Chylopericardium (CPE) is associated with significant morbidity, and one-third of patients require surgical management after medical management fails, according to a study in Heart.
Researchers conducted a systematic review to assess clinical characteristics and outcomes in patients with CPE, which is associated with accumulation of triglyceride-rich fluid in the pericardial space.
A search for published articles on chylous pericardial effusion was performed in multiple databases on December 2, 2020, with an updated search on May 27, 2021. The review was performed with use of the Patient, Intervention, Comparison, Outcome (PICO) framework.
A total of 95 case reports with 98 patients were included. Participants had a mean age of 37±15 years and 55% were men. CPE was the first occurrence in 97% of participants, and the median time to symptom onset from diagnosis was 5 days. Most of the cases (30%) were reported in the United States. Common comorbidities included malignancy (12%), valvular surgery (11%), and coronary artery bypass grafting (7%).
Idiopathic/viral was the predominant etiology (60%), followed by postcardiac injury syndrome (23%), and malignancy (11%). The most common initial presenting symptom was dyspnea (38%), and cardiac tamponade (38%).
Of the cohort, 51 patients had a transthoracic echocardiogram, which showed pericardial effusion as small (6%), moderate (14%), or large (80%). Pericardial effusion was the predominant computed tomography chest finding (45%). Cardiac magnetic resonance imaging was conducted in 10% of patients, with pericardial effusion (70%) and pleural effusion (20%) the most frequent findings. Lymphangiography was needed in 59% of participants and identified the thoracic duct as the predominant leakage site (70%).
Conservative medical management was used in 63% of patients and included total parenteral nutrition, medium-chain triglyceride diet, or octreotide for a median of 10 days. Concomitant medical and surgical intervention was performed in 16% of cases. Progression to surgical management after medical management failure occurred in 34% of cases.
The mean follow-up was 180 days and follow-up data were available in 72% of cases. A total of 6% patients died secondary to underlying comorbidities, and rehospitalization for recurrent dyspnea or re-accumulation of effusion was reported in 10% of patients. CPE recurred in 16% of patients and 6% needed repeat pericardiocentesis or pericardial window.
The researchers noted that their sample size limited the power to perform multivariable analyses, and the data were pooled from case reports, which limits their generalizability. In addition, case reports from the centers could vary in quality and amount of data presented, and under-reporting could affect the outcomes. Also, the study authors were unable to compare the cases with a control group.
“CPE management is complex and requires a multidisciplinary team,” wrote the researchers. “A subset of patients may fail medical therapy, thereby necessitating surgical intervention. While CPE mortality is low, morbidity associated with the disease is high. Therefore, sufficient consideration should be taken during diagnosis and management of patients.”
Disclosure: One of the study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors’ disclosures.
Verma B, Kumar A, Verma N, et al. Clinical characteristics, evaluation and outcomes of chylopericardium: a systematic review. Heart. Published online January 26, 2023. doi: 10.1136/heartjnl-2022-321798