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Abstract
Objective Chylopericardium (CPE) is a rare condition associated with accumulation of triglyceride-rich chylous fluid in the pericardial cavity. Due to minimal information on CPE within the literature, we conducted a systematic review of all published CPE cases to understand its clinical characteristics, management and outcomes.
Methods We performed a literature search and identified cases of patients with CPE from 1946 until May 2021 in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. We identified relevant articles for pooled analyses of clinical, diagnostic and outcome data.
Results A total of 95 articles with 98 patients were identified. Patient demographics demonstrated male predominance (55%), with a mean age of 37±15 years. Time from symptom onset to diagnosis was 5 (Q1 4.5, Q3 14) days, with 74% of patients symptomatic on presentation. Idiopathic CPE (60%) was the most common aetiology. Cardiac tamponade secondary to CPE was seen in 38% of cases. Pericardial fluid analysis was required in 94% of cases. Lymphangiography identified the leakage site in 59% of patients. Medical therapy (total parenteral nutrition, medium-chain triglycerides or octreotide) was undertaken in 63% of cases. In our cohort, 32% progressed towards surgical intervention. During a median follow-up of 180 (Q1 180, Q3 377) days, CPE recurred in 16% of cases. Of the patients with recurrence, 10% were rehospitalised.
Conclusion CPE tends to develop in younger patients and may cause serious complications. Many patients fail medical therapy, thereby requiring surgical intervention. Although overall mortality is low, associated morbidities warrant close follow-up and possible reintervention and hospitalisations.
- echocardiography
- Magnetic Resonance Imaging
- pericarditis
- cardiac tamponade
- pericardial effusion
Data availability statement
Data are available upon reasonable request.
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Data availability statement
Data are available upon reasonable request.
Footnotes
BV and AK are joint first authors.
Twitter @AnkitAgrawalMD
BV and AK contributed equally.
Contributors BV, AK: conceptualisation, methodology, formal analysis, validation, writing—original draft, writing—review and editing. NV, AA: conceptualisation, methodology, formal analysis, writing—original draft, writing—review and editing. AY, MMF, GM: conceptualisation, methodology, writing—original draft, writing—review and editing. FB, SM: methodology, validation, writing—original draft, writing—review and editing. MS: methodology, formal analysis, validation. PR-G: validation, writing—original draft, writing—review and editing. MN, AJ: conceptualisation, validation, writing—original draft. TKMW: formal analysis, validation, writing—review and editing. ALK: guarantor, writing—original draft, writing—review and editing, supervision, project administration.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests ALK: research grant and scientific advisory board for Kiniksa Pharmaceuticals, research grant and scientific advisory board for Cardiol Therapeutics, and scientific advisory board for Pfizer.
Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.
Provenance and peer review Not commissioned; externally peer reviewed.
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