© 2015, Wiley Periodicals, Inc. DOI: 10.1111/echo.12920
Echocardiography
Unruptured Sinus of Valsalva Aneurysm with Right Ventricular Outflow Tract Obstruction and Ventricular Septal Defect—A Rare Combination Sambhunath Das, M.D.,* Suruchi Ladha, M.D.,* and Balram Airan, M.Ch.† *Department of Cardiac Anaesthesia, AIIMS, New Delhi, India; and †Cardio Thoracic and Vascular Surgery, AIIMS, New Delhi, India
Sinus of Valsalva aneurysms are rare cardiac anomalies that may be acquired or congenital. We describe the case of a patient who had an unruptured sinus of Valsalva aneurysm causing right ventricular outflow obstruction, with an associated subaortic ventricular septal defect. Sinus of Valsalva aneurysms rarely present until rupture occurs. However, the unruptured aneurysm of the right sinus of Valsalva caused significant right ventricular outflow tract obstruction, resulting in exertional dyspnea. Intraoperative multiplane transesophageal echocardiography and color Doppler helped in precise identification of structural anomalies, shunt location, and definitive surgical repair. (Echocardiography 2015;32:1322–1324) Key words: right ventricular outflow tract obstruction, sinus of Valsalva aneurysms, ventricular septal defect, transesophageal echocardiography
Sinus of Valsalva aneurysm (SVA) is an uncommon cardiac anomaly that may be congenital or acquired.1 Congenital SVA is usually clinically silent but may vary from a mild, asymptomatic dilatation detected in routine 2D echocardiography to symptomatic presentations. Associated structural defects in congenital SVAs include ventricular septal defect (VSD) (30– 60%), bicuspid aortic valve (15–20%), and aortic regurgitation (AR) (44–50%).1 Transesophageal echocardiography (TEE) and color Doppler can help in precise identification of associated structural anomaly and shunt locations. We report a rare case of unruptured SVA associated with VSD and right ventricular outflow tract (RVOT) obstruction. Case Report: A 34-year-old woman presented to our institute with 1-year history of progressing dyspnea on exertion and palpitation. On clinical examination, the patient had raised jugular venous pressure and pedal edema. Her pulse rate was 120/ min, blood pressure was 110/50 mmHg, and chest auscultation revealed a diastolic murmur in Address for correspondence and reprint requests: Dr Sambhunath Das, Additional Professor, Department of Cardiac Anaesthesia, 7th Floor, CN Center, AIIMS, Ansari Nagar, New Delhi 110029, India. Fax: +91-11-26588641; E-mail: [email protected]
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aortic area and a systolic murmur over lower left sternal border. The liver was enlarged on abdominal examination. The chest x-ray showed mildly dilated aortic root and cardiomegaly. Transthoracic echocardiography revealed a large aneurysm of the right coronary sinus of Valsalva with obstruction of the RVOT, moderate AR, mild tricuspid regurgitation (TR), and a subaortic outlet VSD. Cardiac catheterization showed a large aneurysm originating from right coronary cusp (RCC) and occluding the RVOT. The peak systolic right ventricular (RV) pressure was 88 mmHg and the peak pulmonary artery (PA) pressure was 15 mmHg; there was RV-to-PA gradient of 73 mmHg. Oxygen saturation measurements showed significant step-up increase from 73% in RA to 90% in PA. However, it was difficult to determine whether the step-up in PA was from a leaking aneurysm or due to unruptured aneurysm with VSD. Intraoperative TEE revealed the presence of a large SVA arising from the RCC and causing a significant RVOT obstruction with a peak gradient of 60 mmHg in continuous wave Doppler (Figs. 1 and 2). Echocardiography also detected mild TR. There was no evidence to suggest a “wind sock” effect, no evidence of rupture, or of any fistulous communication between the aneurysm and any cardiac chambers. However, it was associated with an outlet VSD (Figs. 3 and 4).
Unruptured Sinus of Valsalva Aneurysm
Figure 1. Transesophageal echocardiography 2D and color Doppler compare mid-esophageal aortic valve short-axis view showing the right coronary sinus of Valsalva aneurysm. RA = right atrium; LA = left atrium; NCC = noncoronary cusp; LCC = left coronary cusp; and RCC = right coronary cusp.
Figure 2. Transesophageal echocardiography 2D and color Doppler compare midesophageal short-axis right ventricle inflow outflow view showing the RVOT obstruction by sinus of Valsalva aneurysm.
Figure 4. Transesophageal echocardiography 2D and color Doppler compare midesophageal aortic valve long-axis view showing the unruptured sinus of Valsalva aneurysm and VSD. SV An = sinus of Valsalva aneurysm and VSD = ventricular septal defect.
Figure 5. Transesophageal echocardiography 2D and color Doppler compare midesophageal five chamber view showing severe aortic regurgitation. LA = left atrium and AV = aortic valve.
Figure 3. Transesophageal echocardiography 3D live midesophageal aortic valve long-axis view showing the sinus of Valsalva aneurysm and VSD. SV = sinus of Valsalva and VSD = ventricular septal defect.
Figure 6. Postoperative transesophageal echocardiography 2D and color Doppler compare midesophageal RVOT view showing no obstruction. RVOT = right ventricular outflow tract.
The presence of a large SVA had led to thickening and inadequate closure of the cusps of aortic valve resulting in severe AR (Fig. 5). Surgery confirmed the TEE findings. The right coronary sinus was aneurysmal, unruptured
and obstructed the RVOT. The aneurysm was projecting through an outlet VSD of 1 cm diameter in the RVOT. The surgical approach was planned both through an aortic and right ventricular approach. Myocardial preservation was 1323
Das, et al.
achieved by ostial infusion of cold cardioplegia and systemic hypothermia. SVA was everted and excised. The outlet VSD was repaired with transaortic direct pledget suture closure, and the aortic valve was replaced with a 19 mm St. Jude Medical bileaflet prosthetic valve. The postoperative TEE examination confirmed complete repair of SVA and no RVOT obstruction (Fig. 6). Postoperatively, the patient had an uneventful recovery and was discharged from the hospital on the seventh postoperative day.
detection if the wall of the aneurysm or the prolapsing cusp of the aortic valve occludes it. Transthoracic echocardiography and TEE have a diagnostic accuracy of 75% and 90%, respectively, for ruptured or unruptured SVA.7,8 In our case intraoperative TEE provided conclusive information regarding the origin and size of the aneurysm, the adequacy of surgical repair of SVA, relief of RVOT obstruction, successful closure of VSD, and correct surgical placement of prosthetic aortic valve.
Discussion: Sinus of Valsalva aneurysm may be congenital or acquired.1 In our case, the patient had no history of risk factors for acquired aneurysm such as Marfan syndrome, atherosclerosis, syphilis, infective endocarditis, cystic medial sclerosis, chest trauma, and hypereosinophilic syndrome.1 She was asymptomatic during her childhood, and she experienced progressive exertional dyspnea. Therefore, we regarded the etiology of her condition to be congenital. The slowly progressive nature of aneurysm eventually led to significant RVOT obstruction. There have been a few reported cases in which patients with an unruptured SVA have presented with exertional dyspnea, palpitations, and angina-like chest pain.1,2 Unruptured aneurysms of the RCC have been found to cause pulmonary insufficiency, RVOT obstruction, myocardial ischemia, conduction disturbances, and TR.2–4 A coexisting VSD has also been reported.5 The association between the right and noncoronary SVA and VSD has been proposed to result from incomplete fusion between the right and left distal bulbous septum.6 Cardiac catheterization and angiography are generally performed to analyze the aneurysm and the presence of associated anomalies, particularly VSD, AR, and pulmonary stenosis. The true size of the VSD cannot be estimated angiographically when RCC is prolapsed into the VSD. In our case, there was difficulty in determining the presence of VSD with cardiac catheterization. Two-dimensional and Doppler echocardiography can adequately show unruptured SVA. However, an associated VSD may occasionally escape
Conclusion: The combined presence of SVA and VSD, resulting in RVOT obstruction, AR and TR is a very rare combination. Intraoperative TEE is helpful to confirm the diagnosis, detect the associated anomalies, plan the surgical approach, and assess the adequacy of surgical correction.
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References 1. Feldman DN, Roman MJ: Aneurysms of the sinuses of Valsalva. Cardiology 2006;106:73–81. 2. 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. 3. Gavali SA, Phadke MS, Kerkar PG, et al: Unruptured aneurysm of the sinus of Valsalva presenting with right ventricular outflow tract obstruction, complete heart block, and protrusion into left ventricular outflow tract. J Am Coll Cardiol 2013;61:e169. 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. 5. Liang CD, Chang JP, Kao CL: Unruptured sinus of Valsalva aneurysm with right ventricular out flow tract obstruction associated with ventricular septal defect. Cathet Cardiovasc Diagn 1996;37:158–161. 6. Kirklin JW, Barratt-Boyes BG. Congenital aneurysm of the sinus of Valsalva. In: Kirklin JW, Barratt-Boyes BG (eds) Cardiac Surgery. Morphology, Diagnostic Criteria, Natural History, Techniques, Results, and Indications, 2nd edn. New York: Churchill Livingstone, 1993, p. 825–840. 7. Wang K-Y, St. John Sutton M, Ho H-Y, et al: Congenital sinus of Valsalva aneurysm: a multiplane transesophageal echocardiographic experience. J Am Soc Echocardiogr 1997;10:956–63. 8. Abdelkhirane C, Roudaut R, Dallocchio M. Diagnosis of ruptured sinus of Valsalva aneurysms: potential value of transesophageal echocardiography. Echocardiography 1990;7:555–560.
Echocardiography
Unruptured Sinus of Valsalva Aneurysm with Right Ventricular Outflow Tract Obstruction and Ventricular Septal Defect—A Rare Combination Sambhunath Das, M.D.,* Suruchi Ladha, M.D.,* and Balram Airan, M.Ch.† *Department of Cardiac Anaesthesia, AIIMS, New Delhi, India; and †Cardio Thoracic and Vascular Surgery, AIIMS, New Delhi, India
Sinus of Valsalva aneurysms are rare cardiac anomalies that may be acquired or congenital. We describe the case of a patient who had an unruptured sinus of Valsalva aneurysm causing right ventricular outflow obstruction, with an associated subaortic ventricular septal defect. Sinus of Valsalva aneurysms rarely present until rupture occurs. However, the unruptured aneurysm of the right sinus of Valsalva caused significant right ventricular outflow tract obstruction, resulting in exertional dyspnea. Intraoperative multiplane transesophageal echocardiography and color Doppler helped in precise identification of structural anomalies, shunt location, and definitive surgical repair. (Echocardiography 2015;32:1322–1324) Key words: right ventricular outflow tract obstruction, sinus of Valsalva aneurysms, ventricular septal defect, transesophageal echocardiography
Sinus of Valsalva aneurysm (SVA) is an uncommon cardiac anomaly that may be congenital or acquired.1 Congenital SVA is usually clinically silent but may vary from a mild, asymptomatic dilatation detected in routine 2D echocardiography to symptomatic presentations. Associated structural defects in congenital SVAs include ventricular septal defect (VSD) (30– 60%), bicuspid aortic valve (15–20%), and aortic regurgitation (AR) (44–50%).1 Transesophageal echocardiography (TEE) and color Doppler can help in precise identification of associated structural anomaly and shunt locations. We report a rare case of unruptured SVA associated with VSD and right ventricular outflow tract (RVOT) obstruction. Case Report: A 34-year-old woman presented to our institute with 1-year history of progressing dyspnea on exertion and palpitation. On clinical examination, the patient had raised jugular venous pressure and pedal edema. Her pulse rate was 120/ min, blood pressure was 110/50 mmHg, and chest auscultation revealed a diastolic murmur in Address for correspondence and reprint requests: Dr Sambhunath Das, Additional Professor, Department of Cardiac Anaesthesia, 7th Floor, CN Center, AIIMS, Ansari Nagar, New Delhi 110029, India. Fax: +91-11-26588641; E-mail: [email protected]
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aortic area and a systolic murmur over lower left sternal border. The liver was enlarged on abdominal examination. The chest x-ray showed mildly dilated aortic root and cardiomegaly. Transthoracic echocardiography revealed a large aneurysm of the right coronary sinus of Valsalva with obstruction of the RVOT, moderate AR, mild tricuspid regurgitation (TR), and a subaortic outlet VSD. Cardiac catheterization showed a large aneurysm originating from right coronary cusp (RCC) and occluding the RVOT. The peak systolic right ventricular (RV) pressure was 88 mmHg and the peak pulmonary artery (PA) pressure was 15 mmHg; there was RV-to-PA gradient of 73 mmHg. Oxygen saturation measurements showed significant step-up increase from 73% in RA to 90% in PA. However, it was difficult to determine whether the step-up in PA was from a leaking aneurysm or due to unruptured aneurysm with VSD. Intraoperative TEE revealed the presence of a large SVA arising from the RCC and causing a significant RVOT obstruction with a peak gradient of 60 mmHg in continuous wave Doppler (Figs. 1 and 2). Echocardiography also detected mild TR. There was no evidence to suggest a “wind sock” effect, no evidence of rupture, or of any fistulous communication between the aneurysm and any cardiac chambers. However, it was associated with an outlet VSD (Figs. 3 and 4).
Unruptured Sinus of Valsalva Aneurysm
Figure 1. Transesophageal echocardiography 2D and color Doppler compare mid-esophageal aortic valve short-axis view showing the right coronary sinus of Valsalva aneurysm. RA = right atrium; LA = left atrium; NCC = noncoronary cusp; LCC = left coronary cusp; and RCC = right coronary cusp.
Figure 2. Transesophageal echocardiography 2D and color Doppler compare midesophageal short-axis right ventricle inflow outflow view showing the RVOT obstruction by sinus of Valsalva aneurysm.
Figure 4. Transesophageal echocardiography 2D and color Doppler compare midesophageal aortic valve long-axis view showing the unruptured sinus of Valsalva aneurysm and VSD. SV An = sinus of Valsalva aneurysm and VSD = ventricular septal defect.
Figure 5. Transesophageal echocardiography 2D and color Doppler compare midesophageal five chamber view showing severe aortic regurgitation. LA = left atrium and AV = aortic valve.
Figure 3. Transesophageal echocardiography 3D live midesophageal aortic valve long-axis view showing the sinus of Valsalva aneurysm and VSD. SV = sinus of Valsalva and VSD = ventricular septal defect.
Figure 6. Postoperative transesophageal echocardiography 2D and color Doppler compare midesophageal RVOT view showing no obstruction. RVOT = right ventricular outflow tract.
The presence of a large SVA had led to thickening and inadequate closure of the cusps of aortic valve resulting in severe AR (Fig. 5). Surgery confirmed the TEE findings. The right coronary sinus was aneurysmal, unruptured
and obstructed the RVOT. The aneurysm was projecting through an outlet VSD of 1 cm diameter in the RVOT. The surgical approach was planned both through an aortic and right ventricular approach. Myocardial preservation was 1323
Das, et al.
achieved by ostial infusion of cold cardioplegia and systemic hypothermia. SVA was everted and excised. The outlet VSD was repaired with transaortic direct pledget suture closure, and the aortic valve was replaced with a 19 mm St. Jude Medical bileaflet prosthetic valve. The postoperative TEE examination confirmed complete repair of SVA and no RVOT obstruction (Fig. 6). Postoperatively, the patient had an uneventful recovery and was discharged from the hospital on the seventh postoperative day.
detection if the wall of the aneurysm or the prolapsing cusp of the aortic valve occludes it. Transthoracic echocardiography and TEE have a diagnostic accuracy of 75% and 90%, respectively, for ruptured or unruptured SVA.7,8 In our case intraoperative TEE provided conclusive information regarding the origin and size of the aneurysm, the adequacy of surgical repair of SVA, relief of RVOT obstruction, successful closure of VSD, and correct surgical placement of prosthetic aortic valve.
Discussion: Sinus of Valsalva aneurysm may be congenital or acquired.1 In our case, the patient had no history of risk factors for acquired aneurysm such as Marfan syndrome, atherosclerosis, syphilis, infective endocarditis, cystic medial sclerosis, chest trauma, and hypereosinophilic syndrome.1 She was asymptomatic during her childhood, and she experienced progressive exertional dyspnea. Therefore, we regarded the etiology of her condition to be congenital. The slowly progressive nature of aneurysm eventually led to significant RVOT obstruction. There have been a few reported cases in which patients with an unruptured SVA have presented with exertional dyspnea, palpitations, and angina-like chest pain.1,2 Unruptured aneurysms of the RCC have been found to cause pulmonary insufficiency, RVOT obstruction, myocardial ischemia, conduction disturbances, and TR.2–4 A coexisting VSD has also been reported.5 The association between the right and noncoronary SVA and VSD has been proposed to result from incomplete fusion between the right and left distal bulbous septum.6 Cardiac catheterization and angiography are generally performed to analyze the aneurysm and the presence of associated anomalies, particularly VSD, AR, and pulmonary stenosis. The true size of the VSD cannot be estimated angiographically when RCC is prolapsed into the VSD. In our case, there was difficulty in determining the presence of VSD with cardiac catheterization. Two-dimensional and Doppler echocardiography can adequately show unruptured SVA. However, an associated VSD may occasionally escape
Conclusion: The combined presence of SVA and VSD, resulting in RVOT obstruction, AR and TR is a very rare combination. Intraoperative TEE is helpful to confirm the diagnosis, detect the associated anomalies, plan the surgical approach, and assess the adequacy of surgical correction.
1324
References 1. Feldman DN, Roman MJ: Aneurysms of the sinuses of Valsalva. Cardiology 2006;106:73–81. 2. 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. 3. Gavali SA, Phadke MS, Kerkar PG, et al: Unruptured aneurysm of the sinus of Valsalva presenting with right ventricular outflow tract obstruction, complete heart block, and protrusion into left ventricular outflow tract. J Am Coll Cardiol 2013;61:e169. 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. 5. Liang CD, Chang JP, Kao CL: Unruptured sinus of Valsalva aneurysm with right ventricular out flow tract obstruction associated with ventricular septal defect. Cathet Cardiovasc Diagn 1996;37:158–161. 6. Kirklin JW, Barratt-Boyes BG. Congenital aneurysm of the sinus of Valsalva. In: Kirklin JW, Barratt-Boyes BG (eds) Cardiac Surgery. Morphology, Diagnostic Criteria, Natural History, Techniques, Results, and Indications, 2nd edn. New York: Churchill Livingstone, 1993, p. 825–840. 7. Wang K-Y, St. John Sutton M, Ho H-Y, et al: Congenital sinus of Valsalva aneurysm: a multiplane transesophageal echocardiographic experience. J Am Soc Echocardiogr 1997;10:956–63. 8. Abdelkhirane C, Roudaut R, Dallocchio M. Diagnosis of ruptured sinus of Valsalva aneurysms: potential value of transesophageal echocardiography. Echocardiography 1990;7:555–560.
