Constrictive Pericarditis after Repeated Accumulation of Pericardial Effusion due to Long-term Chylopericardium

Primary chylopericardium is a rare disease characterized by the accumulation of chylous fluid containing high triglyceride in the pericardial cavity without a specific cause. We report a case of severe constrictive pericarditis due to 9-year history of primary chylopericardium and surgical ligation of the thoracic duct and pericardial window creation. A 24-year old woman diagnosed as primary chyropericardium, was admitted to our hospital for further treatment of heart failure. She had undergone ligation of the thoracic duct and the creation of a pericardial window. She complained of exertional dyspnea and physical examination revealed elevated jugular venous pressure. Chest X-ray showed a normal cardiac silhouette with extensive transudative pleural effusion. Echocardiogram showed severe mitral regurgitation which was slightly observed before surgery, a few pericardial effusion. Left and right ventricular simultaneous pressure recordings revealed equilibration of both ventricular diastolic pressures and a dip-and-plateau configuration. A CT scan demonstrated the thickened pericardium. Based on these findings, she was diagnosed as constrictive pericarditis and severe mitral regurgitation probably due to worsening mitral annular disjunction caused by the tight pericardium adhesion to the myocardium. During surgery, extensive tissue adhesions were observed from pericardium to epicardium. Histological examination revealed fibro-adipose pericardium with chronic inflammation and myxoid degeneration. She successfully underwent pericardiectomy and mitral valve plasty, and discharged home with greatly improved symptoms.