Unruptured Sinus of Valsalva Aneurysm, With Dissection into the

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1 Case Report Unruptured Sinus of Valsalva Aneurysm, With Dissection into the Interventricular Septum Udora NC, Ejim EC *, Okoye JU Department of Medicine, University of Nigeria, Teaching Hospital, Ituku- ozalla, Enugu state, Nigeria Date Accepted: Date Received: Abstract Sinuses of Valsalva or aortic sinuses are 3 focal expansions at the root of the aorta and are named right, left and non-coronary sinuses. These sinuses serve as support system for the aortic valve and prevent occlusion of the coronary ostia as the aortic cusps open in systole. Sinus of Valsalva aneurysm (SOVA) is congenital in most cases, due to deficiency of the elastic lamina between the aortic media and the annulus fibrosus. The anatomical location of these sinuses can predict some clinical consequences, when complications occur. We report the case of a 29yr old Nigerian man who presented at the accident and emergency unit of the University of Nigeria Teaching Hospital (UNTH), Ituku-Ozalla, Enugu, with clinical features of heart failure. Electrocardiography showed complete heart block, and later supraventricular tachycardia, while echocardiography showed a right sinus of valsalva aneurysm dissecting into the interventricular septum, without evidence of rupture. Keywords: Dissection, Interventricular Septum, Unruptured Sinus, of Valsalva Aneurysm. Introduction Sinuses of Valsalva or aortic sinuses are 3 focal expansions at the root of the aorta [1] and are named right, left and non-coronary sinuses. These sinuses serve as support system for the aortic valve and prevent occlusion of the coronary ostia as the aortic cusps open in systole. [1,2] The sizes of these sinuses vary by gender with the upper limit for males being 4.0cm 2 and 3.6cm 2 for females. Sinus of Valsalva aneurysm (SOVA) is congenital in most cases, due to deficiency of the elastic lamina between the aortic media and the annulus fibrosus [2]. Acquired causes include atherosclerosis, bacterial (tuberculosis, Syphilis), connective tissue diseases (Marfan syndrome, Ehlers Danlos syndrome), infective endocarditis, blunt or penetrating chest injury, vasculitis (Takayasu s arteritis), and iatrogenic injury during aortic valve replacement. [1-4] Congenital SOVA is associated with supracristal/perimembranous ventricular septal defects, bicuspid aotic valve and aortic regurgitation and rarely with coarctation of the aorta, pulmonary stenosis and atrial septal defects. [4] The anatomical location of Access this article online Quick Response Code: Website: DOI: sinuses can predict some clinical consequences, when complications occur. We report the case of a 29yr old man who presented at the accident and emergency unit of the University of Nigeria Teaching Hospital (UNTH), Ituku-Ozalla, Enugu, with features of heart failure, conduction defects and a right sinus of valsalva aneurysm dissecting into the interventricular septum. Case Report Mr. AE - a 29 year-old Nigerian man presented at the Emergency unit of the UNTH with a history of progressive exertional dyspnoea for 4 months, bilateral leg swelling for 3 weeks, and syncopal attacks for a day. There was associated orthopnoea, paroxysmal nocturnal dyspnoea, palpitation, marked effort intolerance and chest pain. Address for correspondence: Dr. Ejim Emmanuel Department of Medicine University of Nigeria Ituku-Ozalla campus emmanuel.ejim@unn.edu.ng This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms. How to cite this article: Udora NC, Ejim EC, Okoye JU. Unruptured Sinus of Valsalva Aneurysm, With Dissection into the terventricular Septum. Int J Med Health Dev 2017; 22(2): International Journal of Medicine and Health Development 150

2 Chest pain was dull and described as heaviness on the left chest and had been recurrent for about 2 years. There was no relieving factor for the pain. His childhood history was uneventful. He had neither a personal nor family history of hypertension, diabetes mellitus or heart disease. He drank about 2 units of alcohol per day and had used marijuana for 3 years. Examination revealed an acutely ill-looking young man in respiratory distress, grade 3 digital clubbing and bilateral pitting leg oedema. The pulse was 160beats/minute, regular, but of small volume; blood pressure was 120/80mmHg, with elevated jugular venous pulsation, and a diplaced apex beat at the 6 th left intercostal space. He had a summation gallop rhythm and a loud P2. His respiratory rate was 38cycles/minute, and he had bibasal fine inspiratory crepitations, and a tender hepatomegaly, with a hepatic span of 14cm. Other systems had unremarkable findings. Chest radiograph showed an enlarged aorta, increased cardiothoracic ratio with left ventricular predominance, and upper lobe diversion. Impression was congestive cardiac failure. Echocardiography showed dilated chambers, with spontaneous echo contrasts in the left ventricular cavity. The aortic valve was trileaflet with a non-mobile right coronary cusp, aneurysmal bulge of the right aortic sinus into the right ventricle and interventricular septum without obvious rupture (Figure 1). Other findings were grade 2 aortic regurgitation (Vmax-168cm/, PG-17mmHg), functional pulmonary regurgitation, functional mitral regurgitation, and functional tricuspid regurgitation (Vmax-251cm/s, PG-25mmHg). Additionally, thick right ventricular anterior wall, thick interventricular septum with cystic masses were noted (Figure 2). Left ventricular mass index was 194g/m 2, with a relative wall thickness is The systolic functions of the left and right ventricles were normal, but the tricuspid annular velocity ratio was reversed. The parietal pericardium was thick (5.8mm), with mild pericardial effusion. The aortic arch and descending aorta were of normal sizes, based on normal z scores for age and gender. The conclusion was a right sinus of Valsalva aneurysm, and eccentric left ventricular hypertrophy. Serial electrocardiograms showed varying findings - complete heart block, left atrial abnormality and inferior myocardial strain, and later sinus tachycardia, left atrial abnormality and bifascicular block (Figure 3). The results of full blood count, urinalysis, fasting lipid profile, serum electrolytes, urea and creatinine, and liver function tests were normal. VDRL and HIV screening were negative. Discussion Sinus of valsalva aneurysms are extremely rare [2, 5, 6] and dissection into the interventricular septum rarer [7, 8]. Most cases are diagnosed after rupture because of the attendant wide pulse pressure. The finding of an unruptured aneurysm with significant cardiac symptoms and signs, as in the index case is rather a rare finding, especially in our environment. There is a male preponderance in affectation, with a male to female ratio of 4:1 [2,5]. The index case is a male with an uruptured aneurysm dissecting into the interventricular septum. Aneurysms of the right and non-coronary cusps occur more frequently 4, and involve the right sinus, as in our patient. SOVA can be an incidental finding on imaging with no symptoms, though, some may cause dyspnoea, chest pain and/or palpitation, as in our patient. All of these can be due to pressure effect on the right or left ventricular outflow tract, coronary or conducting tissues. The paroxysmal arrhythmia experienced by the index patient is most likely due to the effect of direct pressure of the aneurysm on the conducting tissues in the interventricular septum, and the arrhythmias may explain the syncopal attacks. Pressure on the outflow tracts may explain the recurrent leg swelling (right ventricular outflow tract), and syncopal attacks (left ventricular outflow tract).any of the aortic sinusal aneurysms can rupture or expand into the interventricular septum, but often times, it is the right and it causes atrioventricular nodal or His bundle dysfunction which is secondary to direct pressure effect or inflammatory reaction. [2] Aortic regurgitation complicates ruptured or unruptured SOVAs in % of cases [2], and this was evident in our patient. The diagnosis would have been impossible without echocardiography, and this further underscores the need for routine echocardiographic assessment of all patients with suspected cardiac disease. Int J Med Health Dev Volume 22 Issue 2 December

3 Figure 1: Parasternal long axis and short axis views showing sinus of Valsalva aneurysm (SOVA) Int J Med Health Dev Volume 22 Issue 2 December

4 Figure 2: Apical 4 Chamber views showing cystic masses in the interventricular septum (aneurysm). Int J Med Health Dev Volume 22 Issue 2 December

5 Figure 3: 12-lead ECG showing complete heart block, and a rhythm strip showing sinus tachycardia. Int J Med Health Dev Volume 22 Issue 2 December

6 References 1. Weinreich M, Yu P, Trost B. Sinus of Valsalva aneurysm: Review of the literature and an update on management. Clinical Cardiology 2015; 38: Hoey ET, Kanagasingam A, Sivanantham MU. Sinus of valsalva aneurysms: assessment with cardiovascular MRI. American Journal of Roentgenology 2010; 194: White C, Plotnick G. Sinus of valsalva aneurysm. Radiology 2001; 219: Antonio Lijoi, Enrico Parodi, Mario Vito lanneti. Unruptured aneurysm of the left sinus of Valsalva, causing coronary insufficiency. Texas Heart Institute Journal 2002; 29: Goldberg N, Kransnow N. Reviews - Sinus of Valsalva aneurysms. Clinical Cardiology 1990; 13: Feldman DN, Gade CL, Roman MJ.Ruptured aneurysm of the right sinus of Valsalva. Texas Heart Journal 2005; 32: Asad C. Congenital aneurysm of the sinus of Valsalva dissecting into the interventricular septum. Cardiovascular surgery 1995; 3: Taher T,Singal R, Sonnenberg B, Ross D, Graham M. Sinus of Valsalva aneurysm with dissection into the interventricular septum diagnosis by echocardiography and magnetic resonance imaging. Circulation 2005; 111: Int J Med Health Dev Volume 22 Issue 2 December