Esophageal-Pericardial Fistula: A Systematic Review of Etiology, Diagnostic Features, Management, and Outcomes

Esophageal-pericardial fistula (EPF) is rare with an incidence of 0.016%-0.04%. With novel procedures involving intracardiac or esophageal instrumentation in the last few decades, including atrial fibrillation (AF) ablation, further understanding of this unique entity is warranted. In this systematic review, we summarize the etiology, diagnostic features, management, and outcomes of EPF.

A librarian conducted a systematic literature review of case reports/series describing adults diagnosed with EPF through 7 major databases/registries from inception to 7/30/2024.

One hundred twenty patients from 115 papers met the inclusion criteria. Median age(IQR) was 57(44-67) years, with a male predominance (73.7%). The primary etiology of EPF shifted from benign esophageal diseases before 2000 to AF ablation on/after 2000. Median time(IQR) from AF ablation to EPF presentation/diagnosis was 17(13-23) days. Chest pain (70.8%) and pneumopericardium (90.0%) were, respectively, the most common presenting symptom and pericardial finding. Water-soluble contrast esophagram (WSCE) had the highest diagnostic sensitivity (82.8%). Management included surgery (47.5%), stent placement (22.5%), combined surgery/stenting (19.2%), and conservative (10.8%). Overall mortality was 37.5%; median(IQR) time to death was 20(9-41) days. EPF from malignancy had poorer survival than those from benign esophageal causes (HR 2.65 [1.36-5.17]; p = 0.004) and cardiac ablation (HR 5.74 [2.41-13.65]; p < 0.0001). Patients who received any intervention (HR 0.28 [0.13-0.57]; p = 0.0005) or had confirmed EPF resolution (HR 0.27 [0.12-0.59]; p = 0.0009) demonstrated better survival. There was no difference in survival between surgery and stenting alone (p = 0.91).

AF ablation is now the leading etiology of EPF. Given its high mortality, a high degree of clinical suspicion is essential in those presenting with key signs/symptoms following AF ablation to enable urgent diagnosis and multi-disciplinary management. WSCE may be the diagnostic modality to perform, given its high sensitivity. Future studies are warranted to investigate whether stenting should be the primary management given possible non-inferiority to surgery.