Chylous Pericardium (Chylopericardium)
What is chylous pericardium?
Chylous pericardium is a rare condition in which lymphatic fluid leaks into the space around the heart. When this fluid builds up it can compress the heart and lead to poor heart function.
Chylous pericardium is a lymphatic flow disorder. This group of diseases is characterized by abnormal circulation of lymph fluid.
Lymph vessels carry lymph fluid to veins, where it returns to the bloodstream. The fluid plays a crucial role in immune function and fat and protein transport. When something disrupts or damages normal lymphatic flow, it can cause leakage of lymphatic fluid into the pericardium, chest, abdomen or other body cavities.
Causes of chylous pericardium
Chylous pericardium has many potential causes, including:
- Injuries to the central lymphatic system due to surgery or trauma (this is also known as traumatic chylous pericardium)
- Congenital lymphatic malformations such as lymphangiomatosis
- Systemic diseases or infections such as tuberculosis
- Congenital syndromes such as Gorham-Stout, Noonan and Turner syndromes
- Elevated venous pressures due to heart failure, pulmonary hypertension or congenital heart surgery
Signs and symptoms of chylous pericardium
Initially, chylous pericardium can be asymptomatic if there is only a small volume of fluid around the heart. When enough fluid accumulates, affected individuals will have poor heart function, which can lead to difficulty breathing, chest pain, and a fast heart rate. If this condition is not treated it can become life threatening.
Diagnosis of chylous pericardium
Doctors use a combination of imaging tests to diagnose chylous pericardium. Tests may include:
A diagnosis of chylous pericardium is made when these tests demonstrate the presence of fluid around the heart, and when the fluid is sampled and found to have originated in the lymphatic system.
Additionally, lymphatic imaging modalities, such as intranodal lymphangiography and dynamic contrast magnetic resonance lymphangiography, might be used to determine the exact source of the lymphatic leak.
Treatment for chylous pericardium
Treatment of chylous pericardium depends on its cause. Patients are treated by a team of experts who specialize in lymphatic imaging and interventions through the Jill and Mark Fishman Center for Lymphatic Disorders. The team will determine the best treatment approach for each patient’s condition.
When lymphatic imaging is able demonstrate the source of the leak leading to chylous pericardium it can be sealed using an embolization procedure.
Long-term outlook for chylous pericardium
The long-term outlook for patients with chylous pericardium varies. Many patients with chylous pericardium can be cured. Still, some causes of chylous pericardium are more challenging. Experts at the Lymphatic Center have been developing new imaging and treatment options for these patients.
Most of the follow-up care will be done by your or your child’s primary physician in coordination with our team for special needs related to the procedure or the lymphatic disorder. This can include recommendations for new medications, medication weans or diet recommendations.
Reviewed by Yoav Dori, MD, PhD