Loeffler endocarditis as a rare cause of heart failure with preserved ejection fraction

Abstract

Rationale:Hypereosinophilic syndrome (HES) is a rare disease characterized by hypereosinophilia and its ensuing organ damage. Cardiac involvement is divided into 3 chronological stages: an acute necrotic stage; a thrombus formation stage; and a fibrotic stage. Infiltration of the myocardium by eosinophilic cells followed by endomyocardial fibrosis is known as “Loeffler endocarditis.”Patient concerns:We report a case of a 60-year-old man diagnosed with left-sided restrictive cardiomyopathy.Diagnosis:The patient experienced heart failure with preserved ejection fraction. The cardiac MRI showed intense, linear, delayed gadolinium enhancement of the endocardium of the lateral wall of the left ventricle, and obliteration of the LV apex. He was ultimately identified as Loeffler endocarditis.Intervention:A bone marrow smear and biopsy revealed the FIP1L1-PDGFRA fusion gene was positive in 82% of segmented nucleated cells.Outcome:Our patient responded well to prednisone at 1 mg/kg/d.Lessons:HES is a rare disease that often afflicts the heart. Cardiac involvement in hypereosinophilia, especially Loeffler endocarditis, carries a poor prognosis and significant mortality. Early detection and treatment of the disease is therefore essential. Further studies are needed to ascertain therapeutic corticosteroid dosages and develop targeted gene therapies, both important steps to ameliorate the effects of Loeffler endocarditis and improve patient outcomes.

Idiopathic Hypereosinophilic Syndrome Presenting With Embolic Stroke

“…Cardiovascular complications occur in 40 to 60% of cases [9] and are the major cause of morbidity and mortality [10]. A classic presentation of eosinophilic infiltration is Loeffler's endocarditis, in which there is thickening and fibrosis of the endocardium and consequent restrictive cardiomyopathy [11]. Cardiac involvement usually occurs in three phases: necrotic (acute, in which there is myocardial infiltration by eosinophils), thrombotic and fibrotic [10].…”

Löeffler endocarditis due to idiopathic hypereosinophilic syndrome

“…Dermatologic involvement followed by pulmonary, gastrointestinal, and cardiac manifestations are the most common clinical implications reported². Cardiac involvement usually follows 3 stages: the fi rst stage, frequently asymptomatic, with acute necrosis, the second stage characterized by mural thrombi formation, and third stage with fi brosis and restrictive cardiomyopathy ensues 4,8 .…”

“…Cardiac magnetic resonance is crucial for LE diagnosis due to detection and characterization of ventricular thrombi and early detection of subendocardial thickening associated with myocardial tissue abnormalities. Due to the focal nature of the disease, the endomyocardial biopsy has a low sensitivity 4 .…”

“…Löeffl er endocarditis is a restrictive cardiomyopathy caused by eosinophilic infi ltration of the heart. It represents the cardiac manifestation of HES and is associated with high mortality and morbidity rates 4 . LE progresses through three stages: acute necrotic stage, thrombotic stage, and fi brotic stage.…”

Loeffler's Endocarditis and the Diagnostic Utility of Multimodality Imaging

“…The second stage is associated with thrombus formation. The final stage is characterized by fibrosis resulting in restrictive cardiomyopathy [2,3]. Patients with eosinophil-associated endocarditis can present with a wide array of symptoms, including dyspnea, chest pain, cough, congestive heart failure, and valvular involvement.…”