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Mr Meraj-ud Din Shah
Atrial septal aneurysm is a rare cardiac abnormality that is usually detected during routine echocardiography or evaluation of ischemic stroke. It may be isolated or in the presence of other defects, most often patent foramen ovale. Atrial arrhythmias and arterial embolisms are associated complications that must be treated with anticoagulants and rate control.
A 53-year-old female presents with transient left side weakness which slowly improves over three days thereafter. Patient has a few years’ history of on-and-off palpitations. Clinical examination reveals an irregular pulse at 102 beats per minute (bpm), pulse deficit of 6 bpm, and 130/80mmHg blood pressure. Peripheral pulses are normal. Cardiovascular system examination confirms irregular heart. Although heart sounds are normal, a systolic click and a grade iii/iv systolic murmur in the pulmonary area is heard. Central nervous system examination reveals mild weakness in left lower limb. Patient is nevertheless able to walk unsupported. Hemogram, serum lipids, kidney function tests and liver function test results are within normal range. The echocardiogram reveals atrial fibrillation with a ventricular rate of 90/min, normal QRS and T waves. Chest X-ray shows normal cardiac size and clear lungs. Computerised tomography of the brain is normal and shows no evidence of hemorrhage. Transthoracic echocardiography reveals normal-size chambers, valves, and a prominent moderator band in right ventricle. Intraventricular septum is intact. Intra-atrial septum shows a large bulge in fossa ovalis area towards right atrium with limited excursions of this part of the atrial septum. No evidence of thrombus in any chamber or atrial appendage nor evidence of shunt (Figures 1, 2, 3). Carotid Doppler and ultrasound studies are normal. Fig. 1 Transthoracic echocardiography of heart a) Apical 4-C view
b) Apical 4-C view, annotated.
c) Subcostal view, annotated. Patient has ischemic cerebrovascular embolic stroke with left side hemiparesis, atrial fibrillation with atrial septal aneurysm fossa ovalis. The ischemic cerebrovascular incident is most likely a cardioembolic stroke. Atrial septal aneurysm is rare (ASA) and is most often an accidental finding. However it could be a contributing factor to cardioembolic stroke even though no thrombus in aneurysm or left atrium can be seen in transthoracic echo. Patient was started on anticoagulants and rate control for atrial fibrillation.
Although exact definitions of ASA vary according to size (2, 3, 7), and stage (mobility) of the aneurysm (17, 18), atrial septal aneurysm is a localised 'saccular' deformity, generally at the level of the fossa ovalis, which protrudes to the right or left atrium, or on both sides. Albeit rare, atrial septal aneurysm is a well recognised cardiac abnormality. Previously diagnosed during autopsy only, it is now frequently picked up on routine echocardiography or during evaluation of ischemic stroke. Studies link it with peripheral embolism and cardioembolic stroke, pulmonary embolism and atrial arrhythmias, even though clinical significance is uncertain. Further, it can be secondary to interatrial pressure difference or may be the result of a primary malformation involving the fossa ovalis region or the entire septum (1). In patients with chronically elevated atrial pressures, as in mitral stenosis, atrial septal aneurysms are also rare: therefore acquired origin seems unlikely. Congenital malformation of the atrial septum probably contributes to development of ASA, as was suggested by Hanley PC and colleagues (2).
Atrial septal aneurysm may be isolated or associated with another anomaly. Commonest association is patent foramen ovale (PFO). Silver and Dorsy found patent foramen ovale in eight out of 16 patients (3). Other associations are atrial septal defect (2), mitral valve prolapsed (4,5) tricuspid valve prolapse, marfans syndrome, sinus of valsalva aneurysm and aortic dissection (1). Shunt across ASA is more frequently detected with transesophageal echocardiography than with transthoracic echocardiography (1). Association with mitral and tricuspid valve prolapse and other abnormalities, such as Marfan's syndrome and sinus of valsalva aneurysm may point to common inherent connective tissue deficiency (6). Familial clustering of ASA has also been reported (13).
Manifestations attributed to ASA are 1) atrial arrhythmias and 2) arterial embolisms. Interatrial septal aneurysm can act as an arrhythmic focus, generating focal atrial tachycardias. Hanley et al. (2) noted atrial arrhythmias in 20 out of 80 patients (25%). Mugge A. et al., in a multicentre study of 195 patients, found atrial tachyarrhythmia in 47 patients (24%), and 28 patients (>14%) had atrial fibrillation (1). Schneider B. et al. reported prevalence of atrial tachyarrhythmia in 26% of cases of ASA (8). Mechanism of increased prevalence of atrial tachyarrhythmia in ASA is not clear, though redundancy of atrial septum could be responsible for pathogenesis of arrhythmia. Arterial embolism is another complication associated with ASA. Presence of ASA tends to aggravate stasis of left arterial (LA) blood flow and predispose to minute LA clots and systemic thrombo embolisms. With cardiac embolisms reported in 20-52% of cases of ASA, various studies have found significant association between ASA and arterial embolism (7,9,10). Most patients (70%) had left to right shunts (7), and a higher prevalence of ASA was reported in cerebrovascular accident patients compared with the general population (7.9% vs. 2.2%) (11). A retrospective study by Mugge A. et al. reported that patients with ASA, and especially those with shunts, showed increased frequency of clinical events in their past, compatible with cardiogenic embolism. Atrial septal aneurysm was often the only source of embolism, as judged by transesophageal echocardiography (1). Salmasi A. M. reported higher prevalence of ASA and patent foramen ovale in the Afro-Caribbean population, compared with Indo-Asians, suggesting ASA as a possible cause for increased incidence of stroke in Afro-Caribbeans (12). Mechanism of cardioembolic stroke could be right to left shunting, as detected in most cases of ASA or in the thrombogenic properties of aneurysm itself. A non- ejection click may occasionally be heard, possibly as the IAS aneurysm bulges and tenses within the LA/RA cavity, thus suggesting ASA as one of the causes of systolic click (20).
Echocardiography is used to diagnose ASA, either during routine echocardiography or in cases of cardioembolic cerebrovascular stroke and peripheral embolism. Compared with transthoracic echo, transesophageal echo is more sensitive in picking up ASA (1, 7). Cardiac computed tomography and magnetic resonance imaging are also useful for diagnosis of ASA (14, 15).
Conclusion: The report looks at the chances of finding atrial septal aneurysm in a case of cerebral embolism. Although atrial fibrillation is a well known cause of stroke, presence of atrial septal aneurysm needs some attention as it could be contributory. There is a possibility that atrial septal aneurysm could be a culprit for strokes.
Main text 1. Mugge A. et al: Atrial septal aneurysm in adult patients: A multicenter study using transthoracic and transesophageal echocardiography. Circulation 91: 2785, 1995. 2. Hanley P. C., Tajik A. J., Hynes J. K., Edwards W. D., Reeder G. S., Hagler D. J., Seward J. B. Diagnosis and classification of atrial septal aneurysm by two-dimensional echocardiography: report of 80 consecutive cases. J Am Coll Cardiol. 1985; 6: 1370-1382 3. Silver M. D., Dorsey J. S. Aneurysms of the septum primum in adults. Arch Pathol Lab Med. 1978; 102: 62-65. 4. Iliceto S., Papa A., Sorino M, Rizzon P. Combined atrial septal aneurysm and mitral valve prolapse: detection by two-dimensional echocardiography. Am J Cardiol. 1984; 54: 1151-1154. 5. Rahko P. S., Xu Q. B. Increased prevalence of atrial septal aneurysm in mitral valve prolapse. Am J Cardiol. 1990; 66: 253-257. 6. Roberts W. C. Aneurysm (redundancy) of the atrial septum (fossa ovale membrane) and prolapse (redundancy) of the mitral valve. Am J Cardiol. 1984; 54: 1153-1154. 7. Pearson A. C., Nagelhout D., Castello R., Gomez C. R., Labovitz A. J. Atrial septal aneurysm and stroke: a transesophageal echocardiographic study. J Am Coll Cardiol. 1991; 18: 1223-1229. 8. Schneider B., Hofmann T., Meinertz T. Atrial septal aneurysm: is there an association between arrhythmias and stroke? Circulation. 1993; 88(suppl I): I-222. 9. Gallet B., Malergue M. C., Adam C., Saudemont J. P., Collot A. M. C., Druon M. C., Hiltgen M. Atrial septal aneurysm: a potential cause of systemic embolism. Br Heart J. 1985; 53: 292-297. 10. Belkin R. N., Hurwitz B. J., Kisslo J. Atrial septal aneurysm: association with cerebrovascular and peripheral embolic events. Stroke. 1987; 18: 856-862. 11. Agmon Y., Khandheria B.K., Meissner I., et al. Frequency of atrial septal aneurysm in patients with cerebral ischemic events. Circulation. 1999; 99: 1942–1944. 12. Salmasi A. M. et al. Atrial Septal Aneurysm and Patent Foramen Ovale Are Less Prevalent in the Indo-Asian Than in the Caucasian or Afro-Caribbean Population. Angiology February 2010; 61( 2): 205-210 13. Werren M. Clinical implications of familial occurrence of atrial septal aneurysm - - G Ital Cardiol (Rome) - 01-AUG-2008; 9(8): 579-82 14. Hur J., Kim Y. J., Lee H.-J., Ha J.-W., Heo J. H., Choi E.-Y., Shim C.-Y., Kim T. H., Nam J. E., Choe K. O., et al. Cardiac Computed Tomographic Angiography for Detection of Cardiac Sources of Embolism in Stroke Patients Stroke, June 1, 2009; 40(6): 2073-2078. 15. F. Saremi S., Channual A., Raney S., Gurudevan V., Narula J., Fowler S., Abolhoda A., and Milliken J. C. Imaging of Patent Foramen Ovale with 64-Section Multidetector CT Radiology, November 1, 2008; 249(2): 483 - 492. 16. Wahl A., Krumsdorf U., Meier B., et al. Transcatheter treatment of atrial septal aneurysm associated with patent foramen ovale for prevention of recurrent paradoxical embolism in high-risk patients. J Am Coll Cardiol 2005; 45: 377-380 17. Olivares-Reyes A., Chan S., Lazar E. J., Bandlamudi K., et al. Atrial septal aneurysm: A new classification in two hundred five adults J Am Soc Echocardiogr 1997; 10: 644-56. 18. Longhini C., Brunazzi M. C., Musacci G., et al. Atrial septal aneurysm: Echopolycardiographic study. Am J Cardiol 1985; 56 :653-67 19. Mas J. L., Arquizan C., Lamy C., et al. Recurrent cerebrovascular events associated with patent foramen ovale, atrial septal aneurysm, or both. N Engl J Med 2001; 345: 1740. 20. Alexander M .D, Bloom K. R., Hart P., D'Silva F., and Murgo J. P. Atrial septal aneurysm: a cause for midsystolic click. Report of a case and review of the literature. Circulation 1981; 63: 1186-1188 21. Prospective A., Furlan A. J., et al. Multicenter, Randomized, Controlled Trial to Evaluate the Safety and Efficacy of the STARFlex Septal Closure System Versus Best Medical Therapy in Patients With Stroke or Transient Ischemic Attack Due to Presumed Paradoxical Embolism Through a Patent Foramen Ovale for the CLOSURE I Investigators. Stroke 2010, 41: 2872-2883. Author's response 1.Mugge A. et al: Atrial septal aneurysm in adult patients: A multicenter study using transthoracic and transesophageal echocardiography. Circulation 91: 2785, 1995. 2. Hanley P. C., Tajik A. J., Hynes J. K., Edwards W. D., Reeder G. S., Hagler D. J., Seward J. B. Diagnosis and classification of atrial septal aneurysm by two-dimensional echocardiography: report of 80 consecutive cases. J Am Coll Cardiol. 1985; 6: 1370-1382
Meraj-ud Din Shah. MD., DM. (Cardiology), FICC Srinagar Kashmir Author's disclosures: None declared. Reader's remark: The direction of septal motion denotes which artium has elevated pressure, i.e. mean bulging of IAS to the right denoting high left atrial pressure and vice versa. Would you like to comment? Author's response: I agree with my friend Dr. Elsheikh. Indeed, direction of IAS bulge depends on pressure differences between atria. We see IAS bulges to the right in severe mitral stenosis and similarly, IAS bulges to the left in severe pulmonary hypertension with tricuspid regurgitation. But it should be clear that the bulge of IAS as such is not ASA. There should be at least 15mm (some consider 10mm) protrusion of IAS or part of it from atrial septal plane to diagnose ASA. We do see oscillation of ASA with respiratory and cardiac cycle. A hypothesis based on interatrial pressure gradients is proposed to explain the different motions and configurational characteristics of atrial septal aneurysms. In parallel, however, we should be aware that ASA is rare in situations with chronically elevated atrial pressure - as in mitral stenosis. Possible inherent congenital malformation of IAS contributes to development of ASA.
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