management of coronary artery fistulae. Patient
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
624
Jariwala et al.
selection and results of transcatheter closure. J Am Coll Cardiol 2002;39:1026–1032. 7. Wilson W, Taubert KA, Gewitz M, Lockhart PB, Baddour LM, Levison M, et al. Prevention of infective endocarditis: Guidelines from the american heart association: A guideline from the american heart association rheumatic fever, en-
docarditis, and kawasaki disease committee, council on cardiovascular disease in the young, and the council on clinical cardiology, council on cardiovascular surgery and anesthesia, and the quality of care and outcomes research interdisciplinary working group. Circulation 2007;116:1736– 1754.
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
An Unusual Case of Infective Endocarditis Involving a Right Coronary Artery to Superior Vena Cava Fistula Ujjval Jariwala,1,2 MD, Rani K. Hasan,1 MD, MHS, Eric M. Thorn,3 MD, and Sammy Zakaria,1* MD, MPH Coronary artery fistulas (CAFs) are rare and mostly congenital anomalous connections between a coronary artery and a cardiac chamber or great vessel. Most CAFs are small, asymptomatic, and found incidentally during cardiac imaging. However, they can lead to serious complications including myocardial infarction, congestive heart failure, arrhythmias, or fistula rupture. CAFs have been associated with infective endocarditis, but to our knowledge, this complication has never been reported involving an isolated CAF to an otherwise anatomically normal great vessel. We report the first case of this complication in a 49-year-old man with a presumed streptococcus vegetation found within an isolated large, tortuous CAF connecting the right coronary artery to the superior vena cava. After completing antibiotic treatment, transcatheter closure of the CAF was performed. Since then, the patient has remained symptom-free. This case demonstrates that CAF closure is feasible following CAF-associated endocarditis, and that closure may represent a viable strategy for reducing risk of recurrent infection. C 2014 Wiley Periodicals, Inc. V
Key words: angiography; coronary; anomalous coronaries; closure; vascular access; fistula/shunts
INTRODUCTION
A coronary artery fistula (CAF) is an abnormal connection between a coronary artery and a cardiac chamber or great vessel. While uncommon, CAFs can lead to clinically significant complications such as myocardial ischemia due to coronary steal, congestive heart failure from volume overload, arrhythmias, or fistula rupture [1]. Rarely, CAFs are associated with infective endocarditis. However, to our knowledge, there are no reports of endocarditis occurring in a patient with an isolated coronary fistula to an otherwise anatomically normal great vessel. We report the first description of this complication and subsequent transcatheter closure (TCC) of the CAF, which connected the right coronary artery (RCA) to the superior vena cava (SVC).
CASE REPORT
A 49-year-old man with diabetes mellitus complained of intermittent fever, fatigue, headache, cough, occasional dizziness, and anorexia. He had a normal chest radiograph and a negative tuberculin sensitivity skin test. He was empirically treated with seven days of clarithromycin followed by seven days of doxycycline. After six weeks, he continued to have symptoms and was hospitalized for further evaluation. On admisC 2014 Wiley Periodicals, Inc. V
sion, he had a temperature of 38.6 C (101.6 F), a blood pressure of 170/92 mm Hg, a heart rate of 129/min, and a respiratory rate of 16/min. His physical examination revealed a moderate intensity continuous murmur over the left lower sternal border, which was not previously noted. Otherwise, his physical examination was unremarkable, and he did not have any signs of heart failure. He had a normal electrocardiogram. His laboratory studies were remarkable for a white blood cell count of 11.9/lL (76% neutrophils and 14% Additional Supporting Information may be found in the online version of this article. 1
Johns Hopkins University School of Medicine, Baltimore, Maryland 2 Greater Baltimore Medical Center, Towson, Maryland 3 Virginia Cardiovascular Associates, Manassas, Virginia Conflict of interest: Nothing to report. *Correspondence to: Sammy Zakaria, MD, MPH, 4940 Eastern Avenue, Building 301, Suite 2400, Baltimore, MD 21224. E-mail: [email protected] Received 15 July 2013; Revision accepted 6 July 2014 DOI: 10.1002/ccd.25597 Published online 10 July (wileyonlinelibrary.com)
2014
in
Wiley
Online
Library
Coronary Artery Fistula Endocarditis
Fig. 1. A high esophageal transesophageal echocardiogram view demonstrating a vegetation (*) presumably near the ostium of the RCA to SVC fistula. RPA: right pulmonary artery, LA: left atrium, RA: right atrium, SVC: superior vena cava. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
Fig. 2. 3D cardiac computed tomography image highlighting a CAF originating from the RCA, following a tortuous course, and ending within the SVC.
lymphocytes), erythrocyte sedimentation rate of 101 mm/hr, and C-reactive protein of 10.4 mg/dL. Blood cultures obtained at admission grew viridans streptococci. Since endocarditis was suspected, transesophageal echocardiography (TEE) was performed. The TEE demonstrated evidence of a large CAF connecting the RCA with the SVC, coursing between the
621
Fig. 3. Contrast injection into the right coronary artery demonstrates a dilated and tortuous coronary artery fistula connecting into the superior vena cava.
aorta and the right atrium, with blood flow shunting toward the SVC based on color flow Doppler imaging. In addition, a vegetation was visualized at the junction between the fistula and the SVC (Fig. 1, Supporting Information Movie 1). The patient was then successfully treated with six weeks of intravenous ceftriaxone and vancomycin. Antibiotics were then stopped, and subsequent repeat blood cultures were negative. Since the fistula was considered a nidus for recurrent infection, the patient was referred for CAF closure. A cardiac computed tomographic angiogram (CTA) was first performed to further delineate CAF anatomy, and showed dilation and tortuosity of the proximal RCA, with fistulous communication to the anterior aspect of the SVC just above the insertion of the SVC to the right atrium (Fig. 2). The maximum diameter of fistula was 13 mm. There was a left to right shunt via the fistula with mild enlargement of the right heart chambers. Guided by the findings on CTA, the patient then underwent TCC of fistula. Initially, diagnostic angiography was performed which confirmed the RCA to SVC fistula (Fig. 3, Supporting Information Movie 2). After attempts to access the fistula from a venous approach failed, a coronary guide catheter was then successfully advanced from the right femoral artery into the fistulous tract via the RCA. Based on the angiography images, a number of closure approaches were then considered, including deploying multiple coils or earlier generation Amplatzer devices. Ultimately, the Amplatzer Vascular Plug
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
622
Jariwala et al.
Fig. 4. Coronary cine imagining during initial deployment of an Amplatzer Vascular Plug II.
II (Fig. 4, Supporting Information Movie 3) was selected despite being harder to deploy, because its three relatively large mesh disk layers increased the probability of fistula closure using a single device. Subsequent RCA angiography demonstrated closure of the fistula with minimal residual flow, and markedly improved filling of the distal RCA (Fig. 5, Supporting Information Movie 4). One month later, the patient had no cardiovascular complaints and his heart murmur resolved. Since then, he has not needed any further therapies, except for prophylactic antibiotics prescribed prior to dental procedures.
DISCUSSION
CAFs are very rare, found in 0.002% of the general population and 0.2%–0.6% of those undergoing coronary angiography [1]. They are most often congenital, but can also be acquired as a complication of invasive cardiac procedures such as coronary artery catheterization, cardiac surgery, pacemaker insertion, or endomyocardial biopsy. Most CAFs are single (90%) and arise most frequently from the RCA (50–60%), followed by the left anterior descending (25–42%), and left circumflex arteries [2]. In most cases, a CAF connects into right heart structures (57–93%), including the pulmonary artery, right atrium, or ventricle [2]. Less commonly, a CAF leads into the left heart and rarely (1%) connects directly into the SVC, as in the present case [2].
Fig. 5. Contrast angiography within the right coronary artery 10 min after initial deployment, subsequent repositioning and advancement of the Amplatzer Vascular Plug II, and withdrawal of the deployment system. There is near-closure of the coronary artery fistula with minimal residual flow and improved filling of the distal artery.
The physiologic derangements and clinical manifestations associated with CAFs depend on fistula size, tortuosity, and site. Most CAFs are small and asymptomatic and found incidentally during imaging or angiography [3]. While the natural history is highly variable, CAFs tend to grow larger with age, and can result in symptoms. In a series of 187 patients with CAF, exertional dyspnea, fatigue, or chest pain were only present in 19% of patients less than 20 years of age but in 63% of adults older than 20 years [3]. Symptoms are generally a consequence of shunting, leading to progressive right or left ventricular volume overload, heart failure, pulmonary hypertension, and/or atrial or ventricular tachyarrhythmia [3]. In addition, CAFs can lead to coronary steal—shunting of blood away from the myocardium, with subsequent myocardial ischemia or infarction [3]. Also, the coronary artery proximal to CAF or the fistula itself can become enlarged and aneurysmal, potentially leading to rupture with resultant hemopericardium and pericardial tamponade [3]. Finally, infective endocarditis can occur as in this case of our patient. However, this complication is very rare and has only been reported to occur in those with other significant cardiovascular congenital abnormalities [3,4]. To our knowledge, our report is the first case of infective endocarditis in a patient with an isolated coronary fistula leading to a structure outside of the cardiac chambers without any other associated cardiac anomalies.
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Coronary Artery Fistula Endocarditis
As demonstrated by our case, the diagnosis of a CAF complication can be difficult and delayed. Physical examination may disclose a continuous murmur over the left sternal border. Electrocardiography may show changes in QRS axis and signs of hypertrophy, and chest radiography may demonstrate cardiomegaly or pulmonary edema [3]. Echocardiography is useful, as this modality can detect significantly enlarged coronary arteries and other structural abnormalities, especially with transesophageal imaging [5]. In addition, CAF location, size, and function can be further defined with administration of echocardiographic-contrast microbubbles and the use of color flow Doppler imaging [5]. Traditionally, the gold standard for CAF detection has been invasive coronary angiography. However, while cardiac catheterization allows for hemodynamic assessment, angiography often cannot elucidate the relationship between a CAF and other cardiac structures. Therefore, complementary non-invasive coronary imaging, such as cardiac computed tomography angiography or magnetic resonance angiography, is often used to determine the origin, course, and anatomy of the CAF [5]. Spontaneous closure of CAFs due to thrombus has been reported, but it is rare [3]. Contemporary treatment guidelines recommend the closure of symptomatic CAFs. In addition, asymptomatic large CAFs (i.e., those that have a diameter three times greater than the proximal normal coronary artery) are also generally closed [1]. For small CAFs with a diameter less than two times the size of the proximal artery, treatment is more controversial, since there are no well-established criteria for closure [1]. If treatment is considered, potential options include surgical ligation with or without cardiopulmonary bypass. Alternatively, TCC can be considered using devices such as coils, double umbrella devices, vascular plugs, balloons, or covered stents [1]. In general, TCC is preferred over surgery, because the procedure is less invasive and allows for a faster recovery and a shorter hospitalization. However, some patients are not TCC candidates, especially those with CAFs that are extremely tortuous, have multiple drainage sites, involve coronary branches, or are associated with additional cardiac abnormalities that require surgical repair [6]. If TCC is performed, the rate of complete closure found on long-term follow up ranges from 81% to 91%, which is comparable to surgical intervention [6]. Procedural complications of TCC include improper device implantation or migration (7%), coronary spasm (3%), myocardial ischemia, transient arrhythmias, coronary dissection or thrombosis (3%), and death (2.2%) [1,6].
623
Post-intervention, the optimal duration of antiplatelet or anticoagulation therapy is unclear; current expert consensus suggests at least four weeks of therapy [1]. Also, infective endocarditis prophylaxis is needed with dental procedures within the first 6 months after device implantation, with life-long antibiotic prophylaxis with dental procedures for those patients with a history of endocarditis [7]. Prognosis after successful closure of CAF is excellent and most patients remain symptom free, but long-term followup is essential, especially for TCC-treated patients, as up to 20% of these patients may require repeat procedures [1].
CONCLUSION
CAFs are rare and can lead to adverse clinical outcomes, including myocardial infarction, congestive heart failure, arrhythmia, fistula rupture. Seldom, infective endocarditis can occur, but it has not previously been described to occur in patients with otherwise normal cardiovascular anatomy. Our case suggests that CAF closure may be an important part of management in such patients, since such a CAF may be a nidus for infection and lead to recurrent infective endocarditis. This case report should motivate future prospective studies of CAF patients to determine whether closure or antibiotic treatment alone should be the preferred treatment strategy in such patients. REFERENCES 1. Jama A, Barsoum M, Bjarnason H, Holmes DR Jr, Rihal CS. Percutaneous closure of congenital coronary artery fistulae: Results and angiographic follow-up. JACC Cardiovasc Interv 2011;4:814–821. 2. Dodge-Khatami A, Mavroudis C, Backer CL. Congenital heart surgery nomenclature and database project: Anomalies of the coronary arteries. Ann Thorac Surg 2000;69(4 Suppl):S270– S297. 3. Liberthson RR, Sagar K, Berkoben JP, Weintraub RM, Levine FH. Congenital coronary arteriovenous fistula. Report of 13 patients, review of the literature and delineation of management. Circulation 1979;59:849–854. 4. Stansel HC Jr, Fenn JE. Coronary arteriovenous fistula between the left coronary artery and persistent left superior vena cava complicated by bacterial endocarditis. Ann Surg 1964;160:292– 296. 5. Joshi JK, Beache GM, Slaughter MS, Sobieski MA, Schneider W, Stoddard MF. Coronary artery fistula: 64-slice computed tomographic delineation and correlation with multiplane transesophageal echocardiography and surgical findings. Echocardiography 2012;29:E69–E71. 6. Armsby LR, Keane JF, Sherwood MC, Forbess JM, Perry SB, Lock JE. Management of coronary artery fistulae. Patient
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
624
Jariwala et al.
selection and results of transcatheter closure. J Am Coll Cardiol 2002;39:1026–1032. 7. Wilson W, Taubert KA, Gewitz M, Lockhart PB, Baddour LM, Levison M, et al. Prevention of infective endocarditis: Guidelines from the american heart association: A guideline from the american heart association rheumatic fever, en-
docarditis, and kawasaki disease committee, council on cardiovascular disease in the young, and the council on clinical cardiology, council on cardiovascular surgery and anesthesia, and the quality of care and outcomes research interdisciplinary working group. Circulation 2007;116:1736– 1754.
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
