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Case Study
Aneurysm of the sinus of Valsalva dissecting into the intraventricular septum
Asian Cardiovascular & Thoracic Annals 0(0) 1–3 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314549585 aan.sagepub.com
Takayoshi Kato, Shinji Tomita, Mototsugu Tamaki and Yasuhide Okawa
Abstract Aneurysm of the sinus of Valsalva is an uncommon entity and especially rare when it dissects into the intraventricular septum. This uncommon clinical manifestation often takes a drastic clinical course with life-threatening arrhythmia, coronary ischemia, rupture, and heart failure. We present the case of a 36-year-old man with an aneurysm of the sinus of Valsalva dissecting into the intraventricular septum, which induced severe aortic insufficiency and heart block.
Keywords Aneurysm, dissecting, aortic aneurysm, heart block, heart septum, sinus of Valsalva
Introduction Aneurysm of the sinus of Valsalva (ASV) is an uncommon entity and especially rare when it dissects into the intraventricular septum (IVS). This uncommon clinical manifestation often takes a drastic clinical course with life-threatening arrhythmia, coronary ischemia, rupture, and heart failure. We describe a case of ASV with dissection into the IVS, which induced severe aortic insufficiency and heart block.
Case report A 36-year-old Japanese man was referred to our hospital with a diagnosis of complete atrioventricular (AV) block. After detailed examinations, a pacemaker was implanted under a diagnosis of idiopathic AV block. Three months after the procedure, he complained of progressive breathlessness and high-grade fever. He denied any history of trauma or chest pain preceding his symptoms. Physical examination did not reveal the stigmata of Behc¸et’s disease, Marfan’s syndrome, or any other collagen vascular disease. There were no signs of infective endocarditis or pacemaker generator infection. Laboratory investigations showed a raised white blood cell count (9210/mL) and C-reactive protein level (1.6 mg dL 1). Blood cultures were negative for pathogens. Echocardiography disclosed severe aortic
insufficiency and a cyst-like blind formation in the IVS, approximately 20 10 mm in size, originating just below the orifice of the right coronary artery (Figure 1A). We reviewed the previous echocardiography carried out before pacemaker implantation, and found a cystic formation in the IVS, measuring 10 10 mm in diameter, without evidence of aortic insufficiency (Figure 1B). Computed tomography confirmed the presence of a large saccular aneurysm in the IVS, changing shape with the cardiac cycle (Figure 2). After an aortotomy, complete detachment of the right coronary annulus from the sinus of Valsalva was seen. The edge of the dehiscence was thickened and the right cusp was extremely shortened. The aneurysm extending into the IVS had a smooth surface and no thrombus within it. No calcification was seen on the aneurysmal sac. There was no evidence of infective endocarditis or perforation of other cardiac chambers, and the other two cusps and sinuses appeared normal. The aneurysmal wall was plicated using a 5/0 polypropylene running suture to obliterate the aneurysmal sac.
Department of Cardiovascular Surgery, Gifu Heart Center, Gifu, Japan Corresponding author: Takayoshi Kato, MD, Department of Cardiovascular Surgery, Gifu Heart Center, 4-14-4 Yabutaminami, Gifu City, Gifu 500-8384, Japan. Email: [email protected]
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Figure 1. (A) Echocardiography revealing a cyst-like blind formation (arrows) in the intraventricular septum, approximately 20 10 mm in size, originating just below the orifice of the right coronary artery. (B) Echocardiography before pacemaker implantation showed a small cystic lesion in the intraventricular septum, measuring 10 10 mm in diameter without any aortic insufficiency. The arrow heads indicate the left coronary cusp. Ao: aorta; LA: left atrium; LV: left ventricle.
Figure 2. Computed tomography confirming the presence of a large saccular aneurysm in the intraventricular septum, changing shape with the cardiac cycle.
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After closure of the dehiscence with bovine pericardium using 5/0 polypropylene sutures, an 18-mm mechanical valve (ATS Medical, Inc., Annapolis, MN, USA) was implanted successfully. The patient was discharged uneventfully, and postoperative echocardiography showed disappearance of the echo-free space within the IVS. The thickness of the IVS returned to normal, but paradoxical wall motion remained.
Discussion ASV is an infrequent cardiac anomaly that usually remains asymptomatic and undetected unless rupture occurs. Since the first report of ASV with IVS dissection in 1947, such an entity has been rarely reported.1,2 Moreover, rupture of an ASV into the IVS is a uncommon manifestation which comprises less than 2% of ASV ruptures.3 Sakakibara and Konno suspected that this was a result of intramural rupture of the ASV and organized hematoma (pseudoaneurysm), and this opinion has been shared by others.2,4 Other hypotheses regarding to the formation of IVS dissection include the Venturi effect of the left ventricular outflow tract and septal compression due to the ASV.2,5 Secondary causes are rare but include bacterial endocarditis, myocardial infarction, trauma, syphilis, tuberculosis, Behc¸et’s disease, and others.6 Common clinical manifestations reported in ASV with IVS dissection are AV conduction disturbances and congestive heart failure due to aortic insufficiency.1 Direct pressure by the aneurysm and inflammatory changes near the AV node are considered responsible for various types of conduction disturbance.7 Rarely, the aneurysm presents with obstruction of the ventricular outflow tract, coronary artery compression, unexplained arrhythmia, and sudden death.1 In the current case, the patient remained asymptomatic for 3 months with a pacemaker until significant aortic insufficiency developed. Walters and colleagues6 reported cases with a similar clinical course of ASV with IVS dissection. Choudhary and colleagues1 experienced 2-, 5-, and 20-year survival of cases of ASV with IVS dissection without surgical treatment. However, these patients had no severe aortic insufficiency at initial presentation, and 2 of 3 patients disappeared from follow-up. We seldom encounter cases of ASV with IVS dissection surviving without repair. We conducted plication of the aneurysmal wall and closure of the dehiscence before aortic valve replacement. Some authors noted that plication of the cavity by direct suturing accompanied by closure of the orifice with a patch is effective and simple, and resection of the sac is unnecessary and
may damage the IVS.8 However, plication is impossible in the presence of calcification on the aneurysmal sac. In that case, patch closure of the aneurysmal sac with an obturator might be a palliative idea.8 Valve replacement might be unnecessary if the leaflets maintain their normal aspects. The clinical time course of ASV with IVS dissection is unknown. In our patient, dynamic progression of the ASV induced a severe conduction disturbance progressing to aortic insufficiency with heart failure. Early detection and prompt surgical therapy may be required before the patient’s condition deteriorates. Acknowledgment We thank Dr. Ryuhei Tanaka for fruitful discussion.
Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
Conflict of interest statement None declared.
References 1. Choudhary SK, Bhan A, Reddy SC, et al. Aneurysm of sinus of Valsalva dissecting into interventricular septum. Ann Thorac Surg 1998; 65: 735–740. 2. Wu Q, Xu J, Shen X, Wang D and Wang S. Surgical treatment of dissecting aneurysm of the interventricular septum. Eur J Cardiothorac Surg 2002; 22: 517–520. 3. Bricker AO, Avutu B, Mohammed TL, et al. Valsalva sinus aneurysms: findings at CT and MR imaging. Radiographics 2010; 30: 99–110. 4. Sakakibara S and Konno S. Congenital aneurysm of the sinus of Valsalva. Anatomy and classification. Am Heart J 1962; 63: 405–424. 5. Kervan U, Bardakci H, Altintas G, Tufekcioglu O and Birincioglu CL. A case of intraventricular septum dissection presenting with aneurysmal dilatation through the outflow track of the left ventricle. J Card Surg 2008; 23: 173–176. 6. Walters MI, Ettles D, Guvendik L and Kaye GC. Interventricular septal expansion of a sinus of Valsalva aneurysm: a rare cause of complete heart block. Heart 1998; 80: 202–203. 7. Kutay V, Ekim H and Yakut C. Surgical repair of postoperative left sinus of Valsalva aneurysm dissecting into the interventricular septum [Review]. Ann Thorac Surg 2005; 79: 341–343. 8. Kalimanovska-Ostric´ D, Ostojic´ M, Petrovic´ P, Krotin M, Ostric´ V and Avramovic´ D. Unruptured congenital aneurysm of the right sinus of Valsalva. Tex Heart Inst J 1996; 23: 217–221.
Downloaded from aan.sagepub.com at UNIVERSITE LAVAL on November 16, 2015
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Case Study
Aneurysm of the sinus of Valsalva dissecting into the intraventricular septum
Asian Cardiovascular & Thoracic Annals 0(0) 1–3 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314549585 aan.sagepub.com
Takayoshi Kato, Shinji Tomita, Mototsugu Tamaki and Yasuhide Okawa
Abstract Aneurysm of the sinus of Valsalva is an uncommon entity and especially rare when it dissects into the intraventricular septum. This uncommon clinical manifestation often takes a drastic clinical course with life-threatening arrhythmia, coronary ischemia, rupture, and heart failure. We present the case of a 36-year-old man with an aneurysm of the sinus of Valsalva dissecting into the intraventricular septum, which induced severe aortic insufficiency and heart block.
Keywords Aneurysm, dissecting, aortic aneurysm, heart block, heart septum, sinus of Valsalva
Introduction Aneurysm of the sinus of Valsalva (ASV) is an uncommon entity and especially rare when it dissects into the intraventricular septum (IVS). This uncommon clinical manifestation often takes a drastic clinical course with life-threatening arrhythmia, coronary ischemia, rupture, and heart failure. We describe a case of ASV with dissection into the IVS, which induced severe aortic insufficiency and heart block.
Case report A 36-year-old Japanese man was referred to our hospital with a diagnosis of complete atrioventricular (AV) block. After detailed examinations, a pacemaker was implanted under a diagnosis of idiopathic AV block. Three months after the procedure, he complained of progressive breathlessness and high-grade fever. He denied any history of trauma or chest pain preceding his symptoms. Physical examination did not reveal the stigmata of Behc¸et’s disease, Marfan’s syndrome, or any other collagen vascular disease. There were no signs of infective endocarditis or pacemaker generator infection. Laboratory investigations showed a raised white blood cell count (9210/mL) and C-reactive protein level (1.6 mg dL 1). Blood cultures were negative for pathogens. Echocardiography disclosed severe aortic
insufficiency and a cyst-like blind formation in the IVS, approximately 20 10 mm in size, originating just below the orifice of the right coronary artery (Figure 1A). We reviewed the previous echocardiography carried out before pacemaker implantation, and found a cystic formation in the IVS, measuring 10 10 mm in diameter, without evidence of aortic insufficiency (Figure 1B). Computed tomography confirmed the presence of a large saccular aneurysm in the IVS, changing shape with the cardiac cycle (Figure 2). After an aortotomy, complete detachment of the right coronary annulus from the sinus of Valsalva was seen. The edge of the dehiscence was thickened and the right cusp was extremely shortened. The aneurysm extending into the IVS had a smooth surface and no thrombus within it. No calcification was seen on the aneurysmal sac. There was no evidence of infective endocarditis or perforation of other cardiac chambers, and the other two cusps and sinuses appeared normal. The aneurysmal wall was plicated using a 5/0 polypropylene running suture to obliterate the aneurysmal sac.
Department of Cardiovascular Surgery, Gifu Heart Center, Gifu, Japan Corresponding author: Takayoshi Kato, MD, Department of Cardiovascular Surgery, Gifu Heart Center, 4-14-4 Yabutaminami, Gifu City, Gifu 500-8384, Japan. Email: [email protected]
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Figure 1. (A) Echocardiography revealing a cyst-like blind formation (arrows) in the intraventricular septum, approximately 20 10 mm in size, originating just below the orifice of the right coronary artery. (B) Echocardiography before pacemaker implantation showed a small cystic lesion in the intraventricular septum, measuring 10 10 mm in diameter without any aortic insufficiency. The arrow heads indicate the left coronary cusp. Ao: aorta; LA: left atrium; LV: left ventricle.
Figure 2. Computed tomography confirming the presence of a large saccular aneurysm in the intraventricular septum, changing shape with the cardiac cycle.
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After closure of the dehiscence with bovine pericardium using 5/0 polypropylene sutures, an 18-mm mechanical valve (ATS Medical, Inc., Annapolis, MN, USA) was implanted successfully. The patient was discharged uneventfully, and postoperative echocardiography showed disappearance of the echo-free space within the IVS. The thickness of the IVS returned to normal, but paradoxical wall motion remained.
Discussion ASV is an infrequent cardiac anomaly that usually remains asymptomatic and undetected unless rupture occurs. Since the first report of ASV with IVS dissection in 1947, such an entity has been rarely reported.1,2 Moreover, rupture of an ASV into the IVS is a uncommon manifestation which comprises less than 2% of ASV ruptures.3 Sakakibara and Konno suspected that this was a result of intramural rupture of the ASV and organized hematoma (pseudoaneurysm), and this opinion has been shared by others.2,4 Other hypotheses regarding to the formation of IVS dissection include the Venturi effect of the left ventricular outflow tract and septal compression due to the ASV.2,5 Secondary causes are rare but include bacterial endocarditis, myocardial infarction, trauma, syphilis, tuberculosis, Behc¸et’s disease, and others.6 Common clinical manifestations reported in ASV with IVS dissection are AV conduction disturbances and congestive heart failure due to aortic insufficiency.1 Direct pressure by the aneurysm and inflammatory changes near the AV node are considered responsible for various types of conduction disturbance.7 Rarely, the aneurysm presents with obstruction of the ventricular outflow tract, coronary artery compression, unexplained arrhythmia, and sudden death.1 In the current case, the patient remained asymptomatic for 3 months with a pacemaker until significant aortic insufficiency developed. Walters and colleagues6 reported cases with a similar clinical course of ASV with IVS dissection. Choudhary and colleagues1 experienced 2-, 5-, and 20-year survival of cases of ASV with IVS dissection without surgical treatment. However, these patients had no severe aortic insufficiency at initial presentation, and 2 of 3 patients disappeared from follow-up. We seldom encounter cases of ASV with IVS dissection surviving without repair. We conducted plication of the aneurysmal wall and closure of the dehiscence before aortic valve replacement. Some authors noted that plication of the cavity by direct suturing accompanied by closure of the orifice with a patch is effective and simple, and resection of the sac is unnecessary and
may damage the IVS.8 However, plication is impossible in the presence of calcification on the aneurysmal sac. In that case, patch closure of the aneurysmal sac with an obturator might be a palliative idea.8 Valve replacement might be unnecessary if the leaflets maintain their normal aspects. The clinical time course of ASV with IVS dissection is unknown. In our patient, dynamic progression of the ASV induced a severe conduction disturbance progressing to aortic insufficiency with heart failure. Early detection and prompt surgical therapy may be required before the patient’s condition deteriorates. Acknowledgment We thank Dr. Ryuhei Tanaka for fruitful discussion.
Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
Conflict of interest statement None declared.
References 1. Choudhary SK, Bhan A, Reddy SC, et al. Aneurysm of sinus of Valsalva dissecting into interventricular septum. Ann Thorac Surg 1998; 65: 735–740. 2. Wu Q, Xu J, Shen X, Wang D and Wang S. Surgical treatment of dissecting aneurysm of the interventricular septum. Eur J Cardiothorac Surg 2002; 22: 517–520. 3. Bricker AO, Avutu B, Mohammed TL, et al. Valsalva sinus aneurysms: findings at CT and MR imaging. Radiographics 2010; 30: 99–110. 4. Sakakibara S and Konno S. Congenital aneurysm of the sinus of Valsalva. Anatomy and classification. Am Heart J 1962; 63: 405–424. 5. Kervan U, Bardakci H, Altintas G, Tufekcioglu O and Birincioglu CL. A case of intraventricular septum dissection presenting with aneurysmal dilatation through the outflow track of the left ventricle. J Card Surg 2008; 23: 173–176. 6. Walters MI, Ettles D, Guvendik L and Kaye GC. Interventricular septal expansion of a sinus of Valsalva aneurysm: a rare cause of complete heart block. Heart 1998; 80: 202–203. 7. Kutay V, Ekim H and Yakut C. Surgical repair of postoperative left sinus of Valsalva aneurysm dissecting into the interventricular septum [Review]. Ann Thorac Surg 2005; 79: 341–343. 8. Kalimanovska-Ostric´ D, Ostojic´ M, Petrovic´ P, Krotin M, Ostric´ V and Avramovic´ D. Unruptured congenital aneurysm of the right sinus of Valsalva. Tex Heart Inst J 1996; 23: 217–221.
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