Clinical Images: Löffler endocarditis: a rare cardiac manifestation of eosinophilic granulomatosis with polyangiitis

Cardiac manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) involve vascular injury as well as disease from hypereosinophilia.1 Löffler endocarditis can occur in EGPA when eosinophils infiltrate the endomyocardium, leading to cardiac damage, mural thrombosis, and endocardial fibrosis.2-5 Here, we present such a case. The patient, a 49-year-old woman with a history of bronchial asthma, hypothyroidism, and osteoarthritis, presented with dyspnea secondary to new-onset heart failure. She had elevated levels of troponin, N-terminal pro–brain natriuretic peptide, eosinophils (2,000 cells/μL), and C-reactive protein, as well as a positive anti-myeloperoxidase antibody level of 2.4 antibody index (AI) (normal <1.0 AI). Additional workup for hypereosinophilic syndrome was negative, including the parasite screen, IgE levels (149 IU/mL, normal 6–495 IU/mL), platelet-derived growth factor receptor gene rearrangements, and T cell receptor gene rearrangements. An echocardiogram revealed an akinetic cardiac apex with an extensive burden of the laminated thrombus within the apex. Left ventricular ejection fraction was 42%. Cardiac magnetic resonance imaging before and after contrast with (A) two-chamber and (B) four-chamber white blood cell images demonstrated obliteration of the left ventricular apex by a large mural filling defect. (C) Short axis and (D) four-chamber T2 fat suppression images showed no evidence of myocardial edema or myocarditis. (E) Basal and (F) mid short axis T2 mapping images also confirmed no evidence of myocardial edema or myocarditis. (G) Four-chamber and (H) two-chamber late gadolinium enhancement images after contrast administration showed the V sign of endomyocardial fibrosis at the apex, characterized by a three-layered appearance of the dark nonenhancing overlying thrombus (asterisk), the bright fibrotic endomyocardium (arrows), and the dark underlying myocardium, consistent with Löffler endocarditis. Endomyocardial biopsy findings were nondiagnostic. The patient was treated with anticoagulation, high-dose glucocorticoids, and cyclophosphamide for cardiac EGPA with Löffler endocarditis and has had recovery of her ejection fraction. Disclosure form. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.