Eosinophil-mediated endomyocardial damage is a well-known complication in patients with hypereosinophilic syndromes (HES). Although management and survival have improved significantly, some patients continue to develop severe cardiomyopathy as a direct consequence of uncontrolled hypereosinophilia. Cardiologists play a key role in early detection and treatment. At the early generally asymptomatic stage, related to subendocardial eosinophilic infiltrates, elevation of the biomarker of cardiac damage (serum troponin) and cardiac MRI are the best tools for diagnosis. As disease progresses, patients typically develop intracardiac mural thrombi and may experience variable degrees of heart failure due to valve damage and/or subendocardial fibrosis, all of which are more readily detectable with traditional echocardiographic investigation. New imaging modalities such as strain imaging and specific sequences in MRI offer the perspective of detecting subtle perturbations and distinguishing inflammatory versus fibrotic stages. Endomyocardial biopsy may help in difficult settings, namely, when blood eosinophilia is not prominent, but may be non-contributive due to sampling issues or eosinophil degranulation or replacement by fibrosis, and must always be performed after careful consideration of the risk:benefit ratio. Although treatment of the HES itself should be managed by clinicians with expertise in this rare disorder with the aim of lowering eosinophil counts to prevent and treat eosinophil-mediated organ damage and dysfunction, cardiologists play a key role in managing the associated cardiopathy. There are no consensual disease-specific guidelines for treating eosinophil-mediated thrombotic complications and cardiopathy, which should be managed according to classical international recommendations.
- diagnostic imaging
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Contributors AB, FR and CC contributed equally to the writing and the review of the article.
Funding FNRS (Belgian National Fund for Scientific Research) grant number J.0011.19F (AB) and F 5/4/150/5 (FR). AB is coholder of an Actelion Chair for Research in Pulmonary Hypertension. ULB Fonds Erasme (CC).
Competing interests AB has received consultancy fees from Amicus, Baeyer, Boehringer Ingelheim, Sanofi, Pfizer, Novartis and Alnylam; speaker fees from Pfizer, Sanofi and Alnylam. FR has received consultancy fees from GlaxoSmithKline and AstraZeneca for expertise in hypereosinophilic syndromes and royalties from UpToDate.
Patient and public involvement Patients and/or the public were not involved in the design, conduct, reporting or dissemination plans of this research.
Provenance and peer review Commissioned; externally peer reviewed.