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Background Endomyocardial fibrosis (EMF) is a rare and underdiagnosed cause of restrictive cardiomyopathy. Its aetiology is not yet defined and could be caused by the influence of different clinical factors that seem to combine with genetic aspects of individuals susceptible to an inflammatory process that leads to formation of fibrosis. Case summary We describe a case of a 50-year-old man from the northeastern region of Brazil, where there is high prevalence of schistosomiasis. He presented to our centre with symptoms of right heart failure. The echocardiogram showed normal left ventricular ejection fraction. Right ventricular had normal systolic function but in the apical region was filled with a homogeneous and hypoechoic image causing obliteration and restriction of the apex. The late gadolinium enhancement with cardiac magnetic resonance showed diffuse and heterogeneous subendocardial fibrosis in the right ventricle apex consistent with EMF, but declined endocardiectomy. Discussion This report presents an interesting case of EMF and schistosomiasis simultaneously. The hypothesis of parasitosis as a probable cause of EMF was raised by helminth-induced hypereosinophilia. Complementary imaging tests such as magnetic resonance imaging and echocardiography, in addition to clinical and epidemiological suspicion, are essential for its diagnosis. Early surgical resolution becomes crucial for long-term survival. [ABSTRACT FROM AUTHOR]

Hypereosinophilia (HE) is defined as an eosinophil count exceeding 1500 cells/microL in peripheral blood in two tests, performed with an interval of at least one month and/or anatomopathological confirmation of HE, with eosinophils comprising more than 20% of all nucleated cells in the bone marrow. Hypereosinophilic syndrome (HES) indicates the presence of HE with organ involvement due to eosinophil action, which can be classified as primary (or neoplastic), secondary (or reactive), and idiopathic. Cardiac involvement occurs in up to 5% of cases in the acute phase and 20% of the chronic phase of the disease, ranging from oligosymptomatic cases to fulminant acute myocarditis or chronic restrictive cardiomyopathy (Loeffler endomyocarditis). However, the degree of cardiac dysfunction does not directly correlate with the degree of eosinophilia. The cardiac involvement of HES occurs in three phases: initial necrotic, thrombotic, and finally necrotic. It can manifest as heart failure, arrhythmias, and thromboembolic phenomena. The diagnosis of cardiopathy is based on multimodality imaging, with an emphasis on the importance of echocardiography (echo) as the primary examination. TTE with enhanced ultrasound agents can be used for better visualization, allowing greater accuracy in assessing ventricular apex, and myocardial deformation indices, such as longitudinal strain, may be reduced, especially in the ventricular apex (reverse apical sparing). Cardiac magnetic resonance imaging allows the characterization of subendocardial late gadolinium enhancement, and endomyocardial biopsy is considered the gold standard in diagnosing cardiopathy. Treatment is based on the etiology of HES.

Central Illustration : Position Statement on the Use of Myocardial Strain in Cardiology Routines by the Brazilian Society of Cardiology's Department Of Cardiovascular Imaging - 2023 Proposal for including strain in the integrated diastolic function assessment algorithm, adapted from Nagueh et al.67 Am: mitral A-wave duration; Ap: reverse pulmonary A-wave duration; DD: diastolic dysfunction; LA: left atrium; LASr: LA strain reserve; LVGLS: left ventricular global longitudinal strain; TI: tricuspid insufficiency. Confirm concentric remodeling with LVGLS. In LVEF, mitral E wave deceleration time < 160 ms and pulmonary S-wave < D-wave are also parameters of increased filling pressure. This algorithm does not apply to patients with atrial fibrillation (AF), mitral annulus calcification, > mild mitral valve disease, left bundle branch block, paced rhythm, prosthetic valves, or severe primary pulmonary hypertension.