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Cardiac tamponade, the accumulation of fluid in the pericardial space, leads to impaired venous return, loss of left ventricular preload, and hemodynamic collapse. The many causes of tamponade include malignancy, infection, inflammation, connective tissue disorders, and uremia.
Herein, we report the case of a young woman who presented with syncope. She was found to have cardiac tamponade secondary to a chylous pericardial effusion that was due to a mature and benign anterior mediastinal cystic teratoma. Numerous reports have described pericardial effusions secondary to an anterior mediastinal cystic teratoma; however, to our knowledge, this is the 1st case of a teratoma causing chylopericardium that presented as tamponade.
Cardiac tamponade is a well-known clinical entity that can be rapidly fatal if it is not treated promptly. The condition is caused by the accumulation of fluid in the pericardial space, which leads to impaired venous return, loss of left ventricular preload, and hemodynamic collapse. The pericardial effusion can be a sequela of malignancy, inflammation, infection, connective tissue disorders, uremia, pleural effusion with extension, or trauma, or it can be idiopathic.1 A rarer cause of tamponade is the accumulation of chyle. Chylopericardium can result from inflammatory disorders, such as pancreatitis; however, most cases of chylopericardium can ultimately be attributed to microperforations in the thoracic duct.2–7 We present the case of a young woman who experienced cardiac tamponade from chylopericardium that was secondary to an anterior mediastinal mature cystic teratoma.
In August 2003, an 18-year-old woman with no personal or family history of cardiac disorders presented at our emergency department after an episode of syncope. She had spent the day at an amusement park where the ambient temperature was high, and she reportedly had not consumed much liquid all day. While standing in line, she had felt dizzy and passed out, and then rapidly regained consciousness and awareness of her surroundings. She had not experienced chest pain, dyspnea on exertion, shortness of breath, nausea, or palpitations. There was no loss of bowel or bladder function.
She was rehydrated with intravenous fluids and sent home. Over the next few weeks, she developed a fever of 102 °F as measured orally, and she began to experience nonexertional chest pain and shortness of breath. Her pediatrician diagnosed infectious mononucleosis. She experienced 3 more syncopal episodes over the next 2 to 3 weeks, along with chest pain and shortness of breath.
She was re-evaluated and referred to a cardiologist. Physical examination revealed pulsus paradoxus and jugular venous distention without pericardial friction rub or murmurs. Electrocardiography showed low-voltage complexes and nonspecific T-wave changes in leads III and aVF (Fig. 1). An echocardiogram revealed extensive anterior and posterior pericardial effusion, with evidence of cardiac tamponade.
The patient was immediately sent to the hospital for pericardiocentesis. Approximately 900 mL of thick yellow fluid was drained from the pericardium and analyzed; it contained 503 mg/dL total cholesterol and 328 mg/dL triglycerides, consistent with chylous pericardial fluid. Gram-stain, bacterial, echinococcal, and viral cultures were negative. A pericardial drain was placed. Repeat echocardiography confirmed reaccumulation of the effusion. A computed tomograph-ic scan of the chest revealed a 10.3 × 8.7 × 11-cm cys-tic mass in the anterior mediastinum, extending into the right hemithorax with pericardial effusion (Fig. 2). Surgical resection revealed a mass (16 × 11 × 7 cm) that consisted of skin (Fig. 3), bronchial tissue, pancre-atic tissue (Fig. 4), smooth muscle (Fig. 5), fat, and brain tissue. The mass appeared not to have ruptured, but there was a communication with the pericardium. Histopathologic analysis confirmed that the mass was a mature cystic teratoma. Lymph node biopsies were negative for metastasis. The patient did well and was discharged from the hospital on the 7th postoperative day without complications.
Chylopericardium is a relatively rare clinical entity that constitutes only a fraction of cardiac tamponade cases. Inflammatory disorders and malignancies are the usual causes of chylous pericardial effusions; in such cases, there is demonstrable communication between the thoracic duct and the pericardium that may or may not be visible on lymphangiography.8–10 Disruptions in the integrity of the thoracic duct can occur consequent to trauma (including surgery and catheter-related sequelae), thereby creating a conduit for chyle to pass into the pericardial and pleural spaces.11,12 Numerous reports describe chylothorax and chylous pericardial effusions that have resulted from primary pulmonary neoplasms such as lymphangioleiomyomatosis and signet-ring cell carcinoma, and from extrapulmonary malignancies such as leukemia.13–16
Anterior mediastinal teratomas are known to cause both pleural and pericardial effusions, and there are documented cases of cardiac tamponade due to local invasion and rupture into the pericardial space.17–21 When these tumors rupture, however, analysis frequently reveals exudative fluid that is hemorrhagic but not chylous. In a case similar to ours, a young woman had turbid fluid drained from the pericardium; however, on analysis, that fluid was not chyle.22 Although multiple manifestations of cardiopulmonary disorders secondary to mature cystic teratoma have been reported,23–29 there are, to our knowledge, no other reported cases of cardiac tamponade from chylopericardium that originated from an anterior mediastinal mature, benign cystic teratoma.
Address for reprints: David J. Revere, MD, Department of Medicine, Long Island Jewish Medical Center, 270–05 76th Avenue, New Hyde Park, NY 11040. E-mail: moc.oohay@dmrd;f5000x#&rd