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PEDIATRICS Vol. 113 No. 4 April 2004, pp. e374-e376

ELECTRONIC ARTICLE

Intracardiac Yolk Sac Tumor and Dysrhythmia as an Etiology of Pediatric Syncope

Melinda J. Morin, MD*, Richard A. Hopkins, MD{ddagger}, William S. Ferguson, MD§ and James W. Ziegler, MD||

* Division of Pediatric Critical Care
§ Division of Pediatric Hematology-Oncology
|| Division of Pediatric Cardiology, Department of Pediatrics
{ddagger} Division of Cardiothoracic Surgery, Department of Cardiothoracic Surgery, Rhode Island Hospital and Brown University School of Medicine, Providence, Rhode Island

This report describes a new etiology of pediatric syncope. Epilepsy, brain anomalies, infection, electrolyte abnormalities, and trauma are commonly identified etiologies of seizures in the pediatric population. We report here a child with third-degree heart block and right ventricular outflow tract obstruction related to an intracardiac tumor presenting with syncope and seizure-like activity. Echocardiography revealed a large (3 x 8-cm) intracardiac mass filling the right atrium, extending across the tricuspid valve into the right ventricle and crossing the atrial septum into the left atrium, extending into the left ventricular outflow tract. She underwent emergent cardiopulmonary bypass with removal of the majority of the tumor mass, clearing both the left and right ventricular outflow tracts of obstruction and repairing the tricuspid valve. Postoperative cardiac conduction remained blocked and required permanent pacing. The initial serum {alpha}-fetoprotein level was grossly elevated, and the tumor showed characteristic histopathologic features of a yolk sac tumor. Four years after the completion of her chemotherapy, she remains clinically well, with no evidence of recurrent tumor by echocardiography or radiographic studies, and her {alpha}-fetoprotein remains in the normal range. The clinical manifestations of tumor infiltration of the heart with complete heart block resulting in loss of consciousness with tonic-clonic movements are detailed. Although rare, cardiac syncope has multiple known causes and should be suspected in any patient with sudden loss of consciousness and pallor. In the pediatric population, cardiac rhythm disturbances are typically the result, rather than the cause, of acute cardiac emergencies. Pediatricians should be aware of depressed cardiac output and dysrhythmias as etiologies of new-onset syncope. Evaluation should include a cardiac assessment with electrocardiogram to exclude a life-threatening arrhythmia as a potential cause.

Key Words: yolk sac tumor • dysrhythmia • seizure

Abbreviations: A-V, atrioventricular • ECG, electrocardiogram • AFP, {alpha}-fetoprotein

Received for publication Aug 19, 2003; Accepted Nov 24, 2003.