
Loeffler endocarditis as a rare cause of heart failure with preserved ejection fraction
Author(s) -
Ming Gao,
Weihua Zhang,
Waiou Zhao,
Qin Ling,
Fei Pei,
Yang Zheng
Publication year - 2018
Publication title -
medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.59
H-Index - 148
eISSN - 1536-5964
pISSN - 0025-7974
DOI - 10.1097/md.0000000000010079
Subject(s) - medicine , hypereosinophilia , hypereosinophilic syndrome , endocarditis , ejection fraction , restrictive cardiomyopathy , cardiology , endomyocardial fibrosis , endocardium , heart failure , prednisone , eosinophilia , cardiomyopathy , ventricle , fibrosis
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.