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Atrial septal aneurysm with biatrial mass: A rare combination presenting as diagnostic conundrum
i n d i a n h e a r t j o u r n a l 6 6 ( 2 0 1 4 ) 7 1 6 e7 1 8
Available online at www.sciencedirect.com
ScienceDirect journal homepage: www.elsevier.com/locate/ihj
Case Report
Atrial septal aneurysm with biatrial mass: A rare combination presenting as diagnostic conundrum Sudarshan Kumar Vijay a,*, Bhuwan Tiwari b, Mukul Misra b, Lalit Mohan Joshi c, Dharmendra Kumar Srivastava c a
Assistant Professor, Department of Cardiology, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, U.P., 226010, India b Department of Cardiology, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, U.P., 226010, India c Department of Cardiovascular Surgery, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, U.P., 226010, India
article info Article history: Received 18 May 2014 Accepted 9 October 2014 Available online 31 October 2014 Keywords: Atrial septal aneurysm Biatrial mass Trans esophageal echocardiography
1.
Case report
A 50-year-old female presented to us with history of effort dyspnea and palpitations for last 5- years. She was diagnosed as a case of atrial septal aneurysm with inlying thrombus at a peripheral medical center, and treated with oral anticoagulation. Her physical examination revealed a short systolic murmur in the left paratsernal area. The blood tests and electrocardiogram did not reveal any significant abnormality. The chest X ray posteroanterior view showed mild cardiomegaly. Her 2-dimensional transthoracic echocardiogram (Apical four chamber view) revealed an atrial septal aneurysm
(ASA) bulging into right atrium, with an attached irregular mobile mass which spanned into both atrium, giving impression of a large thrombus (Fig. 1). The color doppler examination showed mild tricuspid regurgitation with estimated right ventricular systolic pressure of 40 mm of Hg. Subsequently, she underwent transesophageal echocardiogram (TEE) which showed a large atrial septal aneurysm (16 mm) with a freely mobile biatrial mass, seems to be attached to the center of aneurysm (Fig. 2A). TEE bicaval view revealed the presence of a 12 mm high ostium secondum atrial septal defect (ASD) in the vicinity of ASA, with left to right shunt (Fig. 2B). All four pulmonary veins normally drained into the left atrium. 3-dimensional TEE showed the
* Corresponding author. Department of Cardiology, Dr. Ram Manohar Lohia Institute of Medical sciences, Gomti Nagar, Lucknow, U.P., PO-226003, India. Tel.: þ91 9559660560; fax: þ91 5222258948. E-mail address: [email protected] (S.K. Vijay). http://dx.doi.org/10.1016/j.ihj.2014.10.401 0019-4832/Copyright © 2014, Cardiological Society of India. All rights reserved.
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heterogeneous echo characteristics of the mass, clear attachment of the mass with the septum by a stalk on the left atrial side (Fig. 3A) and bicaval view showed the spatial relationship of the mass with the ASD (Fig. 3B). Under hypothermic cardiopulmonary bypass, resection of the biatrial septal mass with septal aneurysm and pericardial patch repair of atrial septal defect was done. Histopathological evaluation of the resected mass revealed the diagnosis of atrial myxoma. The patient is doing good after 6- months of follow up.
2.
Fig. 1 e 2D transthoracic echocardiogram apical 4chamber view showing interatrial septal aneurysm with large mass attached to it.
Discussion
Atrial septal aneurysm is a rare localized saccular deformity of the atrial septum, generally arising at the level of fossa ovalis, which bulges to either right or left atrium or both sides. The definitions of ASA vary according to the size1,2 and mobility of the aneurysm.3,4 The commonly associated abnormalities with ASA include patent foramen ovale, atrial septal defect, mitral or tricuspid valve prolapse and sinus of valsalva aneurysm. The congenital malformation of the atrial septum has been suggested to play a causative role in the development of ASA.1 The usual clinical manifestations of ASA can occur due to atrial arrhythmias or thromboembolism.5 ASAs
Fig. 2 e 2D TEE Showing A. Large atrial septal neurysm with two different freely mobile masses attached to each side of ASA B. (Bicaval view) showing 12 mm high OS- ASD in the vicinity of ASA with left to right shunt.
Fig. 3 e 3D TEE showing A. Atrial mass attached to interatrial septum by stalk B. presence of ASD with its relation to biatrial mass.
718
i n d i a n h e a r t j o u r n a l 6 6 ( 2 0 1 4 ) 7 1 6 e7 1 8
can itself act as arrhythmic focus generating atrial tachycardias. The stasis of blood in ASA or in atrium can lead to formation of clots with resultant arterial embolism. Most patients with ASA have left to right shunts and incidence of ASAs is higher in patients with cerebrovascular accidents than in general population.6 TEE is more sensitive in detection of ASAs than transthoracic echocardiography. Myxomas are uncommon primary tumors of the heart, and the myxomas which arise from two different heart chambers is even extremely rare. Cardiac myxomas are usually located in the left atrium and less commonly in the right atrium. Multiple intracardiac myxomas account for less than 5% of all cases and biatrial myxomas in fewer than 2.5%.7 Early diagnosis of cardiac myxomas is usually difficult because the manifestations are usually non specific and major symptoms usually occur in large myxomas leading to intracardiac obstruction or embolization. Other rare cardiac tumors such as myxoid fibrosarcoma presenting as biatrial mass has also been reported.8 Large atrial clot is a close differential diagnosis of atrial myxomas, especially when stalk of myxoma could not be visualized on routine echocardiography. 2D & 3D TEE can provide better differentiation of clot from myxomas due to its spatial higher resolution. Cardiac MRI and multislice CT are other diagnostic modalities, which can be useful in differentiating cardiac tumor from thrombus by enhanced tissue characterization. To the best of our knowledge, association of a biatrial cardiac myxoma with atrial septal aneurysm is not previously reported in the literature. In our case, biatrial mass was erroneously diagnosed as a large clot at a peripheral center and the patient was put on oral anticoagulation. On first look, we also thought the possibility of clot, due to its association with ASA. However, 2-D TEE clearly revealed the presence of ASD with clear extension of the mass which could not be clearly shown by transthoracic echocardiography. 3-D TEE provided the en face views of the mass with visualization of its stalk, spatial relationship and heterogeneous surface characteristics, which strengthened the diagnosis of a cardiac tumor.
Conflicts of interest All authors have none to declare.
Appendix A. Supplementary data Supplementary data related to this article can be found at http://dx.doi.org/10.1016/j.ihj.2014.10.401.
references
1. Hanley PC, Tajik AJ, Hynes JK, et al. Diagnosis and classification of atrial septal aneurysm by two-dimensional echocardiography: report of 80 consecutive cases. J Am Coll Cardiol. 1985;6:1370e1382. 2. Pearson AC, Nagelhout D, Castello R, Gomez CR, Labovitz AJ. Atrial septal aneurysm and stroke: a transesophageal echocardiographic study. J Am Coll Cardiol. 1991;18:1223e1229. 3. Olivares-Reyes A, Chan S, Lazar EJ, Bandlamudi K, Narla V, Ong K. Atrial septal aneurysm: a new classification in two hundred five adults. J Am Soc Echocardiogr. 1997;10:644e656. 4. Longhini Carlo, Brunazzi Maria Cristiana, Musacci Gianfranca, et al. Atrial septal aneurysm: echopolycardiographic study. Am J Cardiol. 1985;56:653e667. 5. Gallet B, Malergue MC, Adam C, et al. Atrial septal aneurysm: a potential cause of systemic embolism. Br Heart J. 1985;53:292e297. 6. Agmon Y, Khandheria BK, Meissner I, et al. Frequency of atrial septal aneurysm in patients with cerebral ischemic events. Circulation. 1999;99:1942e1944. 7. Peachell JL, Mullen JC, Bentley MJ, Taylor DA. Biatrial myxoma: a rare cardiac tumor. Ann Thorac Surg. 1998;65:1768e1769. 8. Gupta P, Kapoor A, Jain M, Kumar S, Agarwal SK, Pande S. A butterfly shaped mobile biatrial cardiac mass: myxoma or something else. Indian Heart J. 2014;66:372e374.
Available online at www.sciencedirect.com
ScienceDirect journal homepage: www.elsevier.com/locate/ihj
Case Report
Atrial septal aneurysm with biatrial mass: A rare combination presenting as diagnostic conundrum Sudarshan Kumar Vijay a,*, Bhuwan Tiwari b, Mukul Misra b, Lalit Mohan Joshi c, Dharmendra Kumar Srivastava c a
Assistant Professor, Department of Cardiology, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, U.P., 226010, India b Department of Cardiology, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, U.P., 226010, India c Department of Cardiovascular Surgery, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, U.P., 226010, India
article info Article history: Received 18 May 2014 Accepted 9 October 2014 Available online 31 October 2014 Keywords: Atrial septal aneurysm Biatrial mass Trans esophageal echocardiography
1.
Case report
A 50-year-old female presented to us with history of effort dyspnea and palpitations for last 5- years. She was diagnosed as a case of atrial septal aneurysm with inlying thrombus at a peripheral medical center, and treated with oral anticoagulation. Her physical examination revealed a short systolic murmur in the left paratsernal area. The blood tests and electrocardiogram did not reveal any significant abnormality. The chest X ray posteroanterior view showed mild cardiomegaly. Her 2-dimensional transthoracic echocardiogram (Apical four chamber view) revealed an atrial septal aneurysm
(ASA) bulging into right atrium, with an attached irregular mobile mass which spanned into both atrium, giving impression of a large thrombus (Fig. 1). The color doppler examination showed mild tricuspid regurgitation with estimated right ventricular systolic pressure of 40 mm of Hg. Subsequently, she underwent transesophageal echocardiogram (TEE) which showed a large atrial septal aneurysm (16 mm) with a freely mobile biatrial mass, seems to be attached to the center of aneurysm (Fig. 2A). TEE bicaval view revealed the presence of a 12 mm high ostium secondum atrial septal defect (ASD) in the vicinity of ASA, with left to right shunt (Fig. 2B). All four pulmonary veins normally drained into the left atrium. 3-dimensional TEE showed the
* Corresponding author. Department of Cardiology, Dr. Ram Manohar Lohia Institute of Medical sciences, Gomti Nagar, Lucknow, U.P., PO-226003, India. Tel.: þ91 9559660560; fax: þ91 5222258948. E-mail address: [email protected] (S.K. Vijay). http://dx.doi.org/10.1016/j.ihj.2014.10.401 0019-4832/Copyright © 2014, Cardiological Society of India. All rights reserved.
i n d i a n h e a r t j o u r n a l 6 6 ( 2 0 1 4 ) 7 1 6 e7 1 8
717
heterogeneous echo characteristics of the mass, clear attachment of the mass with the septum by a stalk on the left atrial side (Fig. 3A) and bicaval view showed the spatial relationship of the mass with the ASD (Fig. 3B). Under hypothermic cardiopulmonary bypass, resection of the biatrial septal mass with septal aneurysm and pericardial patch repair of atrial septal defect was done. Histopathological evaluation of the resected mass revealed the diagnosis of atrial myxoma. The patient is doing good after 6- months of follow up.
2.
Fig. 1 e 2D transthoracic echocardiogram apical 4chamber view showing interatrial septal aneurysm with large mass attached to it.
Discussion
Atrial septal aneurysm is a rare localized saccular deformity of the atrial septum, generally arising at the level of fossa ovalis, which bulges to either right or left atrium or both sides. The definitions of ASA vary according to the size1,2 and mobility of the aneurysm.3,4 The commonly associated abnormalities with ASA include patent foramen ovale, atrial septal defect, mitral or tricuspid valve prolapse and sinus of valsalva aneurysm. The congenital malformation of the atrial septum has been suggested to play a causative role in the development of ASA.1 The usual clinical manifestations of ASA can occur due to atrial arrhythmias or thromboembolism.5 ASAs
Fig. 2 e 2D TEE Showing A. Large atrial septal neurysm with two different freely mobile masses attached to each side of ASA B. (Bicaval view) showing 12 mm high OS- ASD in the vicinity of ASA with left to right shunt.
Fig. 3 e 3D TEE showing A. Atrial mass attached to interatrial septum by stalk B. presence of ASD with its relation to biatrial mass.
718
i n d i a n h e a r t j o u r n a l 6 6 ( 2 0 1 4 ) 7 1 6 e7 1 8
can itself act as arrhythmic focus generating atrial tachycardias. The stasis of blood in ASA or in atrium can lead to formation of clots with resultant arterial embolism. Most patients with ASA have left to right shunts and incidence of ASAs is higher in patients with cerebrovascular accidents than in general population.6 TEE is more sensitive in detection of ASAs than transthoracic echocardiography. Myxomas are uncommon primary tumors of the heart, and the myxomas which arise from two different heart chambers is even extremely rare. Cardiac myxomas are usually located in the left atrium and less commonly in the right atrium. Multiple intracardiac myxomas account for less than 5% of all cases and biatrial myxomas in fewer than 2.5%.7 Early diagnosis of cardiac myxomas is usually difficult because the manifestations are usually non specific and major symptoms usually occur in large myxomas leading to intracardiac obstruction or embolization. Other rare cardiac tumors such as myxoid fibrosarcoma presenting as biatrial mass has also been reported.8 Large atrial clot is a close differential diagnosis of atrial myxomas, especially when stalk of myxoma could not be visualized on routine echocardiography. 2D & 3D TEE can provide better differentiation of clot from myxomas due to its spatial higher resolution. Cardiac MRI and multislice CT are other diagnostic modalities, which can be useful in differentiating cardiac tumor from thrombus by enhanced tissue characterization. To the best of our knowledge, association of a biatrial cardiac myxoma with atrial septal aneurysm is not previously reported in the literature. In our case, biatrial mass was erroneously diagnosed as a large clot at a peripheral center and the patient was put on oral anticoagulation. On first look, we also thought the possibility of clot, due to its association with ASA. However, 2-D TEE clearly revealed the presence of ASD with clear extension of the mass which could not be clearly shown by transthoracic echocardiography. 3-D TEE provided the en face views of the mass with visualization of its stalk, spatial relationship and heterogeneous surface characteristics, which strengthened the diagnosis of a cardiac tumor.
Conflicts of interest All authors have none to declare.
Appendix A. Supplementary data Supplementary data related to this article can be found at http://dx.doi.org/10.1016/j.ihj.2014.10.401.
references
1. Hanley PC, Tajik AJ, Hynes JK, et al. Diagnosis and classification of atrial septal aneurysm by two-dimensional echocardiography: report of 80 consecutive cases. J Am Coll Cardiol. 1985;6:1370e1382. 2. Pearson AC, Nagelhout D, Castello R, Gomez CR, Labovitz AJ. Atrial septal aneurysm and stroke: a transesophageal echocardiographic study. J Am Coll Cardiol. 1991;18:1223e1229. 3. Olivares-Reyes A, Chan S, Lazar EJ, Bandlamudi K, Narla V, Ong K. Atrial septal aneurysm: a new classification in two hundred five adults. J Am Soc Echocardiogr. 1997;10:644e656. 4. Longhini Carlo, Brunazzi Maria Cristiana, Musacci Gianfranca, et al. Atrial septal aneurysm: echopolycardiographic study. Am J Cardiol. 1985;56:653e667. 5. Gallet B, Malergue MC, Adam C, et al. Atrial septal aneurysm: a potential cause of systemic embolism. Br Heart J. 1985;53:292e297. 6. Agmon Y, Khandheria BK, Meissner I, et al. Frequency of atrial septal aneurysm in patients with cerebral ischemic events. Circulation. 1999;99:1942e1944. 7. Peachell JL, Mullen JC, Bentley MJ, Taylor DA. Biatrial myxoma: a rare cardiac tumor. Ann Thorac Surg. 1998;65:1768e1769. 8. Gupta P, Kapoor A, Jain M, Kumar S, Agarwal SK, Pande S. A butterfly shaped mobile biatrial cardiac mass: myxoma or something else. Indian Heart J. 2014;66:372e374.