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Transient Hemiparesis Caused by a Left Atrial Myxoma in a Child

2005; Elsevier BV; Volume: 18; Issue: 10 Linguagem: Inglês

10.1016/j.echo.2004.12.012

1097-6795

Kathryn W. Holmes, William Ravekes,

Coronary Artery Anomalies

A previously healthy 4-year-old girl developed symptoms of an upper respiratory tract infection with fever; she was evaluated by her pediatrician on the fifth day of the illness and antibiotics were started because of concern of pneumonia. Although she had transient improvement, she presented to a local emergency department on day 7 of the illness after she developed sudden onset of left facial droop and a limp while playing hide-and-seek with her grandfather. She was subsequently transferred to our institution where her examination was significant for lethargy, right-sided weakness with facial droop, and an unusual low-pitched middiastolic clunk or “tumor plop.”1Adams F.H. Moss A.J. Allen H.D. Moss and Adams’ heart disease in infants, children, and adolescents including the fetus and young adult. 6th ed. Lippincott Williams and Wilkins, Philadelphia2001: 1439Google Scholar Electrocardiography demonstrated frequent premature ventricular contractions, especially when lying on her left side, and echocardiography demonstrated a large left atrial mass (Figure, A) that unfolded with long villi into the left ventricle during diastole (Figure, B). Computed tomography and magnetic resonance imaging of the brain failed to demonstrate evidence of an acute infarct. She then underwent emergency surgical resection of a large 8-cm myxomatous tumor that was adherent to the left atrial septum (Figure, C). Pathology demonstrated a villous myxoma. Blood and urine cultures produced negative results. Her postoperative course was uneventful and she was symptom-free for 1 year with repeated echocardiography; Holter monitor and electrocardiography were normal without evidence of recurrent arrhythmia. Intracardiac tumors are rare in children, with an incidence of cases of 0.0017% on necropsy evaluation.2Freedom R.M. Lee K.J. MacDonald C. Taylor G. Selected aspects of cardiac tumors in infancy and childhood.Pediatr Cardiol. 2000; 21: 299-316Crossref PubMed Scopus (158) Google Scholar The most common intracardiac tumor in childhood is the rhabdomyoma followed by the fibroma and teratoma. Isolated cases of myxomas in children are reported, including one case detected on a prenatal ultrasound.3Isaacs Jr, H. Fetal and neonatal cardiac tumors.Pediatr Cardiol. 2004; 3: 252-273Google Scholar Other neoplasms include hemangioma, hamartoma, lipoma, and Purkinje cell tumor. Malignant and metastatic tumors are extremely rare but include rhabomyosarcoma, fibrosarcoma, leukemia, and neuroblastoma. Although myxomas are the most common intracardiac tumors in adults, comprising up to 60% to 75% of primary cardiac tumors,4Endo A. Ohtahara A. Kinugawa T. Mori M. Fujimoto Y. Yoshida A. et al.Characteristics of 161 patients with cardiac tumors diagnosed during 1993 and 1994 in Japan.Am J Cardiol. 1997; 79: 1708-1711Abstract Full Text Full Text PDF PubMed Scopus (34) Google Scholar they are rare in children. Although often pedunculated with a smooth lobulated surface, one third of cases reported are villous or papillary.5Becker A.E. Primary heart tumors in the pediatric age group a review of salient pathologic features relevant for clinicians.Pediatr Cardiol. 2000; 21: 317-323Crossref PubMed Scopus (148) Google Scholar, 6Pinede L. Duhaut P. Loire R. Clinical presentation of left atrial cardiac myxoma a series of 112 consecutive cases.Medicine (Baltimore). 2001; 80: 159-172Crossref PubMed Scopus (626) Google Scholar The villous extensions are gelatinous with thin and friable extensions that are susceptible to embolization. Although a myxoma is an unusual source of a cerebral embolic event in children, case reports are well described in the pediatric population and, therefore, should be part of the differential diagnosis.7Al Mateen M. Hood M. Trippel D. Insalaco S.J. Otto R.K. Vitikainen K.J. Cerebral embolism from atrial myxoma in pediatric patients.Pediatrics. 2003; 112: e162-e167Crossref PubMed Scopus (47) Google Scholar, 8Ruiz Perez L. Sempere Perez A. Garcia Aonso A. Alenda Gonzalez C. Flores Serrano J. Cerebrovascular stroke as a sign of atrial myxoma in childhood [in Spanish].An Pediatr (Barc). 2003; 58: 273-276PubMed Google Scholar, 9Elderkin R.A. Radford D.J. Primary cardiac tumors in a pediatric population.J Paediatr Child Health. 2002; 38: 173-177Crossref PubMed Scopus (56) Google Scholar, 10Nogueira D.C. Bontempo D. Menardi A.C. Vicente W.V. Ribeiro P.J. Evora P.R. Left atrial myxoma as the cause of syncope in an adolescent.Arq Bras Cardiol. 2003; 81 (206-5)Crossref Scopus (4) Google Scholar Echocardiographically, myxomas are generally homogenous structures with a pedunculated base with a smooth lobulated surface. Some, as in our case, have a villous appearance; these are known to be friable and are prone to embolization. Although myxomas are most commonly adherent to the left atrial septum, they may be located in the right atrium, adherent to atrioventricular valves, or, rarely, ventricular in origin. The tumors may be so large as to fall through the mitral or tricuspid valves, thus, obstructing ventricular filling, increasing atrial size, and causing a characteristic tumor plop1Adams F.H. Moss A.J. Allen H.D. Moss and Adams’ heart disease in infants, children, and adolescents including the fetus and young adult. 6th ed. Lippincott Williams and Wilkins, Philadelphia2001: 1439Google Scholar, 6Pinede L. Duhaut P. Loire R. Clinical presentation of left atrial cardiac myxoma a series of 112 consecutive cases.Medicine (Baltimore). 2001; 80: 159-172Crossref PubMed Scopus (626) Google Scholar on auscultation. Interestingly, although the initial history of fever in our case may be incidental, there are several reports in the literature of fever as a presenting symptom6Pinede L. Duhaut P. Loire R. Clinical presentation of left atrial cardiac myxoma a series of 112 consecutive cases.Medicine (Baltimore). 2001; 80: 159-172Crossref PubMed Scopus (626) Google Scholar including one report of a left atrial myxoma presenting with a significant systemic inflammatory response.11Maze Y. Kajimoto M. Tenpaku H. Satou T. Left atrial myxoma with severe inflammatory response.Jpn J Thorac Cardiovasc Surg. 2004; 52: 221-223Crossref PubMed Scopus (6) Google Scholar In addition, the premature ventricular contractions are unusual in this case. There are occasional reports of ventricular arrhythmias associated with myxomas12Bauer M.F. Aebert H. Zurbrugg H. Ruschoff J. Birnbaum D.E. Torsades de pointes arrhythmia in a patient with left ventricular myxoma.Chest. 1994; 105: 1876-1878Crossref PubMed Scopus (21) Google Scholar, 13Badui D.E. Cordero E. Soberanis N. Verdin R. Arguero R. Cardiac myxoma a report of 23 cases [in Spanish].Gac Med Mex. 1992; 128: 245-252PubMed Google Scholar; however, most arrhythmias in adults are atrial in origin. We speculate the premature ventricular contractions may have been the result of ventricular response to obstruction of the tumor within the mitral valve or the villi irritating the left ventricular wall as they passed through the mitral valve. These premature ventricular contractions resolved after resection and were not evident on follow-up Holter recording at 1 year postresection. The importance of follow-up cannot be underestimated. Although most causes of recurrence are thought to be secondary to incomplete resection, there is another group with recurrence in multiple sites that are associated with Carney’s syndrome, a constellation of myxomas, spotty pigmentation, and endocrine overactivity14Edwards A. Bermudez C. Piwonka G. Berr M.L. Zamorano J. Larrain E. et al.Carney’s syndrome complex myxomas; report of four cases and review of the literature.Cardiovasc Surg. 2002; 10: 264-275Crossref PubMed Scopus (43) Google Scholar; this complex of genes has been mapped to a locus on chromosome 17q2(2). Also, there is one isolated case report of multiple intracranial aneurysms as a late complication of a left atrial myxoma excision 5 years previously.15Jean W.C. Walski-Easton S.M. Nussbaum E.S. Multiple intracranial aneurysms as delayed complications of an atrial myxoma case report.Neurosurgery. 2001; 49: 200-202PubMed Google Scholar Although there was no evidence of tumor recurrence on transthoracic echocardiography, clearly patients who present with new neurologic symptoms may require additional imaging. This case report demonstrates an unusual presentation of a cardiac tumor in a child where possible significant neurologic damage was prevented by rapid identification of the tumor by echocardiography.