Primary Idiopathic Chylopericardium as Unusual Cause of Cardiac Tamponade

Abstract

The chylopericardium is defined as the accumulation of chylous fluid containing high concentrations of triglycerides in the pericardial space. It is quite an uncommon condition first described in 1888 by Hasebroek. The absence of an underlying etiology defines it as primary idiopathic chylopericardium, term initially used in a study by Groves and Effler in 1954. Even when most patients are asymptomatic, constrictive pericarditis or cardiac tamponade may be referred. Cardiac tamponade, the most serious complication, represents an entity characterized by fluid accumulation in the pericardial cavity. Claudius Galen from Pergamum (131-201 D. C.) described pericardial effusions in gladiators with stab injuries of the chest and Richard Lowe (1669) described its physiology. It took two hundred years for the term "cardiac tamponade" was coined by german surgeon Edmund Rose. Cardiac tamponade can be presented from asymptomatic setting to life-threatening conditions. By the former reason, the early recognition of this entity has crucial importance. Idiopathic chylopericardium occurs in all age groups and affects both sexes equally. Although several mechanisms have been proposed in order to explain the development of chylopericardium, the underlying pathophysiology remains unclear. Damaged lymphatic vessels with abnormal communication of the thoracic duct to the pericardial lymphatics and elevated pressure in the thoracic duct can justify the presence of chyle into the pericardial sac. Diagnosis is confirmed by pericardiocentesis which must reveal at least two of the classical five criteria. Chylopericardium may be managed through conservative or surgical treatment. The conservative treatment consist a medium chain triglyceride diet or total parenteral nutrition but has a high degree of recurrence, for which reason the safest option is thoracic duct ligation with a pericardial window. The present study reports a case of chylopericardium wherein the leak was diagnosed by lymphoscintigraphy that was successfully treated by a low-fat diet, rich in medium-chain triglycerides and octreotide infusion.