C43-18 Idiopathic Chylopericardium - A Case Report

Abstract IntroductionChylopericardium (IC) is a rare condition associated with the accumulation of chylous fluid in the pericardial space. Idiopathic chylopericardium is the most common etiology. Description of Case45-year old female with medical history only significant for primary Raynaud’s phenomenon, presented with 2 months of an increasingly noticeable nonproductive cough, generally noted during extended conversation. This cough was associated with intermittent voice hoarseness, and the feeling of needing to exhale first to achieve a subjectively “complete” inspiratory excursion. Symptoms were intermittent, daily, and mild. These symptoms prompted a chest radiograph which was normal with the exception of a prominent cardiac silhouette, raising the concern for pericardial effusion. Subsequent echocardiography confirmed a large pericardial effusion which appeared simple, non-loculated, and without features of cardiac tamponade. One week after presentation, a total of 1.6 liters of yellow, milky fluid was drained (to completion) via transapical approach revealing triglyceride 1800mg/dL, +chylomicrons, 83% lymphocytes (666 nucleated cells). The chylous fluid flow cytometry, cytology, mycobacterial tuberculosis PCR (and extensive microbiology PCR studies) at the University of Washington were unremarkable. An extensive rheumatologic serology panel was completely negative. CT imaging of her chest and abdomen/pelvis were completely unremarkable in terms of masses/nodules, lymphadenopathy, or other abnormal serosal surfaces. The patient was started on a low-fat diet, medium chain triglyceride supplementation, and subcutaneous octreotide. Unfortunately, after initial improvement in all symptoms, a repeat echocardiogram one week after drainage demonstrated complete recurrence of the chylous pericardial fluid and associated symptoms. Consequently, one month after presentation, she underwent combined repeat complete drainage of the chylopericardium and lymphangiogram, the latter with successful embolization of a noted leak into the pericardium at the level of the mid-thoracic spine, with multiple coils and N-acetyl cyanoacrylate. As anticipated, the symptoms significantly improved once again. Six months after embolization, sequential echocardiography shows a stable mild to moderate pericardial effusion without increasing size over time and mild ongoing symptomatology, although markedly improved when compared to the symptom complex pre-embolization. DiscussionThis case highlights that mild respiratory complaints including a nonproductive cough may be the presenting symptoms of idiopathic chylopericardium. This respiratory symptom complex appeared to improve or deteriorate depending on the size and extent of the pericardial effusion. The mechanism of the cough is not clear but may be related to mechanical compression of lung juxtaposed to the large pericardial effusion. This abstract is funded by: None