MRI as an Effective Tool for the Diagnosis and Monitoring of Leffler Endocarditis at the Stages of Longitudinal Observation

Abstract

Hypereosinophilic syndrome (HES) is an extremely rare disease that is not always diagnosed, and the lack of statistic data does not let to determine its real incidence.

Among patients men predominate, the ratio of men and women is 9: 1, the most vulnerable age is from 20 to 50 years. The familial hypereosinophilia is inherited disease of autosomal dominant type. Two-year mortality was recorded in half of the cases of Leffler’s endocarditis with progressive fibrosis due to heart failure and thromboembolic complications.

Leffler’s endocarditis and endomyocardial fibrosis as components of restrictive cardiomyopathy are accompanied by eosinophilia. The story of the discovery of eosinophils is closely connected to the name of Paul Ehrlich; the further idea of tracing the connection between eosinophilia and the involvement of the heart and other organs belongs to Leffler.

In the presence of Leffler’s syndrome, the probability of thrombosis in the heart cavities and determination of the stage of the disease were analyzed by longitudinal observation using cardiac MRI.

The described clinical case of Leffler syndrome in a young man in real clinical practice clearly demonstrates the difficulties of diagnosis in the outpatient phase, need in interdisciplinary approach in the work of the team “heart team” during the hospital period, the role and importance of long-term cardiac MRI monitoring of the selected optimal therapy.

Leffler’s syndrome in real clinical practice requires from physicians of various specialties, including family physicians, knowledge of etiology, pathogenesis, clinical masks of disease manifestation and tactics of patient management in the outpatient phase.

MRI of the heart remains the “gold standard” for diagnosis and longitudinal monitoring of patients with Leffler syndrome.