EGPA presenting as sudden cardiac arrest: a case report and review of cardiac manifestations

TL;DR

A rare case of EGPA causing sudden cardiac arrest is reported, highlighting the importance of early diagnosis and treatment.

Contribution

This is the first documented case of EGPA initially presenting as Adams-Stokes syndrome.

Findings

A 27-year-old female with EGPA presented with Adams-Stokes syndrome and significant eosinophilia.

Emergency treatment with methylprednisolone and mepolizumab restored sinus rhythm and improved cardiac function.

The case emphasizes the need to consider EGPA in unexplained high-grade heart block.

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis in which cardiac involvement is a primary cause of mortality. Complete heart block presenting as Adams-Stokes syndrome is a rare but critical complication. Notably, to our knowledge, EGPA initially manifesting as Adams-Stokes syndrome has not been previously documented, based on a comprehensive review of the literature. We report a 27-year-old female presenting with recurrent syncope and seizures. Laboratory tests revealed significant eosinophilia (49.5%), and cardiac workup confirmed third-degree atrioventricular block. A diagnosis of EGPA was established based on the 2022 ACR/EULAR criteria (score=13). Emergency treatment involved temporary pacing and methylprednisolone pulse therapy, followed by mepolizumab induction. Sinus rhythm recovered within 24 hours. During a two-month follow-up, the patient maintained remission with normalized eosinophil counts and improved cardiac function. This case highlights the importance of including EGPA in the differential diagnosis of unexplained high-grade heart block and supports the efficacy of early immunosuppressive therapy in reversing life-threatening cardiac complications.

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