Left Atrial Myxoma and Antiphospholipid Syndrome—A Case Report

Abstract

Cardiac myxoma (CM) is the most frequent type of primary cardiac neoplasm and is responsible for 58 to 80% of primary cardiac tumors. The antiphospholipid syndrome (APS) occurs most commonly in the systemic lupus erythematosus but it can be also found in other conditions. The coexistence of CM and APS is rarely described in the literature. We report an unusual case of the left atrial myxoma and concomitant APS in a female patient who presented with right-sided hemiplegia. Although rare, we must think about the CM in patients with a newly diagnosed APS and left atrial mass. Nevertheless, we must make a distinction from other possible cardiac structures, especially atrial thrombus. Transthoracic echocardiography is the most frequently used initial imaging modality to detect CM. The aim of this case report was to emphasize that additional imaging modalities and multidisciplinary approach are mandatory in making a proper diagnosis and to choose a further treatment strategy.

Keywords

cardiac tumor - antiphospholipid syndrome - echocardiography - left atrial myxoma - thromboembolism - ischemia

Publication History

Article published online:
11 December 2021

• References

• 1 ElBardissi AW, Dearani JA, Daly RC. et al. Analysis of benign ventricular tumors: long-term outcome after resection. J Thorac Cardiovasc Surg 2008; 135 (05) 1061-1068
• 2 Tazelaar HD, Locke TJ, McGregor CG. Pathology of surgically excised primary cardiac tumors. Mayo Clin Proc 1992; 67 (10) 957-965
• 3 Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases. Medicine (Baltimore) 2001; 80 (03) 159-172
• 4 Roque A, Kimbrough T, Traner C. et al. Somatic PRKAR1A mutation in sporadic atrial myxoma with cerebral parenchymal metastases: a case report. J Med Case Reports 2019; 13 (01) 389
• 5 Tufano A, Di Minno MND, Guida A, Lembo M, Di Minno G, Galderisi M. Cardiac manifestations of antiphospholipid syndrome: clinical presentation, role of cardiac imaging, and treatment strategies. Semin Thromb Hemost 2019; 45 (05) 468-477
• 6 Tenedios F, Erkan D, Lockshin MD. Cardiac involvement in the antiphospholipid syndrome. Lupus 2005; 14 (09) 691-696
• 7 Basso C, Valente M, Poletti A, Casarotto D, Thiene G. Surgical pathology of primary cardiac and pericardial tumors. Eur J Cardiothorac Surg 1997; 12 (05) 730-737 , discussion 737–738
• 8 Burke AP, Virmani R. Cardiac myxoma. A clinicopathologic study. Am J Clin Pathol 1993; 100 (06) 671-680
• 9 Cianciulli TF, Cozzarin A, Soumoulou JB. et al. Twenty years of clinical experience with cardiac myxomas: diagnosis, treatment, and follow up. J Cardiovasc Imaging 2019; 27 (01) 37-47
• 10 Scheffel H, Baumueller S, Stolzmann P. et al. Atrial myxomas and thrombi: comparison of imaging features on CT. AJR Am J Roentgenol 2009; 192 (03) 639-645
• 11 Reynen K. Cardiac myxomas. N Engl J Med 1995; 333 (24) 1610-1617
• 12 Oliveira R, Branco L, Galrinho A. et al. Cardiac myxoma: a 13-year experience in echocardiographic diagnosis. Rev Port Cardiol 2010; 29 (7-8) 1087-1100
• 13 Knepper LE, Biller J, Adams Jr HP, Bruno A. Neurologic manifestations of atrial myxoma. A 12-year experience and review. Stroke 1988; 19 (11) 1435-1440
• 14 Colin GC, Dymarkowski S, Gerber B, Michoux N, Bogaert J. Cardiac myxoma imaging features and tissue characteristics at cardiovascular magnetic resonance. Int J Cardiol 2016; 202: 950-951
• 15 Kolitz T, Shiber S, Sharabi I, Winder A, Zandman-Goddard G. Cardiac manifestations of antiphospholipid syndrome with focus on its primary form. Front Immunol 2019; 10: 941
• 16 Miyakis S, Lockshin MD, Atsumi T. et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 2006; 4 (02) 295-306
• 17 Barbhaiya M, Erkan D. Primary thrombosis prophylaxis in antiphospholipid antibody-positive patients: where do we stand?. Curr Rheumatol Rep 2011; 13 (01) 59-69
• 18 Cervera R. Recent advances in antiphospholipid antibody-related valvulopathies. J Autoimmun 2000; 15 (02) 123-125
• 19 Cervera Miguel JI, Navarro Ibáñez V, Calabuig Muñoz E, Tordera Higón P, Montero Alonso M. Antiphospholipid syndrome. The use of transesophageal echocardiography. Med Interna 2003; 20 (10) 532-533
• 20 Gertner E, Leatherman JW. Intracardiac mural thrombus mimicking atrial myxoma in the antiphospholipid syndrome. J Rheumatol 1992; 19 (08) 1293-1298
• 21 Quintanilla S, Ferrer S, Bravo M. Myxoma and antiphospholipid antibody syndrome. Rev Med Chil 1998; 126 (06) 670-676
• 22 Pliakos C, Alexiadou E, Metallidis S. et al. Right atrium myxoma coexisting with antiphospholipid syndrome: a case report. Cardiovasc Ultrasound 2009; 7: 47
• 23 Esteban E, Blanes A, Moreno MT, Cánovas SJ, Montero JA, Sotillo JF. Right atrial myxoma in a patient with antiphospholipid antibodies. Rev Esp Cardiol 2002; 55 (09) 999-1002
• 24 Wen XY, Chen YM, Yu LL. et al. Neurological manifestations of atrial myxoma: a retrospective analysis. Oncol Lett 2018; 16 (04) 4635-4639
• 25 Cao GF, Bi Q, Cao L, Wang C. The clinical characteristics of stroke in young patients with cardiac myxoma. Zhonghua Nei Ke Za Zhi 2017; 56 (04) 263-267
• 26 Riedl J, Pabinger I, Ay C. Platelets in cancer and thrombosis. Hamostaseologie 2014; 34 (01) 54-62
• 27 Obrenović-Kirćanski B, Mikić A, Parapid B. et al. A 30-year-single-center experience in atrial myxomas: from presentation to treatment and prognosis. Thorac Cardiovasc Surg 2013; 61 (06) 530-536
• 28 Uthman I, Noureldine MHA, Ruiz-Irastorza G, Khamashta M. Management of antiphospholipid syndrome. Ann Rheum Dis 2019; 78 (02) 155-161