Phenotypic Spectrum of Cardiac Involvement in Eosinophilic Granulomatosis With Polyangiitis

Cardiac involvement in eosinophilic granulomatosis with polyangiitis (EGPA) is a major determinant of morbidity and mortality and may occur independently of systemic disease activity.

We describe 3 patients with EGPA demonstrating distinct cardiac phenotypes. Patient presentation included: 1) inflammatory cardiomyopathy with severe biventricular dysfunction that improved following escalation of immunosuppression and guideline-directed medical therapy; 2) advanced nonischemic cardiomyopathy and cardiogenic shock requiring temporary and durable mechanical circulatory support, representing an irreversible fibrotic phenotype; and 3) relapsing-remitting eosinophilic perimyocarditis and recurrent ventricular dysfunction despite multiple immunosuppressive regimens. Cardiac magnetic resonance imaging and hemodynamic assessment were central to phenotypic classification and management.

EGPA cardiomyopathy exists along a spectrum from reversible inflammatory myocarditis to advanced fibrotic remodeling. Cardiac involvement may progress despite improvement in systemic manifestations and is not reliably predicted by antineutrophil cytoplasmic antibody status.

Phenotype-based assessment and longitudinal cardiac surveillance may guide immunosuppressive strategies and prognosis in EGPA cardiomyopathy.