Segmental Hypokinetic Cardiomyopathy in Churg Strauss Syndrome: A Case Report and Review of a Spectacular Evolution

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) is a necrotizing systemic vasculitis of small vessels, associated with late-onset asthma, blood and tissue hypereosinophilia. Cardiac involvement is a major concern and has been described as the leading cause of mortality. In this context, we report the case of a 48-year-old woman with Churg Strauss syndrome, suffering from late-onset asthma, pansinusitis, hypereosinophilia, and cardiac damage, consisting of segmental hypokinetic cardiomyopathy with an altered ejection fraction and healthy coronary artery, complicated by a flutter treated by electric cardioversion. The combination of bolus corticosteroid therapy with Azathioprine, as well as the reduction of the rhythm disorder, led to an improvement of the contractile function and the ejection fraction in this patient's case. This observation illustrates a particular and exceptional form of myocardial damage due to Churg-Strauss syndrome and its spectacular evolution under background treatment and rhythm control.