© 2014, Wiley Periodicals, Inc. DOI: 10.1111/echo.12593
Echocardiography
Two Different Presentations of Sinus of Valsalva Aneurysm Nishit Patel, M.D.,* Talla A. Rousan, M.D.,† Marvin D. Peyton, M.D.,‡ and Chittur A. Sivaram, M.D., F.A.C.C., F.A.S.E.† *Department of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma; †Cardiovascular Section, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma; and ‡Section of Thoracic Surgery, Department of Surgery, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma
Sinus of Valsalva aneurysm (SVA) is a rare cardiac anomaly that can be congenital or acquired. We report 2 cases of SVA. The first case involves a 59-year-old male presenting with frequent syncope. Echocardiogram revealed a large right SVA obstructing the right ventricular outflow tract (RVOT). The second case involves a 21-year-old female presenting with sudden onset chest pain and a continuous machinery murmur. Echocardiogram revealed a ruptured right SVA into the right atrium. Although advanced percutaneous techniques have been implemented in the correction of this anomaly, openheart surgery with or without aortic valve replacement remains the treatment of choice. (Echocardiography 2014;31:E181–E184) Key words: aneurysm, rupture, sinus of Valsalva The sinuses of Valsalva are 3 distinct outpouchings of the aortic wall associated with 3 cusps of the aortic valve. As they extend above the level of the aortic valve leaflet commissures, the 3 sinuses taper and join the tubular ascending aorta at the sino-tubular junction. The shape of the sinus of Valsalva creates a vortex within the sinuses that separates the aortic wall from edges of the aortic valve leaflets during systole. Defects in aortic media with separation of the media from aortic annulus fibrosus can lead to sinus of Valsalva aneurysm (SVA). SVA can cause complications with or without rupture. We hereby report 2 cases of SVA; a man with a large unruptured SVA causing significant obstruction of the right ventricular outflow tract (RVOT), and a young female with SVA presented with spontaneous rupture into right atrium.
and mild edema of the lower extremities. A transthoracic echocardiogram (TTE) (Philips, Amsterdam, The Netherlands) demonstrated a large right SVA protruding into the RVOT, dilated right ventricle, and atrium with right ventricular dysfunction. Transesophageal echocardiogram (TEE) (Philips) confirmed these findings as well as an anomalous left circumflex coronary artery (Figs. 1 and 2, movie clips S1 and S2). Continu-
Case Presentation: Case 1: A 59-year-old male with hypertension, and hyperlipidemia presented with frequent syncopal episodes. Physical examination demonstrated faint systolic murmur at the base of the heart, Address for correspondence and reprint requests: Chittur A. Sivaram, M.D., Cardiovascular Section, Department of Medicine, University of Oklahoma Health Sciences Center, 920 Stanton L. Young Blvd, Williams Pavilion 3010, Oklahoma City, OK 73104. Fax: +1 (405) 271-2619; E-mail: [email protected]
Figure 1. A mid-esophageal long-axis view of the left ventricle and aortic valve showing a large SVA protruding into the right ventricle (yellow arrow) and an anomalous left circumflex artery (red arrow). LV = left ventricle; AoV = aortic valve; AscAo = ascending aorta; SVA = sinus V valsalva aneurysm.
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Figure 2. A transgastric short-axis view of the right and left ventricles with an x-plane view showing a large right SVA protruding into the right ventricular outflow tract with color flow Doppler. RV = right ventricle; SVA = sinus of Valsalva aneurysm; TV = tricuspid valve; PA = pulmonary artery.
Figure 4. A mid-esophageal short-axis view showing a ruptured right sinus of Valsalva aneurysm communicating with the right atrium in a characteristic “wind sock” extension (arrow). L = left coronary cusp of aortic valve; LA = left atrium; NC = noncoronary cusp of aortic valve; R = right coronary cusp of aortic valve; RA = right atrium; RV = right ventricle.
Figure 3. Continuous-wave Doppler through the right ventricular outflow tract showing a peak gradient of 49 mmHg.
ous-wave Doppler through the RVOT revealed a peak gradient of 49 mmHg (Fig. 3). The patient underwent excision of aneurysmal sac of right SVA with aortic root replacement.
Figure 5. A mid-esophageal short-axis view with color flow Doppler showing a ruptured right sinus of Valsalva aneurysm communicating with the right atrium. L = left coronary cusp of aortic valve; LA = left atrium; NC = noncoronary cusp of aortic valve; R = right coronary cusp of aortic valve; RA = right atrium; RV = right ventricle.
Case 2: A previously healthy 21-year-old female presented with acute onset of chest pain, dyspnea, and palpitations at rest. Her annual health checkup never revealed any murmur and she was an active athlete in her high school and college. On presentation, she was tachycardic with distended jugular veins. Auscultation revealed a continuous “machinery-type” murmur best heard over the left lower sternal border. B-type natriuretic peptide was mildly elevated at 216 pg/mL (normal value 0–100 pg/mL). TEE revealed a large right SVA communicating
with the right atrium (RA) in a characteristic “wind sock” extension (Fig. 4, arrow, and Fig. 5; movie clips S3 and S4). The dimensions of the ascending aorta were within normal limits. Aortogram confirmed the rupture of SVA into the coronary sinus draining directly into the RA (Fig. 6, arrow; movie clip S5). Right heart catheterization with measurement of oxygen saturation revealed a step-up in oxygen saturation between the RA (73%) and the inferior and superior vena cavae (60% and 52%, respectively). The patient underwent pericardial patch closure of the aortic-RA fistulous tract along with tricuspid valve repair and
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Figure 6. An aortogram frame showing aortocardiac fistula with left-to-right shunt (arrow).
commissuroplasty of anterior septal commissure (moderate tricuspid regurgitation detected off pump following shortening of the height of the septal leaflet as part of SVA repair necessitated the additional tricuspid valve repair) and patent foramen ovale closure. Discussion: Sinus of Valsalva aneurysm is very rare and can be either congenital or acquired. Congenital aneurysms are thought to result from localized weakness of the elastic lamina at the junction of the aortic media and the annulus fibrosus.1 Disease processes that involve aortic root, such as syphilis, endocarditis, cystic medial necrosis, atherosclerotic aneurysms, and chest trauma may also cause SVA.1 Approximately 65–85% of SVAs arise from right sinus of Valsalva.2 Usually, an unruptured SVA is clinically silent. However, it may continue to enlarge and may lead to complication, such as compression of adjacent chamber, coronary artery compression, complete heart block, and sudden cardiac death.1 Clinically significant RVOT obstruction from right SVA is extremely rare presenting with dyspnea, angina, and symptoms and signs of right-sided heart failure.3,4 Our patient in case 1 had significant RVOT obstruction manifesting as frequent syncope. Rupture of the SVA may occur spontaneously or may be precipitated by exertion, chest trauma, or cardiac catheterization. Patients usually present with chest pain, dyspnea, and continuous “machinery-like” murmur.1,5 Rupture into the right ventricle is the most common, followed by RA, and left atrium. Without urgent surgical
intervention, there is a poor prognosis due to progressive heart failure and left-to-right shunt. Transthoracic echocardiography should be used as a first diagnostic test.6 Generally, TEE is needed to confirm the diagnosis and precise identification of the structural anomalies and shunt locations (if ruptured) for preoperative assessment. Electrocardiogram-gated contrastenhanced multisection computed tomography and multiplanar magnetic resonance imaging have been used to detect SVA with the ability of the latter technique to evaluate the left ventricular hemodynamics and to quantify any aortocardiac shunt.7 Cardiac catheterization can be used for definitive diagnosis and for measurement of oxygen saturation in the different cardiac chambers and great vessels. Our patient in case 2 showed definite increase in RA oxygen saturation compared to the superior and inferior venae cavae indicating the presence of a left-to-right shunt at that level. Rupture of SVA requires immediate surgical attention. The average life expectancy is approximately 1 year after diagnosis without surgical treatment.8 Recently, transcatheter closure of ruptured SVA using the Amplatzer duct occluder has been used successfully.9 However, traditional open-heart surgery for correction of the aneurysm and fistula, with or without aortic valve replacement still remains the treatment of choice.10 Conclusion: Sinus of Valsalva aneurysm usually remains asymptomatic and undetected, unless ruptured or aneurysm is large enough to cause RVOT obstruction. In light of fatal complications associated with both of these instances, awareness of this entity is extremely important to enable early diagnosis and treatment. Early cardiac imaging followed by immediate referral to a cardiologist can help limit morbidity and mortality associated with this condition. Despite advancement in percutaneous techniques to treat this condition, the surgical option remains the firstline treatment. References 1. Ott DA. Aneurysm of the sinus of Valsalva. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2006;9:165– 176. 2. Meier JH, Seward JB, Miller FA Jr, et al: Aneurysms in the left ventricular outflow tract: Clinical presentation, causes, and echocardiographic features. J Am Soc Echocardiogr 1998;11:729–745. 3. Thankachen R, Gnanamuthu R, Doshi H, et al: Unruptured aneurysm of the sinus of Valsalva presenting with right ventricular outflow obstruction. Tex Heart Inst J 2003;30:152–154. 4. Avci A, Akcakoyun M, Alizada E, et al: Severe right ventricular outflow obstruction by right sinus of Valsalva aneurysm. Echocardiography 2010;27:341–343.
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5. Moustafa S, Mookadam F, Cooper L, et al: Sinus of Valsalva aneurysms–47 years of a single center experience and systematic overview of published reports. Am J Cardiol 2007;99:1159–1164. 6. Terdjman M, Bourdarias JP, Farcot JC, et al: Aneurysms of sinus of Valsalva: Two-dimensional echocardiographic diagnosis and recognition of rupture into the right heart cavities. J Am Coll Cardiol 1984;3:1227–1235. 7. Bricker AO, Avutu B, Mohammed TL, et al: Valsalva sinus aneurysms: Findings at CT and MR imaging. Radiographics 2010;30:99–110. 8. Sawyers JL, Adams JE, Scott HW Jr: A method of surgical repair for ruptured aortic sinus aneurysms with aorticoatrial fistula. South Med J 1957;50:1075–1078. 9. Kerkar PG, Lanjewar CP, Mishra N, et al: Transcatheter closure of ruptured sinus of Valsalva aneurysm using the Amplatzer duct occluder: Immediate results and midterm follow-up. Eur Heart J 2010;31:2881–2887. 10. Takach TJ, Reul GJ, Duncan JM, et al: Sinus of Valsalva aneurysm or fistula: Management and outcome. Ann Thorac Surg 1999;68:1573–1577.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. A mid-esophageal long-axis clip of the left ventricle and aortic valve with color flow Doppler showing a large SVA protruding into the right ventricle and an anomalous left circumflex artery (asterisk). LV = left ventricle; AoV = aortic valve; AscAo = ascending aorta; SVA = sinus of Valsalva aneurysm.
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Movie clip S2. A transgastric short-axis view of the right and left ventricles with an x-plane view showing a large right SVA protruding into the right ventricular outflow tract with color flow Doppler. RV = right ventricle; SVA = sinus of Valsalva aneurysm; TV = tricuspid valve; PA = pulmonary artery. Movie clip S3. A mid-esophageal short-axis clip showing a ruptured right sinus of Valsalva aneurysm communicating with the right atrium in a characteristic “wind sock” extension. L = left coronary cusp of aortic valve; LA = left atrium; NC = noncoronary cusp of aortic valve; R = right coronary cusp of aortic valve; RA = right atrium; RV = right ventricle. Movie clip S4. A mid-esophageal short-axis clip with color flow Doppler showing a ruptured right sinus of Valsalva aneurysm communicating with the right atrium. L = left coronary cusp of aortic valve; LA = left atrium; NC = noncoronary cusp of aortic valve; R = right coronary cusp of aortic valve; RA = right atrium; RV = right ventricle. Movie clip S5. An aortogram done using a pigtail catheter in the ascending aorta in the left anterior oblique–cranial projection showing the aortocardiac fistula with right-to-left shunt (asterisk).
Echocardiography
Two Different Presentations of Sinus of Valsalva Aneurysm Nishit Patel, M.D.,* Talla A. Rousan, M.D.,† Marvin D. Peyton, M.D.,‡ and Chittur A. Sivaram, M.D., F.A.C.C., F.A.S.E.† *Department of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma; †Cardiovascular Section, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma; and ‡Section of Thoracic Surgery, Department of Surgery, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma
Sinus of Valsalva aneurysm (SVA) is a rare cardiac anomaly that can be congenital or acquired. We report 2 cases of SVA. The first case involves a 59-year-old male presenting with frequent syncope. Echocardiogram revealed a large right SVA obstructing the right ventricular outflow tract (RVOT). The second case involves a 21-year-old female presenting with sudden onset chest pain and a continuous machinery murmur. Echocardiogram revealed a ruptured right SVA into the right atrium. Although advanced percutaneous techniques have been implemented in the correction of this anomaly, openheart surgery with or without aortic valve replacement remains the treatment of choice. (Echocardiography 2014;31:E181–E184) Key words: aneurysm, rupture, sinus of Valsalva The sinuses of Valsalva are 3 distinct outpouchings of the aortic wall associated with 3 cusps of the aortic valve. As they extend above the level of the aortic valve leaflet commissures, the 3 sinuses taper and join the tubular ascending aorta at the sino-tubular junction. The shape of the sinus of Valsalva creates a vortex within the sinuses that separates the aortic wall from edges of the aortic valve leaflets during systole. Defects in aortic media with separation of the media from aortic annulus fibrosus can lead to sinus of Valsalva aneurysm (SVA). SVA can cause complications with or without rupture. We hereby report 2 cases of SVA; a man with a large unruptured SVA causing significant obstruction of the right ventricular outflow tract (RVOT), and a young female with SVA presented with spontaneous rupture into right atrium.
and mild edema of the lower extremities. A transthoracic echocardiogram (TTE) (Philips, Amsterdam, The Netherlands) demonstrated a large right SVA protruding into the RVOT, dilated right ventricle, and atrium with right ventricular dysfunction. Transesophageal echocardiogram (TEE) (Philips) confirmed these findings as well as an anomalous left circumflex coronary artery (Figs. 1 and 2, movie clips S1 and S2). Continu-
Case Presentation: Case 1: A 59-year-old male with hypertension, and hyperlipidemia presented with frequent syncopal episodes. Physical examination demonstrated faint systolic murmur at the base of the heart, Address for correspondence and reprint requests: Chittur A. Sivaram, M.D., Cardiovascular Section, Department of Medicine, University of Oklahoma Health Sciences Center, 920 Stanton L. Young Blvd, Williams Pavilion 3010, Oklahoma City, OK 73104. Fax: +1 (405) 271-2619; E-mail: [email protected]
Figure 1. A mid-esophageal long-axis view of the left ventricle and aortic valve showing a large SVA protruding into the right ventricle (yellow arrow) and an anomalous left circumflex artery (red arrow). LV = left ventricle; AoV = aortic valve; AscAo = ascending aorta; SVA = sinus V valsalva aneurysm.
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Figure 2. A transgastric short-axis view of the right and left ventricles with an x-plane view showing a large right SVA protruding into the right ventricular outflow tract with color flow Doppler. RV = right ventricle; SVA = sinus of Valsalva aneurysm; TV = tricuspid valve; PA = pulmonary artery.
Figure 4. A mid-esophageal short-axis view showing a ruptured right sinus of Valsalva aneurysm communicating with the right atrium in a characteristic “wind sock” extension (arrow). L = left coronary cusp of aortic valve; LA = left atrium; NC = noncoronary cusp of aortic valve; R = right coronary cusp of aortic valve; RA = right atrium; RV = right ventricle.
Figure 3. Continuous-wave Doppler through the right ventricular outflow tract showing a peak gradient of 49 mmHg.
ous-wave Doppler through the RVOT revealed a peak gradient of 49 mmHg (Fig. 3). The patient underwent excision of aneurysmal sac of right SVA with aortic root replacement.
Figure 5. A mid-esophageal short-axis view with color flow Doppler showing a ruptured right sinus of Valsalva aneurysm communicating with the right atrium. L = left coronary cusp of aortic valve; LA = left atrium; NC = noncoronary cusp of aortic valve; R = right coronary cusp of aortic valve; RA = right atrium; RV = right ventricle.
Case 2: A previously healthy 21-year-old female presented with acute onset of chest pain, dyspnea, and palpitations at rest. Her annual health checkup never revealed any murmur and she was an active athlete in her high school and college. On presentation, she was tachycardic with distended jugular veins. Auscultation revealed a continuous “machinery-type” murmur best heard over the left lower sternal border. B-type natriuretic peptide was mildly elevated at 216 pg/mL (normal value 0–100 pg/mL). TEE revealed a large right SVA communicating
with the right atrium (RA) in a characteristic “wind sock” extension (Fig. 4, arrow, and Fig. 5; movie clips S3 and S4). The dimensions of the ascending aorta were within normal limits. Aortogram confirmed the rupture of SVA into the coronary sinus draining directly into the RA (Fig. 6, arrow; movie clip S5). Right heart catheterization with measurement of oxygen saturation revealed a step-up in oxygen saturation between the RA (73%) and the inferior and superior vena cavae (60% and 52%, respectively). The patient underwent pericardial patch closure of the aortic-RA fistulous tract along with tricuspid valve repair and
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Figure 6. An aortogram frame showing aortocardiac fistula with left-to-right shunt (arrow).
commissuroplasty of anterior septal commissure (moderate tricuspid regurgitation detected off pump following shortening of the height of the septal leaflet as part of SVA repair necessitated the additional tricuspid valve repair) and patent foramen ovale closure. Discussion: Sinus of Valsalva aneurysm is very rare and can be either congenital or acquired. Congenital aneurysms are thought to result from localized weakness of the elastic lamina at the junction of the aortic media and the annulus fibrosus.1 Disease processes that involve aortic root, such as syphilis, endocarditis, cystic medial necrosis, atherosclerotic aneurysms, and chest trauma may also cause SVA.1 Approximately 65–85% of SVAs arise from right sinus of Valsalva.2 Usually, an unruptured SVA is clinically silent. However, it may continue to enlarge and may lead to complication, such as compression of adjacent chamber, coronary artery compression, complete heart block, and sudden cardiac death.1 Clinically significant RVOT obstruction from right SVA is extremely rare presenting with dyspnea, angina, and symptoms and signs of right-sided heart failure.3,4 Our patient in case 1 had significant RVOT obstruction manifesting as frequent syncope. Rupture of the SVA may occur spontaneously or may be precipitated by exertion, chest trauma, or cardiac catheterization. Patients usually present with chest pain, dyspnea, and continuous “machinery-like” murmur.1,5 Rupture into the right ventricle is the most common, followed by RA, and left atrium. Without urgent surgical
intervention, there is a poor prognosis due to progressive heart failure and left-to-right shunt. Transthoracic echocardiography should be used as a first diagnostic test.6 Generally, TEE is needed to confirm the diagnosis and precise identification of the structural anomalies and shunt locations (if ruptured) for preoperative assessment. Electrocardiogram-gated contrastenhanced multisection computed tomography and multiplanar magnetic resonance imaging have been used to detect SVA with the ability of the latter technique to evaluate the left ventricular hemodynamics and to quantify any aortocardiac shunt.7 Cardiac catheterization can be used for definitive diagnosis and for measurement of oxygen saturation in the different cardiac chambers and great vessels. Our patient in case 2 showed definite increase in RA oxygen saturation compared to the superior and inferior venae cavae indicating the presence of a left-to-right shunt at that level. Rupture of SVA requires immediate surgical attention. The average life expectancy is approximately 1 year after diagnosis without surgical treatment.8 Recently, transcatheter closure of ruptured SVA using the Amplatzer duct occluder has been used successfully.9 However, traditional open-heart surgery for correction of the aneurysm and fistula, with or without aortic valve replacement still remains the treatment of choice.10 Conclusion: Sinus of Valsalva aneurysm usually remains asymptomatic and undetected, unless ruptured or aneurysm is large enough to cause RVOT obstruction. In light of fatal complications associated with both of these instances, awareness of this entity is extremely important to enable early diagnosis and treatment. Early cardiac imaging followed by immediate referral to a cardiologist can help limit morbidity and mortality associated with this condition. Despite advancement in percutaneous techniques to treat this condition, the surgical option remains the firstline treatment. References 1. Ott DA. Aneurysm of the sinus of Valsalva. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2006;9:165– 176. 2. Meier JH, Seward JB, Miller FA Jr, et al: Aneurysms in the left ventricular outflow tract: Clinical presentation, causes, and echocardiographic features. J Am Soc Echocardiogr 1998;11:729–745. 3. Thankachen R, Gnanamuthu R, Doshi H, et al: Unruptured aneurysm of the sinus of Valsalva presenting with right ventricular outflow obstruction. Tex Heart Inst J 2003;30:152–154. 4. Avci A, Akcakoyun M, Alizada E, et al: Severe right ventricular outflow obstruction by right sinus of Valsalva aneurysm. Echocardiography 2010;27:341–343.
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5. Moustafa S, Mookadam F, Cooper L, et al: Sinus of Valsalva aneurysms–47 years of a single center experience and systematic overview of published reports. Am J Cardiol 2007;99:1159–1164. 6. Terdjman M, Bourdarias JP, Farcot JC, et al: Aneurysms of sinus of Valsalva: Two-dimensional echocardiographic diagnosis and recognition of rupture into the right heart cavities. J Am Coll Cardiol 1984;3:1227–1235. 7. Bricker AO, Avutu B, Mohammed TL, et al: Valsalva sinus aneurysms: Findings at CT and MR imaging. Radiographics 2010;30:99–110. 8. Sawyers JL, Adams JE, Scott HW Jr: A method of surgical repair for ruptured aortic sinus aneurysms with aorticoatrial fistula. South Med J 1957;50:1075–1078. 9. Kerkar PG, Lanjewar CP, Mishra N, et al: Transcatheter closure of ruptured sinus of Valsalva aneurysm using the Amplatzer duct occluder: Immediate results and midterm follow-up. Eur Heart J 2010;31:2881–2887. 10. Takach TJ, Reul GJ, Duncan JM, et al: Sinus of Valsalva aneurysm or fistula: Management and outcome. Ann Thorac Surg 1999;68:1573–1577.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. A mid-esophageal long-axis clip of the left ventricle and aortic valve with color flow Doppler showing a large SVA protruding into the right ventricle and an anomalous left circumflex artery (asterisk). LV = left ventricle; AoV = aortic valve; AscAo = ascending aorta; SVA = sinus of Valsalva aneurysm.
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Movie clip S2. A transgastric short-axis view of the right and left ventricles with an x-plane view showing a large right SVA protruding into the right ventricular outflow tract with color flow Doppler. RV = right ventricle; SVA = sinus of Valsalva aneurysm; TV = tricuspid valve; PA = pulmonary artery. Movie clip S3. A mid-esophageal short-axis clip showing a ruptured right sinus of Valsalva aneurysm communicating with the right atrium in a characteristic “wind sock” extension. L = left coronary cusp of aortic valve; LA = left atrium; NC = noncoronary cusp of aortic valve; R = right coronary cusp of aortic valve; RA = right atrium; RV = right ventricle. Movie clip S4. A mid-esophageal short-axis clip with color flow Doppler showing a ruptured right sinus of Valsalva aneurysm communicating with the right atrium. L = left coronary cusp of aortic valve; LA = left atrium; NC = noncoronary cusp of aortic valve; R = right coronary cusp of aortic valve; RA = right atrium; RV = right ventricle. Movie clip S5. An aortogram done using a pigtail catheter in the ascending aorta in the left anterior oblique–cranial projection showing the aortocardiac fistula with right-to-left shunt (asterisk).
