Case Study
Giant coronary artery fistula Mohammed Almansori1 and Muhammed Tamim2
Asian Cardiovascular & Thoracic Annals 2014, Vol. 22(5) 595–597 ß The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492313478627 aan.sagepub.com
Abstract Giant coronary artery fistula is rare. We describe the diagnostic work-up and surgical management of a 55-year-old woman who presented with congestive heart failure caused by a giant coronary artery fistula from the left circumflex artery to the coronary sinus.
Keywords Arteriovenous fistula, Coronary aneurysm, Coronary vessel anomalies
Introduction Huge coronary artery fistulas are extremely rare, and the management has not been clearly defined. We describe the diagnostic work-up and surgical management of a case of congestive heart failure caused by a giant coronary artery fistula from the left circumflex artery to the coronary sinus.
Case report A 55-year-old woman presented with exertional shortness of breath, orthopnea, and paroxysmal nocturnal dyspnea for 3 months. She did not describe chest pain or palpitations, and was not known to have any chronic illnesses. Physical examination showed a heart rate of 90 beats per minute, blood pressure of 120/60 mm Hg, and jugular venous pressure 14 cm H2O. Chest auscultation revealed bilateral basal fine crepitations, normal first and second heart sounds, S3 gallop and a pansystolic murmur of mitral regurgitation. Her lower limbs had pitting edema up to the middle of the legs. An electrocardiogram was normal, a chest radiograph showed pulmonary congestion and cardiomegaly, and an echocardiogram revealed a dilated left ventricle with a moderate reduction in systolic function and a normal right ventricle and atrium. There was a tortuous structure in the pericardium with abnormal flow, suggestive of a fistula. Right heart catheterization demonstrated a step-up in oxygen saturation at the level of the right atrium, with a pulmonary-to-systemic flow ratio of 2.0. The mean pulmonary artery pressure was 28 mm Hg, and the pulmonary capillary wedge pressure
was 16 mm Hg. The calculated (Fick) cardiac output was 6.2 L min 1. A coronary angiogram showed a dominant right coronary artery and a left anterior descending artery that was ectatic proximally and free of disease distally; the left circumflex (LCx) artery was not identified. An aortic root angiogram revealed the LCx to be a giant and tortuous vessel communicating with the right atrium. For further delineation of the left main coronary artery anatomy and the exact site of the fistulous communication, coronary computed tomographic angiography was performed (Figure 1), which showed the left main coronary artery originating at the usual site in the left coronary cusp, but 3 mm from its origin, the left main became huge and ectatic and immediately divided into the left anterior descending artery and the LCx. The LCx was huge and very tortuous, twisting few times before joining the coronary sinus, which was also dilated. The LCx measured 5.5 cm at its largest dilated segment and gave rise to an early obtuse marginal branch. Given the potential risk of rupture of this abnormally dilated vessel and the significant left-to-right shunt as well as left ventricular 1 Department of Medicine, Division of Cardiology, University of Dammam, Dammam, Saudi Arabia 2 Department of Cardiology and Cardiac Surgery, King Fahad Medical Military Complex, Dhahran, Saudi Arabia
Corresponding author: Mohammed Almansori, MD, Department of Medicine, Division of Cardiology, University of Dammam, PO Box 12875, Dammam 31483, Saudi Arabia. Email: [email protected]
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Asian Cardiovascular & Thoracic Annals 22(5)
Figure 1. Reconstructed coronary artery computed tomographic images: (a) sagittal view showing the anatomy of the left main coronary artery bifurcation, (b) short-axis view showing how tortuous the fistula was around the left ventricle, (c) and 3-dimensional reconstructed images showing the anatomy at the terminal portion of the coronary sinus and (d) A view from the apex of the heart demonstrating the tortuosity and the aneurysmal dilatation of the fistula. LAD: left anterior descending artery; LCx: left circumflex coronary artery; LM: left main coronary artery.
systolic dysfunction, the patient was referred for surgical ligation of the shunt. The chest was opened via a median sternotomy, and manual palpation over the extensively dilated coronary sinus revealed a continuous thrill. Because of the difficult location of the fistula and the presence of the aneurysm, cardiopulmonary bypass was instituted. The LCx was very tortuous with a huge saccular aneurysm (5–6 cm) in its middle segment. Myocardial protection was achieved with antegrade cold blood cardioplegia and systemic hypothermia of 32 C. A tissue stabilizer (Urchin Evo Heart Positioner; Medtronic) was used to facilitate heart traction towards the surgeon. The coronary sinus was opened longitudinally, and considerable time was needed to search for the fistulous communication from within the coronary sinus; therefore, the dilated segment of the LCx was opened longitudinally (Figure 2). The artery wall was calcified and no clots were seen inside the aneurysm. The fistula was found at the terminal part of the LCx, communicating with the coronary sinus, which was closed using interrupted pledgeted 4/0 Prolene sutures. Due to the huge size of the LCx and the risk of rupture after fistula closure, we decided
to close the artery from its proximal origin, using continuous suture. Revascularization was performed with a saphenous vein graft to the obtuse marginal artery. The coronary sinus was closed with a running 6/0 Prolene suture. Weaning from cardiopulmonary bypass was smooth, with only a small dose of inotropics. Intraoperative transesophageal echocardiography showed no residual shunt. Follow-up echocardiography 5 months after surgery showed normalization of left ventricular size and function.
Discussion Coronary anomalies are seen in 1.3% of coronary angiograms; 10%–13% of these anomalies are coronary artery fistulas.1 Huge coronary artery fistulas are extremely rare, and only a few case reports have been described.2 Most coronary artery fistulas are asymptomatic and are found incidentally during coronary angiography; however, approximately 20% of these fistulas are clinically relevant.1,3 Our patient presented with congestive heart failure. Although arteriovenous fistulas are known to cause
Downloaded from aan.sagepub.com at UNIV OF NORTH DAKOTA on May 26, 2015
Almansori and Tamim
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Figure 2. Intraoperative images showing (a) aneurysmal dilatation of the left circumflex artery, and (b) the site of the fistulous communication (*) between the left circumflex artery and the coronary sinus. LCx: left circumflex artery.
high-output heart failure, it is unlikely to be the only mechanism of left ventricular dysfunction in our patient because her cardiac output was within the normal range. It is possible that high flow in the fistula resulted in a coronary steal phenomenon with subsequent ischemia and dysfunction of the left ventricle. Given the rarity of such a condition and the anatomical variation of these fistulas, there are no guidelines developed to aid in the management of such patients. In our case, the presence of left ventricular dysfunction, a significant shunt, and the potential risk of rupture were more than enough to refer the patient for surgery with the aim of ligating the fistula and bypassing the obtuse marginal artery.2,4 A transcatheter approach to coronary artery fistula is an attractive alternative treatment option.5 However, this modality of treatment might be suitable for smaller and nonaneurysmal coronary artery fistulas. Multiple imaging techniques, including echocardiography, coronary artery computed tomography, and invasive angiography utilized to delineate the exact anatomy of these tortuous fistulas, were of great help to the surgeon in planning the approach to the fistula and minimizing the time of the surgery.
Conflict of interest statement None declared.
References 1. Yamanaka O and Hobbs RE. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. Cathet Cardiovasc Diagn 1990; 21: 28–40. 2. Darwazah AK, Eida M, Batrawy M, Isleem I and Hanbali N. Surgical treatment of circumflex coronary aneurysm with fistulous connection to coronary sinus associated with persistent left superior vena cava. J Card Surg 2011; 26: 608–612. 3. Yildiz A, Okcun B, Peker T, Arslan C, Olcay A and Bulent Vatan M. Prevalence of coronary artery anomalies in 12,457 adult patients who underwent coronary angiography. Clin Cardiol 2010; 33: E60–E64. 4. Hajj-Chahine J, Haddad F, El-Rassi I and Jebara V. Surgical management of a circumflex aneurysm with fistula to the coronary sinus. Eur J Cardiothorac Surg 2009; 35: 1086–1088. 5. Kabbani Z, Garcia-Nielsen L, Lozano ML, Febles T, Febles-Bethencourt L and Castro A. Coil embolization of coronary artery fistulas. A single-centre experience. Cardiovasc Revasc Med 2008; 9: 14–17.
Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Downloaded from aan.sagepub.com at UNIV OF NORTH DAKOTA on May 26, 2015
Giant coronary artery fistula Mohammed Almansori1 and Muhammed Tamim2
Asian Cardiovascular & Thoracic Annals 2014, Vol. 22(5) 595–597 ß The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492313478627 aan.sagepub.com
Abstract Giant coronary artery fistula is rare. We describe the diagnostic work-up and surgical management of a 55-year-old woman who presented with congestive heart failure caused by a giant coronary artery fistula from the left circumflex artery to the coronary sinus.
Keywords Arteriovenous fistula, Coronary aneurysm, Coronary vessel anomalies
Introduction Huge coronary artery fistulas are extremely rare, and the management has not been clearly defined. We describe the diagnostic work-up and surgical management of a case of congestive heart failure caused by a giant coronary artery fistula from the left circumflex artery to the coronary sinus.
Case report A 55-year-old woman presented with exertional shortness of breath, orthopnea, and paroxysmal nocturnal dyspnea for 3 months. She did not describe chest pain or palpitations, and was not known to have any chronic illnesses. Physical examination showed a heart rate of 90 beats per minute, blood pressure of 120/60 mm Hg, and jugular venous pressure 14 cm H2O. Chest auscultation revealed bilateral basal fine crepitations, normal first and second heart sounds, S3 gallop and a pansystolic murmur of mitral regurgitation. Her lower limbs had pitting edema up to the middle of the legs. An electrocardiogram was normal, a chest radiograph showed pulmonary congestion and cardiomegaly, and an echocardiogram revealed a dilated left ventricle with a moderate reduction in systolic function and a normal right ventricle and atrium. There was a tortuous structure in the pericardium with abnormal flow, suggestive of a fistula. Right heart catheterization demonstrated a step-up in oxygen saturation at the level of the right atrium, with a pulmonary-to-systemic flow ratio of 2.0. The mean pulmonary artery pressure was 28 mm Hg, and the pulmonary capillary wedge pressure
was 16 mm Hg. The calculated (Fick) cardiac output was 6.2 L min 1. A coronary angiogram showed a dominant right coronary artery and a left anterior descending artery that was ectatic proximally and free of disease distally; the left circumflex (LCx) artery was not identified. An aortic root angiogram revealed the LCx to be a giant and tortuous vessel communicating with the right atrium. For further delineation of the left main coronary artery anatomy and the exact site of the fistulous communication, coronary computed tomographic angiography was performed (Figure 1), which showed the left main coronary artery originating at the usual site in the left coronary cusp, but 3 mm from its origin, the left main became huge and ectatic and immediately divided into the left anterior descending artery and the LCx. The LCx was huge and very tortuous, twisting few times before joining the coronary sinus, which was also dilated. The LCx measured 5.5 cm at its largest dilated segment and gave rise to an early obtuse marginal branch. Given the potential risk of rupture of this abnormally dilated vessel and the significant left-to-right shunt as well as left ventricular 1 Department of Medicine, Division of Cardiology, University of Dammam, Dammam, Saudi Arabia 2 Department of Cardiology and Cardiac Surgery, King Fahad Medical Military Complex, Dhahran, Saudi Arabia
Corresponding author: Mohammed Almansori, MD, Department of Medicine, Division of Cardiology, University of Dammam, PO Box 12875, Dammam 31483, Saudi Arabia. Email: [email protected]
Downloaded from aan.sagepub.com at UNIV OF NORTH DAKOTA on May 26, 2015
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Asian Cardiovascular & Thoracic Annals 22(5)
Figure 1. Reconstructed coronary artery computed tomographic images: (a) sagittal view showing the anatomy of the left main coronary artery bifurcation, (b) short-axis view showing how tortuous the fistula was around the left ventricle, (c) and 3-dimensional reconstructed images showing the anatomy at the terminal portion of the coronary sinus and (d) A view from the apex of the heart demonstrating the tortuosity and the aneurysmal dilatation of the fistula. LAD: left anterior descending artery; LCx: left circumflex coronary artery; LM: left main coronary artery.
systolic dysfunction, the patient was referred for surgical ligation of the shunt. The chest was opened via a median sternotomy, and manual palpation over the extensively dilated coronary sinus revealed a continuous thrill. Because of the difficult location of the fistula and the presence of the aneurysm, cardiopulmonary bypass was instituted. The LCx was very tortuous with a huge saccular aneurysm (5–6 cm) in its middle segment. Myocardial protection was achieved with antegrade cold blood cardioplegia and systemic hypothermia of 32 C. A tissue stabilizer (Urchin Evo Heart Positioner; Medtronic) was used to facilitate heart traction towards the surgeon. The coronary sinus was opened longitudinally, and considerable time was needed to search for the fistulous communication from within the coronary sinus; therefore, the dilated segment of the LCx was opened longitudinally (Figure 2). The artery wall was calcified and no clots were seen inside the aneurysm. The fistula was found at the terminal part of the LCx, communicating with the coronary sinus, which was closed using interrupted pledgeted 4/0 Prolene sutures. Due to the huge size of the LCx and the risk of rupture after fistula closure, we decided
to close the artery from its proximal origin, using continuous suture. Revascularization was performed with a saphenous vein graft to the obtuse marginal artery. The coronary sinus was closed with a running 6/0 Prolene suture. Weaning from cardiopulmonary bypass was smooth, with only a small dose of inotropics. Intraoperative transesophageal echocardiography showed no residual shunt. Follow-up echocardiography 5 months after surgery showed normalization of left ventricular size and function.
Discussion Coronary anomalies are seen in 1.3% of coronary angiograms; 10%–13% of these anomalies are coronary artery fistulas.1 Huge coronary artery fistulas are extremely rare, and only a few case reports have been described.2 Most coronary artery fistulas are asymptomatic and are found incidentally during coronary angiography; however, approximately 20% of these fistulas are clinically relevant.1,3 Our patient presented with congestive heart failure. Although arteriovenous fistulas are known to cause
Downloaded from aan.sagepub.com at UNIV OF NORTH DAKOTA on May 26, 2015
Almansori and Tamim
597
Figure 2. Intraoperative images showing (a) aneurysmal dilatation of the left circumflex artery, and (b) the site of the fistulous communication (*) between the left circumflex artery and the coronary sinus. LCx: left circumflex artery.
high-output heart failure, it is unlikely to be the only mechanism of left ventricular dysfunction in our patient because her cardiac output was within the normal range. It is possible that high flow in the fistula resulted in a coronary steal phenomenon with subsequent ischemia and dysfunction of the left ventricle. Given the rarity of such a condition and the anatomical variation of these fistulas, there are no guidelines developed to aid in the management of such patients. In our case, the presence of left ventricular dysfunction, a significant shunt, and the potential risk of rupture were more than enough to refer the patient for surgery with the aim of ligating the fistula and bypassing the obtuse marginal artery.2,4 A transcatheter approach to coronary artery fistula is an attractive alternative treatment option.5 However, this modality of treatment might be suitable for smaller and nonaneurysmal coronary artery fistulas. Multiple imaging techniques, including echocardiography, coronary artery computed tomography, and invasive angiography utilized to delineate the exact anatomy of these tortuous fistulas, were of great help to the surgeon in planning the approach to the fistula and minimizing the time of the surgery.
Conflict of interest statement None declared.
References 1. Yamanaka O and Hobbs RE. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. Cathet Cardiovasc Diagn 1990; 21: 28–40. 2. Darwazah AK, Eida M, Batrawy M, Isleem I and Hanbali N. Surgical treatment of circumflex coronary aneurysm with fistulous connection to coronary sinus associated with persistent left superior vena cava. J Card Surg 2011; 26: 608–612. 3. Yildiz A, Okcun B, Peker T, Arslan C, Olcay A and Bulent Vatan M. Prevalence of coronary artery anomalies in 12,457 adult patients who underwent coronary angiography. Clin Cardiol 2010; 33: E60–E64. 4. Hajj-Chahine J, Haddad F, El-Rassi I and Jebara V. Surgical management of a circumflex aneurysm with fistula to the coronary sinus. Eur J Cardiothorac Surg 2009; 35: 1086–1088. 5. Kabbani Z, Garcia-Nielsen L, Lozano ML, Febles T, Febles-Bethencourt L and Castro A. Coil embolization of coronary artery fistulas. A single-centre experience. Cardiovasc Revasc Med 2008; 9: 14–17.
Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Downloaded from aan.sagepub.com at UNIV OF NORTH DAKOTA on May 26, 2015
