Myxomas are primary cardiac tumors of the most common pathology, with 75% of myxomas originating in the left atrium either at the mitral annulus or the fossa ovalis border of the interatrial septum; the remaining 20% come from the right atrium, and about 5% arise from both atria and the ventricle. Atrial myxomas are usually asymptomatic and are often detected when associated with complications due to tumor obstruction or emboli causing stroke. Due to the absence of symptoms, a diagnosis of a myxoma is often delayed until a significant embolic phenomenon, such as a stroke, occurs. We encountered such a scenario when a 62-year-old female presented to our hospital with right arm and leg weakness with her workup of stroke with a brain MRI showing numerous subacute infarcts consistent with embolic etiology. A routine transthoracic echo revealed a 2 × 5 cm atrial myxoma, which then led to the diagnosis of atrial myxoma along with a discovery of a patent foramen ovale (PFO), which subsequent surgical removal and PFO closure for definitive prevention of future embolic events. In this article, we aim to review the clinical presentation of cardiac myxoma along with appropriate investigations and treatment along with validation of the importance of timely echocardiograms with bubble studies in patients presenting with embolic stroke that can detect the atrial myxomas as a rare but potential cause of ischemic stroke.
Arboix, A. and Alioc, J. (2010) Cardioembolic Stroke: Clinical Features, Specific Cardiac Disorders and Prognosis. Current Cardiology Reviews , 6, 150-161. https://doi.org/10.2174/157340310791658730
Vahedi, K. and Amarenco, P. (2000) Cardiac Causes of Stroke. Current Treatment Options in Neurology , 2, 305-317. https://doi.org/10.1007/s11940-000-0048-4
(1989) Cardiogenic Brain Embolism. The Second Report of the Cerebral Embolism Task Force. Archives of Neurology , 46, 727-743.
Vermeulen, T., Conraads, V.M., Vrints, C. and Rodrigus, I.E. (2009) Recurrent Left Ventricular Myxoma Presenting as Cerebrovascular Accidents in a Teenage Girl. Acta Cardiologica , 64, 811-814. https://doi.org/10.2143/ac.64.6.2044749
Sanya, E.O., Kolo, P.M., Adamu, U.G., Opadijo, O.G., Wahab, K.W., Mustapha, A.F., et al . (2008) Intracardiac Tumor: A Risk Factor for Stroke in the Young—A Case Report. Nigerian Journal of Clinical Practice , 11, 81-84.
Lam, K.Y., Dickens, P. and Chan, A.C. (1993) Tumors of the Heart. A 20-Year Experience with a Review of 12,485 Consecutive Autopsies. Archives of Pathology & Laboratory Medicine , 117, 1027-1031. https://pubmed.ncbi.nlm.nih.gov/8215825/
Cohen, R., Singh, G., Mena, D., Garcia, C.A., Loarte, P. and Miller, B. (2012) Atrial Myxoma: A Case Presentation and Review. Cardiology Research , 3, 41-44. https://doi.org/10.4021/cr145w
Iyer, P., Aung, M.M., Awan, M.U., Kososky, C. and Barn, K. (2016) A Case of Large Atrial Myxoma Presenting as an Acute Stroke. Journal of Community Hospital Internal Medicine Perspectives , 6, Article No. 29604. https://doi.org/10.3402/jchimp.v6.29604
Reynen, K. (1995) Cardiac Myxomas. New England Journal of Medicine , 333, 1610-1617. https://doi.org/10.1056/nejm199512143332407
O’Rourke, F., Dean, N., O’Rourken, M.S., et al . (2003) Atrial Myxoma as a Cause of Stroke: Case Report and Discussion. CMAJ , 169, 1049-1051.
McCarthy, P.M., Piehler, J.M., Schaff, H.V., Pluth, J.R., Orszulak, T.A., Vidaillet, H.J., et al . (1986) The Significance of Multiple, Recurrent, and “Complex” Cardiac Myxomas. The Journal of Thoracic and Cardiovascular Surgery , 91, 389-396. https://doi.org/10.1016/s0022-5223(19)36054-4
Kirschner, L.S., Carney, J.A., Pack, S.D., Taymans, S.E., Giatzakis, C., Cho, Y.S., et al . (2000) Mutations of the Gene Encoding the Protein Kinase a Type I- α Regulatory Subunit in Patients with the Carney Complex. Nature Genetics , 26, 89-92. https://doi.org/10.1038/79238
Knepper, L.E., Biller, J., Adams, H.P. and Bruno, A. (1988) Neurologic Manifestations of Atrial Myxoma. A 12-Year Experience and Review. Stroke , 19, 1435-1440. https://doi.org/10.1161/01.str.19.11.1435
Kassab, R., Wehbe, L., Badaoui, G., et al . (1999) Recurrent Cerebrovascular Accident: an Unusual and Isolated Manifestation of Myxoma of the Left Atrium. Journal Medical Libanais , 47, 246-250.
Hutton, J.T. (1981) Atrial Myxoma as a Cause of Progressive Dementia. Archives of Neurology , 38, 533. https://doi.org/10.1001/archneur.1981.00510080095019
Bienfait, H.P. and Moll, L.C.M. (2001) Fatal Cerebral Embolism in a Young Patient with an Occult Left Atrial Myxoma. Clinical Neurology and Neurosurgery , 103, 37-38. https://doi.org/10.1016/s0303-8467(00)00118-9
Oktaviono, Y.H., Saputra, P.B.T., Arnindita, J.N., Afgriyuspita, L.S., Kurniawan, R.B., Pasahari, D., et al . (2024) Clinical Characteristics and Surgical Outcomes of Cardiac Myxoma: A Meta-Analysis of Worldwide Experience. European Journal of Surgical Oncology , 50, Article ID: 107940. https://doi.org/10.1016/j.ejso.2023.107940
Sohal, R.S., Shergill, K.K., Nagi, G.S. and Pillai, H.J. (2020) Atrial Myxoma—An Unusual Cause of Ischemic Stroke in Young. Autopsy and Case Reports , 10, e2020178. https://doi.org/10.4322/acr.2020.178
Islam, A.K.M.M. (2022) Cardiac Myxomas: A Narrative Review. World Journal of Cardiology , 14, 206-219. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9048271/
Pérez de Isla, L., de Castro, R., Zamorano, J.L., Almería, C., Moreno, R., Moreno, M., Lima, P. and García Fernández, M.A. (2002) Diagnosis and Treatment of Cardiac Myxomas by Transesophageal Echocardiography. Journal of the American College of Cardiology , 90, 1419-1421. https://doi.org/10.1016/S0002-9149(02)02891-6
Abbas, A., Garfath-Cox, K.A., Brown, I.W., Shambrook, J.S., Peebles, C.R. and Harden, S.P. (2015) Cardiac MR Assessment of Cardiac Myxomas. British Journal of Radiology , 88, Article ID: 20140599. https://doi.org/10.1259/bjr.20140599
Bakaeen, F.G., Reardon, M.J., Coselli, J.S., Miller, C.C., Howell, J.F., Lawrie, G.M., et al . (2003) Surgical Outcome in 85 Patients with Primary Cardiac Tumors. The American Journal of Surgery , 186, 641-647. https://doi.org/10.1016/j.amjsurg.2003.08.004
Semb, B.K.H. (1984) Surgical Considerations in the Treatment of Cardiac Myxoma. The Journal of Thoracic and Cardiovascular Surgery , 87, 251-259. https://doi.org/10.1016/s0022-5223(19)37419-7
Khan, M.A., Khan, A.A. and Waseem, M. (2008) Surgical Experience with Cardiac Myxomas. Journal of Ayub Medical College Abbottabad , 20, 76-79.
Farjat-Pasos, J.I., Chamorro, A., Lanthier, S., Robichaud, M., Mengi, S., Houde, C. and Rodés-Cabau, J. (2023) Cerebrovascular Events in Older Patients with Patent Foramen Ovale: Current Status and Future Perspectives. Journal of Stroke , 25, 338-349. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10574307/#:~:text=Every%20major%20clinical%20guideline%20on,with%20a%20PFO%2Drelated%20stroke
Sandrini, F. and Stratakis, C. (2003) Clinical and Molecular Genetics of Carney Complex. Molecular Genetics and Metabolism , 78, 83-92. https://doi.org/10.1016/s1096-7192(03)00006-4
Stratakis, C.A., Kirschner, L.S. and Carney, J.A. (2001) Clinical and Molecular Features of the Carney Complex: Diagnostic Criteria and Recommendations for Patient Evaluation. The Journal of Clinical Endocrinology & Metabolism , 86, 4041-4046. https://doi.org/10.1210/jcem.86.9.7903
Basson, C.T. and Aretz, H.T. (2002) Case Records of the Massachusetts General Hospital. Weekly Clinicopathological Exercises. Case 11-2002. A 27-Year-Old Woman with Two Intra-Cardiac Masses and a History of Endocrinopathy. New England Journal of Medicine , 346, 1152-1158. https://doi.org/10.1056/nejmcpc010057