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A 53 year-old white male with a past medical history of hypertension, hyperlipidemia, and former tobacco use was referred to the Dayton VAMC in Ohio for symptoms that included shortness of breath and a recent abnormal stress test. The patient reported no history of known coronary artery disease (CAD), congestive heart failure, or other cardiovascular diseases. The patient also reported no recent fever, bacterial blood infection, syphilis infection, recreational drug use, or chest trauma.
A physical examination was remarkable for grade 3/6 continuous murmur at the 5th interspace to the left of the sternum and a loud “pistol shot” sound heard over the femoral artery. The patient had jugular venous distension and 2+ leg edema bilaterally. His vital signs were normal, and laboratory blood tests showed normal hemoglobin level and kidney function.
An electrocardiogram showed nonspecific ST segment changes and a transthoracic echocardiogram (TTE) revealed a high-velocity jet in the right atrium (RA) above the tricuspid valve concerning for sinus of Valsalva aneurysm (SVA). 
Right heart catheterization revealed elevated RA pressures with positive shunt study showing oxygen saturation step-up in the RA (Figure 3). Left heart hemodynamic measurement from an aortic approach to the distal part of the noncoronary cusp SVA revealed an RA pressure-tracing pattern consistent with rupture of the noncoronary SVA into the RA (Figure 4). 
The primary diagnosis was of acute heart failure secondary to ruptured aneurysm of the noncoronary SVA into RA. The patient also received a secondary diagnosis of atrial septal aneurysm and PFO.
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Treatment & Outcome
The patient was treated with aggressive diuresis and responded well to therapy. Considering the high mortality rate associated with a ruptured SVA, the patient was referred to a tertiary care center for surgical evaluation. He underwent repair of aorto-right atrial communication with a Cormatrix patch (Roswell, GA) from the aortic side and with primary closure from the right atrial side with resection of the windsock tract; coronary artery bypass graft x1 with right internal mammary artery to the right coronary artery; closure of the PFO with the Cormatrix patch.
The postoperative TEE confirmed preserved LV and RV function, no shunts, no aortic or tricuspid insufficiency. Biopsy of the tissue resected showed intimal fibroplasia. A TTE completed 1 year after surgery showed normal valvular function and without any structural abnormalities. The patient had improvement in symptoms and an uneventful year after surgical intervention followed by 24 session of cardiac rehabilitation.
Discussion
Sinus of Valsalva aneurysm is a dilation of the aortic wall between the aortic valve and the sinotubular junction that is caused by the lack of continuity between the middle layer of the aortic wall and the aortic valve.1 Cases of SVA are rare cardiac anomalies with prevalence of 1% in patients undergoing open-heart surgery.2 Between 65% and 85% of SVA cases originate from the right coronary sinus, 10% to 20% from the noncoronary sinus, and < 5% from the left coronary sinus.3
[embed:render:related:node:106996]
Sinus of Valsalva aneurysm is usually congenital, although cases associated with syphilis, bacterial endocarditis, trauma, Behçet disease, and aortic dissection have been reported. Structural defects associated with congenital SVAs include ventricular septal defect, bicuspid aortic valve, and aortic regurgitation. It is less commonly associated with pulmonary stenosis, coarctation of the aorta, patent ductus arteriosus, tricuspid regurgitation, and atrial septal defects.
The most common complication of the SVA is rupture into another cardiac chamber, frequently the right ventricle (60%) or RA (29%) and less frequently into left atrium (6%), left ventricle (4%), or pericardium (1%).1 Patients with ruptured SVA mainly develop dyspnea and chest pain, but cough, fatigue, peripheral edema, and continuous murmur have been reported.1
Atrial septal aneurysm is an uncommon finding in adults, with an incidence of 2.2 % in the general population, and it is often associated with atrial septal defect and PFO.1,4 Although ASA formation can be secondary to interatrial differences in pressures, it can be a primary malformation involving the region of the fossa ovalis or the entire atrial septum.4 Atrial septal aneurysm may be an isolated anomaly, but often is found in association with other structural cardiac anomalies, including SVA and PFO.4,5
Conclusion
Although coexistence of SVA and ASA has been reported previously, the case reported here, a ruptured noncoronary SVA that was associated with a large ASA and a PFO, has not been previously documented in the English literature. This patient’s anomalies are most likely congenital in origin. Progressive dyspnea and chest pain in the presence of a continuous loud murmur should raise the suspicion of ruptured sinus of Valsalva. Although no significant aortic regurgitation was noted on echocardiography, the pistol shot sound heard over the femoral artery was believed to be due to the rapid diastolic runoff into the RA through the ruptured SVA.
The significant increase in the RA pressure made the ASA and PFO more prominent. A TEE, left and right heart catheterizations with shunt study are vital for the diagnosis of SVA. If left untreated, SVA has an ominous prognosis. Surgical repair of ruptured SVA has an accepted risk and good prognosis with 10-year survival rate of 90%, whereas the mean survival of untreated ruptured SVA is about 4 years.6,7 Hence, the patient in this study was referred to a tertiary care center for surgical intervention.
[embed:render:related:node:134882]
1. Galicia-Tornell MM, Marín-Solís B, Mercado-Astorga O, Espinoza-Anguiano S, Martínez-Martínez M, Villalpando-Mendoza E. Sinus of Valsalva aneurysm with rupture. Case report and literature review. Cir Cir. 2009;77(6):441-445.
2. Takach TJ, Reul GJ, Duncan JM, et al. Sinus of Valsalva aneurysm or fistula: management and outcome. Ann Thorac Surg. 1999;68(5):1573-1577.
3. Meier JH, Seward JB, Miller FA Jr, Oh JK, Enriquez-Sarano M. Aneurysms in the left ventricular outflow tract: clinical presentation, causes, and echocardiographic features. J Am Soc Echocardiogr. 1998;11(7):729-745.
4. Mügge A, Daniel WG, Angermann C et al. Atrial septal aneurysm in adult patients: a multicenter study using transthoracic and transesophageal echocardiography. Circulation. 1995;91(11):2785-2792.
5. Silver MD, Dorsey JS. Aneurysms of the septum primum in adults. Arch Pathol Lab Med. 1978;102(2):62-65.
6. Wang ZJ, Zou CW, Li DC, et al. Surgical repair of sinus of Valsalva aneurysm in Asian patients. Ann Thorac Surg. 2007;84(1):156-160.
7. Yan F, Huo Q, Qiao J, Murat V, Ma SF. Surgery for sinus of valsalva aneurysm: 27-year experience with 100 patients. Asian Cardiovasc Thorac Ann. 2008;16(5):361-365.
A 53 year-old white male with a past medical history of hypertension, hyperlipidemia, and former tobacco use was referred to the Dayton VAMC in Ohio for symptoms that included shortness of breath and a recent abnormal stress test. The patient reported no history of known coronary artery disease (CAD), congestive heart failure, or other cardiovascular diseases. The patient also reported no recent fever, bacterial blood infection, syphilis infection, recreational drug use, or chest trauma.
A physical examination was remarkable for grade 3/6 continuous murmur at the 5th interspace to the left of the sternum and a loud “pistol shot” sound heard over the femoral artery. The patient had jugular venous distension and 2+ leg edema bilaterally. His vital signs were normal, and laboratory blood tests showed normal hemoglobin level and kidney function.
An electrocardiogram showed nonspecific ST segment changes and a transthoracic echocardiogram (TTE) revealed a high-velocity jet in the right atrium (RA) above the tricuspid valve concerning for sinus of Valsalva aneurysm (SVA).
Right heart catheterization revealed elevated RA pressures with positive shunt study showing oxygen saturation step-up in the RA (Figure 3). Left heart hemodynamic measurement from an aortic approach to the distal part of the noncoronary cusp SVA revealed an RA pressure-tracing pattern consistent with rupture of the noncoronary SVA into the RA (Figure 4).
The primary diagnosis was of acute heart failure secondary to ruptured aneurysm of the noncoronary SVA into RA. The patient also received a secondary diagnosis of atrial septal aneurysm and PFO.
[embed:render:related:node:128884]
Treatment & Outcome
The patient was treated with aggressive diuresis and responded well to therapy. Considering the high mortality rate associated with a ruptured SVA, the patient was referred to a tertiary care center for surgical evaluation. He underwent repair of aorto-right atrial communication with a Cormatrix patch (Roswell, GA) from the aortic side and with primary closure from the right atrial side with resection of the windsock tract; coronary artery bypass graft x1 with right internal mammary artery to the right coronary artery; closure of the PFO with the Cormatrix patch.
The postoperative TEE confirmed preserved LV and RV function, no shunts, no aortic or tricuspid insufficiency. Biopsy of the tissue resected showed intimal fibroplasia. A TTE completed 1 year after surgery showed normal valvular function and without any structural abnormalities. The patient had improvement in symptoms and an uneventful year after surgical intervention followed by 24 session of cardiac rehabilitation.
Discussion
Sinus of Valsalva aneurysm is a dilation of the aortic wall between the aortic valve and the sinotubular junction that is caused by the lack of continuity between the middle layer of the aortic wall and the aortic valve.1 Cases of SVA are rare cardiac anomalies with prevalence of 1% in patients undergoing open-heart surgery.2 Between 65% and 85% of SVA cases originate from the right coronary sinus, 10% to 20% from the noncoronary sinus, and < 5% from the left coronary sinus.3
[embed:render:related:node:106996]
Sinus of Valsalva aneurysm is usually congenital, although cases associated with syphilis, bacterial endocarditis, trauma, Behçet disease, and aortic dissection have been reported. Structural defects associated with congenital SVAs include ventricular septal defect, bicuspid aortic valve, and aortic regurgitation. It is less commonly associated with pulmonary stenosis, coarctation of the aorta, patent ductus arteriosus, tricuspid regurgitation, and atrial septal defects.
The most common complication of the SVA is rupture into another cardiac chamber, frequently the right ventricle (60%) or RA (29%) and less frequently into left atrium (6%), left ventricle (4%), or pericardium (1%).1 Patients with ruptured SVA mainly develop dyspnea and chest pain, but cough, fatigue, peripheral edema, and continuous murmur have been reported.1
Atrial septal aneurysm is an uncommon finding in adults, with an incidence of 2.2 % in the general population, and it is often associated with atrial septal defect and PFO.1,4 Although ASA formation can be secondary to interatrial differences in pressures, it can be a primary malformation involving the region of the fossa ovalis or the entire atrial septum.4 Atrial septal aneurysm may be an isolated anomaly, but often is found in association with other structural cardiac anomalies, including SVA and PFO.4,5
Conclusion
Although coexistence of SVA and ASA has been reported previously, the case reported here, a ruptured noncoronary SVA that was associated with a large ASA and a PFO, has not been previously documented in the English literature. This patient’s anomalies are most likely congenital in origin. Progressive dyspnea and chest pain in the presence of a continuous loud murmur should raise the suspicion of ruptured sinus of Valsalva. Although no significant aortic regurgitation was noted on echocardiography, the pistol shot sound heard over the femoral artery was believed to be due to the rapid diastolic runoff into the RA through the ruptured SVA.
The significant increase in the RA pressure made the ASA and PFO more prominent. A TEE, left and right heart catheterizations with shunt study are vital for the diagnosis of SVA. If left untreated, SVA has an ominous prognosis. Surgical repair of ruptured SVA has an accepted risk and good prognosis with 10-year survival rate of 90%, whereas the mean survival of untreated ruptured SVA is about 4 years.6,7 Hence, the patient in this study was referred to a tertiary care center for surgical intervention.
[embed:render:related:node:134882]
A 53 year-old white male with a past medical history of hypertension, hyperlipidemia, and former tobacco use was referred to the Dayton VAMC in Ohio for symptoms that included shortness of breath and a recent abnormal stress test. The patient reported no history of known coronary artery disease (CAD), congestive heart failure, or other cardiovascular diseases. The patient also reported no recent fever, bacterial blood infection, syphilis infection, recreational drug use, or chest trauma.
A physical examination was remarkable for grade 3/6 continuous murmur at the 5th interspace to the left of the sternum and a loud “pistol shot” sound heard over the femoral artery. The patient had jugular venous distension and 2+ leg edema bilaterally. His vital signs were normal, and laboratory blood tests showed normal hemoglobin level and kidney function.
An electrocardiogram showed nonspecific ST segment changes and a transthoracic echocardiogram (TTE) revealed a high-velocity jet in the right atrium (RA) above the tricuspid valve concerning for sinus of Valsalva aneurysm (SVA).
Right heart catheterization revealed elevated RA pressures with positive shunt study showing oxygen saturation step-up in the RA (Figure 3). Left heart hemodynamic measurement from an aortic approach to the distal part of the noncoronary cusp SVA revealed an RA pressure-tracing pattern consistent with rupture of the noncoronary SVA into the RA (Figure 4).
The primary diagnosis was of acute heart failure secondary to ruptured aneurysm of the noncoronary SVA into RA. The patient also received a secondary diagnosis of atrial septal aneurysm and PFO.
[embed:render:related:node:128884]
Treatment & Outcome
The patient was treated with aggressive diuresis and responded well to therapy. Considering the high mortality rate associated with a ruptured SVA, the patient was referred to a tertiary care center for surgical evaluation. He underwent repair of aorto-right atrial communication with a Cormatrix patch (Roswell, GA) from the aortic side and with primary closure from the right atrial side with resection of the windsock tract; coronary artery bypass graft x1 with right internal mammary artery to the right coronary artery; closure of the PFO with the Cormatrix patch.
The postoperative TEE confirmed preserved LV and RV function, no shunts, no aortic or tricuspid insufficiency. Biopsy of the tissue resected showed intimal fibroplasia. A TTE completed 1 year after surgery showed normal valvular function and without any structural abnormalities. The patient had improvement in symptoms and an uneventful year after surgical intervention followed by 24 session of cardiac rehabilitation.
Discussion
Sinus of Valsalva aneurysm is a dilation of the aortic wall between the aortic valve and the sinotubular junction that is caused by the lack of continuity between the middle layer of the aortic wall and the aortic valve.1 Cases of SVA are rare cardiac anomalies with prevalence of 1% in patients undergoing open-heart surgery.2 Between 65% and 85% of SVA cases originate from the right coronary sinus, 10% to 20% from the noncoronary sinus, and < 5% from the left coronary sinus.3
[embed:render:related:node:106996]
Sinus of Valsalva aneurysm is usually congenital, although cases associated with syphilis, bacterial endocarditis, trauma, Behçet disease, and aortic dissection have been reported. Structural defects associated with congenital SVAs include ventricular septal defect, bicuspid aortic valve, and aortic regurgitation. It is less commonly associated with pulmonary stenosis, coarctation of the aorta, patent ductus arteriosus, tricuspid regurgitation, and atrial septal defects.
The most common complication of the SVA is rupture into another cardiac chamber, frequently the right ventricle (60%) or RA (29%) and less frequently into left atrium (6%), left ventricle (4%), or pericardium (1%).1 Patients with ruptured SVA mainly develop dyspnea and chest pain, but cough, fatigue, peripheral edema, and continuous murmur have been reported.1
Atrial septal aneurysm is an uncommon finding in adults, with an incidence of 2.2 % in the general population, and it is often associated with atrial septal defect and PFO.1,4 Although ASA formation can be secondary to interatrial differences in pressures, it can be a primary malformation involving the region of the fossa ovalis or the entire atrial septum.4 Atrial septal aneurysm may be an isolated anomaly, but often is found in association with other structural cardiac anomalies, including SVA and PFO.4,5
Conclusion
Although coexistence of SVA and ASA has been reported previously, the case reported here, a ruptured noncoronary SVA that was associated with a large ASA and a PFO, has not been previously documented in the English literature. This patient’s anomalies are most likely congenital in origin. Progressive dyspnea and chest pain in the presence of a continuous loud murmur should raise the suspicion of ruptured sinus of Valsalva. Although no significant aortic regurgitation was noted on echocardiography, the pistol shot sound heard over the femoral artery was believed to be due to the rapid diastolic runoff into the RA through the ruptured SVA.
The significant increase in the RA pressure made the ASA and PFO more prominent. A TEE, left and right heart catheterizations with shunt study are vital for the diagnosis of SVA. If left untreated, SVA has an ominous prognosis. Surgical repair of ruptured SVA has an accepted risk and good prognosis with 10-year survival rate of 90%, whereas the mean survival of untreated ruptured SVA is about 4 years.6,7 Hence, the patient in this study was referred to a tertiary care center for surgical intervention.
[embed:render:related:node:134882]
1. Galicia-Tornell MM, Marín-Solís B, Mercado-Astorga O, Espinoza-Anguiano S, Martínez-Martínez M, Villalpando-Mendoza E. Sinus of Valsalva aneurysm with rupture. Case report and literature review. Cir Cir. 2009;77(6):441-445.
2. Takach TJ, Reul GJ, Duncan JM, et al. Sinus of Valsalva aneurysm or fistula: management and outcome. Ann Thorac Surg. 1999;68(5):1573-1577.
3. Meier JH, Seward JB, Miller FA Jr, Oh JK, Enriquez-Sarano M. Aneurysms in the left ventricular outflow tract: clinical presentation, causes, and echocardiographic features. J Am Soc Echocardiogr. 1998;11(7):729-745.
4. Mügge A, Daniel WG, Angermann C et al. Atrial septal aneurysm in adult patients: a multicenter study using transthoracic and transesophageal echocardiography. Circulation. 1995;91(11):2785-2792.
5. Silver MD, Dorsey JS. Aneurysms of the septum primum in adults. Arch Pathol Lab Med. 1978;102(2):62-65.
6. Wang ZJ, Zou CW, Li DC, et al. Surgical repair of sinus of Valsalva aneurysm in Asian patients. Ann Thorac Surg. 2007;84(1):156-160.
7. Yan F, Huo Q, Qiao J, Murat V, Ma SF. Surgery for sinus of valsalva aneurysm: 27-year experience with 100 patients. Asian Cardiovasc Thorac Ann. 2008;16(5):361-365.
1. Galicia-Tornell MM, Marín-Solís B, Mercado-Astorga O, Espinoza-Anguiano S, Martínez-Martínez M, Villalpando-Mendoza E. Sinus of Valsalva aneurysm with rupture. Case report and literature review. Cir Cir. 2009;77(6):441-445.
2. Takach TJ, Reul GJ, Duncan JM, et al. Sinus of Valsalva aneurysm or fistula: management and outcome. Ann Thorac Surg. 1999;68(5):1573-1577.
3. Meier JH, Seward JB, Miller FA Jr, Oh JK, Enriquez-Sarano M. Aneurysms in the left ventricular outflow tract: clinical presentation, causes, and echocardiographic features. J Am Soc Echocardiogr. 1998;11(7):729-745.
4. Mügge A, Daniel WG, Angermann C et al. Atrial septal aneurysm in adult patients: a multicenter study using transthoracic and transesophageal echocardiography. Circulation. 1995;91(11):2785-2792.
5. Silver MD, Dorsey JS. Aneurysms of the septum primum in adults. Arch Pathol Lab Med. 1978;102(2):62-65.
6. Wang ZJ, Zou CW, Li DC, et al. Surgical repair of sinus of Valsalva aneurysm in Asian patients. Ann Thorac Surg. 2007;84(1):156-160.
7. Yan F, Huo Q, Qiao J, Murat V, Ma SF. Surgery for sinus of valsalva aneurysm: 27-year experience with 100 patients. Asian Cardiovasc Thorac Ann. 2008;16(5):361-365.