Giant Right Atrial Mass: An Unusual Presentation Of Unruptured Sinus Of Valsalva Aneurysm

摘要

Sinus of valsalva aneurysm (SOV) is an uncommon but well-recognized cardiac condition requiring emergent surgical management when it ruptures. We report a ninety-three-year-old woman with progressive dyspnea on exertion diagnosed to have a giant right atrial “mass” arising in close proximity to the non coronary cusp on sinus of valsalva on transthoracic echocardiogram. She underwent surgical excision of the aneurysm and confirmed to have unruptured sinus of valsalva aneurysm of non coronary cusp extending into the right atrium. This case also highlights the conduction abnormalities associated with this condition. We review the literature on sinus of valsalva aneurysm and discuss the unusual presentation and association with arrhythmias. Case description A 93-year-old white woman presented with gradual onset of progressively worsening dyspnea on exertion for four weeks. She denied orthopnea, paroxysmal nocturnal dyspnea, cough, leg swelling, and angina on exertion. Her past medical history was significant for essential hypertension, gastroesophageal reflux disease, degenerative arthritis of knees and transient ischemic attacks. Her medications included baby aspirin, metoprolol, and lansoprazole. Physical examination revealed a pulse of 86 beats per minute, blood pressure of 132/86 millimeters of mercury with no significant difference between the extremities, and respirations of 16 per minute. Cardiac auscultation was unremarkable and the lungs were clear to auscultation. Her initial work-up revealed normal blood counts, electrolytes and electrocardiogram showed a first degree heart block. Chest X-ray revealed mild cardiomegaly and atherosclerotic aorta (Figure 1).Transthoracic echocardiogram (TTE) revealed a large, 57mm x 65mm, spherical, heterogeneous mass in the right atrial cavity by the apical four chamber view (Figure 2). Short axis view at the level of aortic valve shows the aneurysm arising in close proximity to non coronary cusp of sinus of valsalva (Figure 3/movie clip). The parasternal long axis view showed an apparent defect in the aortic wall (Figure 4) although there was no demonstrable any doppler flow through the defect (Figure 5). There was no evidence of significant regurgitation or stenosis of any valves. The calculated left ventricle ejection fraction was 60%. Right ventricular systolic function was within normal limits. Selective cardiac catheterization revealed non obstructive coronary artery disease involving the mid left anterior descending artery with no evidence of high grade obstruction of other epicardial coronary arteries. A median sternotomy incision was created and cardiopulmonary bypass was created after successfully cannulating the ascending aorta and the vena cavae. On opening the right atrium open, a lobular necrotic mass measuring approximately six centimeters in size was seen to encroach on the entire right atrium (RA) which was found partially adherent to the right atrial roof, aortic wall and dissecting into the interatrial septum (IAS). The mass had a layer of tissue overlying. The entire mass was resected along with a button of the aortic wall which was quite adherent and thinned out in that region of attachment to the mass. Corrective surgeries to repair the defects in the right atrial roof, IAS and the aortic wall were performed successfully. Post-operatively the clinical course was complicated by severe bradycardia. She underwent a permanent pacemaker insertion for her sick sinus syndrome. However, she then developed atrial flutter and was successfully cardioverted. She had to be started on amiodarone to prevent recurrence.Histopathological exam (HPE) of the mass revealed predominantly blood clot with a thin fibrous wall around the periphery composed of denselycollagenous fibrous tissue showing areas of dystrophic calcification, collections of foamy histiocytes, hemosiderin deposition, and focalareas of mild chronic inflammation suggestive of atherosclerotic changes. These findings wer