Löeffler endocarditis due to idiopathic hypereosinophilic syndrome

Laura Benchea,Alexandra Clement,Alina-Elena Nedelcu,Cristian Statescu

doi:10.47803/rjc.2021.31.1.97

Abstract

Abstract Hypereosinophilic syndrome encompasses a heterogenous group of non-hematologic and hematologic disorders defined as peripheral blood eosinophilia >1500/mm3 persisting more than 6 months and eosinophilic end organ complications1,2. Löeffler endocarditis (LE) is the most common cardiac manifestation of the hypereosinophilic syndrome (HES) and represents an acute form of primary restrictive cardiomyopathy3. We report the case of a 74 years-old woman with symptoms related to congestive heart failure and weight loss. At admission the patient had tachycardia and a grade 2/6 systolic mitral murmur. Laboratory findings revealed eosinophilia, hepatocytolysis syndrome and dyslipidemia. The electrocardiogram (ECG) showed non-specific ST-segment and T wave abnormalities. The echocardiography revealed left ventricular apical thrombus and entrapment of chordae tendineae with restricted motion of mitral leaflets leading to mitral regurgitation. The diagnosis of myocarditis was confirmed by the cardiac magnetic resonance imaging which showed the presence of a left ventricular mass with low signal on steady-state free precession imaging and diffuse circumferential subendocardial late gadolinium enhancement (LGE). When discussing the etiology of the HES the following were taken into consideration: hematologic, reactive or secondary disorders. This case is distinguished by diagnosis in an elderly woman and good response to corticosteroid therapy.

Highlights

Löeffler endocarditis is a restrictive cardiomyopathy caused by eosinophilic infiltration of the heart
We report the case of a 74-year-old patient, without family medical history, who addressed to emergency care unit for resting dyspnea, orthopnea, paroxysmal nocturnal dyspnea, atypical chest pain, weight loss, and skin lesion, since two weeks
Cardiac involvement usually follows 3 stages: the first stage, frequently asymptomatic, with acute necrosis, the second stage characterized by mural thrombi formation, and third stage with fibrosis and restrictive cardiomyopathy ensues[4,8]

Summary

INTRODUCTION

Löeffler endocarditis is a restrictive cardiomyopathy caused by eosinophilic infiltration of the heart. Transthoracic echocardiography revealed nondilated cardiac chambers, good left and right ventricular global systolic function Clinical impression and differential diagnosis Given the patient’s presentation and prior investigations, the few top diagnoses included apical thrombus, apical hypertrophic cardiomyopathy, left ventricular non-compaction cardiomyopathy, or Löeffler endocarditis. The final diagnosis was Idiopathic Hypereosinophilic syndrome with Löeffler endocarditis Management of this patient included gradual tapering of methylprednisolone guided by echocardiogram and biological work up, acenocumarol and heart failure treatment according to current guidelines[6] with Furosemide 40 mg od, Spironolactone 25 mg od, Candesartan 16 mg od, and Bisoprolol 2.5 mg od. At seven-months follow-up, she was asymptomatic with no skin lesions, normal hemogram (Eo=360/ mm3) and resolution of left ventricular thrombus, but persisting mitral valve regurgitation and restrictive pattern diastolic dysfunction (Figure 5)

DISCUSSION

CONCLUSIONS