Atrial myxoma with hemiplegia Luciana Rotaru1, Mircea-Cătălin Forțofoiu2, Octavian Istrătoaie3, Cristian Constantin4, Dana Maria Albulescu4, Maria Forțofoiu1. 1 Department of Emergency Medicine and First Aid – University of Medicine and Pharmacy of Craiova, 4 Petru Rareș Street, zip code 200349, Craiova, Dolj County, Romania, lucianarotaru@yahoo.com; maria25081973@yahoo.com. 2 Department of Internal Medicine, University of Medicine and Pharmacy of Craiova; 4 Petru Rareș Street, zip code 200349, Craiova, Dolj County, Romania, catalin1972@hotmail.com. 3 Department of Cardiology, University of Medicine and Pharmacy of Craiova; 4 Petru Rareș Street, zip code 200349, Craiova, Dolj County, Romania, droctavist@yahoo.com. 4 Department of Radiology and Medical Imaging - University of Medicine and Pharmacy of Craiova; 4 Petru Rareș Street, zip code 200349, Craiova, Dolj County, Romania, stiric@lycos.com, nicolescudm@yahoo.com. Corresponding author: Associate Professor, Mircea-Cătălin Forțofoiu MD, PhD – 4 Petru Rareș Street, zip code 200349, Craiova, Dolj County, Romania, phone +40722320438, fax +40351401339, e-mail: catalin1972@hotmail.com. © The Author 2018. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For Permissions, please email: journals.permissions@oup.com Downloaded from https://academic.oup.com/qjmed/advance-article-abstract/doi/10.1093/qjmed/hcy039/4885378 by guest on 24 February 2018 Learning point for clinicians Sudden loss of consciousness without prodromal signs and symptoms in children can be the consequence of embolism in an important vessel of cerebral circulation. The source of the embolus can be the presence of a myxoma in the left atrium with clinically silent evolution even during high intensity training. Background Loss of consciousness in children and adolescents is produced, in most cases, of neurally mediated hypotension whose clinical expression is vasovagal syncope. In the case of other types of syncope, the mechanisms involved may be: impaired of the right or left ventricular outflow tract, arrhythmias, seizures, cerebral circulation disorders, cerebrospinal fluid disruption, anxiety or depression1. Case Report We present the case of an 8-year-old athlete with no significant personal history brought with the ambulance in the emergency department for loss of consciousness, vomiting and seizures with sudden onset, without prodromal signs and symptoms while she was in the playground and jumped on an elastic trampoline. After 30 minutes of beginning sedation, the patient presents right hemiplegia, difficulty in swallowing and clinical signs of right body decerebration. Anamnesis from relatives, physical examination, blood analysis and electrocardiogram has given poor data to establish a certainty diagnosis. Subsequently, parents reported that grandmother was diagnosed with atrial myxoma, but the mother of the patient did not have it. Magnetic resonance imaging (MRI) scan of brain scan revealed multiple areas of ischemia in the cerebellum, pons and medulla also magnetic resonance angiography (MRA) techniques, 3D including time-of-flight (TOF) sequences showed absence of blood flow in the left middle cerebral artery (Figure 1). To determine the source of the emboli, the next exploration was echocardiography which revealed a giant left atrial myxoma, polylobate, with transvalvular protrusion which resulted in 3-4-degree mitral regurgitation (Figure 2). Subsequently, the patient was sent to intensive care for monitoring and treatment but, unfortunately, died a week after the diagnosis was established. Discussion The absence of any clinical symptoms or signs prior to the syncopal episode, both at rest and during training, did not impose a deeper exploration at this patient. Downloaded from https://academic.oup.com/qjmed/advance-article-abstract/doi/10.1093/qjmed/hcy039/4885378 by guest on 24 February 2018 Syncope represents approximately 1% of the total paediatric emergency which addresses to the emergency rooms, given that almost to 15% of children are reported the appearance of at least one syncope in the first twenty years of life2. In 12-90% of syncope with neurological etiology, loss of consciousness is followed by convulsions3. The surprise in this case was the presence of left atrial myxoma, asymptomatic, even in the conditions in which, patient was subjected to intense and prolonged efforts. In this case, the myxoma was not transmitted from mother to daughter, but from grandma to granddaughter. Female sex is most commonly affected with a frequency of 65% and the medical literature records that family aggregation is 4.5-10% for myxoma which requires screening echocardiography for first-degree relatives4. The clinical picture of the atrial myxoma is the result of the triad cardiac obstruction, embolism and chronic systemic inflammation, most patients having symptoms and signs characteristic of at least one of them5. In most patients the electrocardiogram shows left atrial enlargement, atrial conduction disturbances or arrhythmias are sporadic. Chest radiography may be normal in one third of patients with atrial myxoma. Myxoma represents 30-50% of the total primitive tumours of the heart, affecting adults in the 3-5 decades of life, but it can be found between 1 and 83 years. It develops in 83% of cases at the left atrium, usually attached to the oval fossa region of the interatrial septum6. In this case, it seems that, at the time of the effort occurred concurrently both detachment of emboli that migrated into the cerebral circulation and the protrusion of the myxoma through the mitral orifice. Conclusion This case has all the ingredients that leave very limited therapeutic possibilities: ignoring or omitting the family history, absence of screening echocardiography, clinically silent atrial myxoma, and sudden onset with cerebral and cardiac complications. Disclosure All authors have participated in the work and have reviewed and agree with the content of the article. None of the article contents are under consideration for publication in any other journal or have been published in any journal. The manuscript has been read and approved by all the authors, contributed equally to preparing this paper, and share first authorship Conflict of interest: Nothing to declare. Downloaded from https://academic.oup.com/qjmed/advance-article-abstract/doi/10.1093/qjmed/hcy039/4885378 by guest on 24 February 2018 References 1. Jeffrey B. Anderson, Martha Willis, Heidi Lancaster, Karen Leonard, Cameron Thomas. The Evaluation and Management of Paediatric Syncope. Pediatr Neurol 2016; 55: 6-13 2. Driscoll DJ, Jacobsen SJ, Porter CJ, Wollan PC. Syncope in children and adolescents. J Am Coll Cardiol. 1997; 29:1039-1045. 3. Bergfeldt L. Differential diagnosis of cardiogenic syncope and seizure disorders. Heart. 2003; 89:353-358. 4. Ekmektzoglou KA, Samelis GF, Xanthos T: Heart and tumors: Location, metastasis, clinical manifestations, diagnostic approaches and therapeutic considerations. J Cardiovasc Med (Hagerstown). 2008; 9:769. 5. Burke A, Virmani R: Tumors of the Heart and Great Vessels. Atlas of Tumor Pathology. 3rd Series, Fascicle 16. Washington, DC, Armed Forces Institute of Pathology, 1996. 6. Bruce McManus. Primary Tumors of the Heart. In: Bonow RO, Mann DL, Zipes DP, Libby P. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 9th edition. Philadelphia: Elsevier Saunders; 2012:1640-41. Downloaded from https://academic.oup.com/qjmed/advance-article-abstract/doi/10.1093/qjmed/hcy039/4885378 by guest on 24 February 2018 Figure 1. 3D TOF sequences showing the absence of blood flow in the left middle cerebral artery 78x88mm (150 x 147 DPI) Downloaded from https://academic.oup.com/qjmed/advance-article-abstract/doi/10.1093/qjmed/hcy039/4885378 by guest on 24 February 2018 Figure 2. B mode echocardiography shows a large homogenous echoic mass in the left atrium of the heart associated with transvalvular protrusion 82x86mm (150 x 150 DPI) Downloaded from https://academic.oup.com/qjmed/advance-article-abstract/doi/10.1093/qjmed/hcy039/4885378 by guest on 24 February 2018