Cor Vasa 2022, 64(5):542-545 | DOI: 10.33678/cor.2022.011

(Cardiac involvement in a patient with idiopathic hypereosinophilic syndrome)

Benjamin Uwamahoro^a, František Toušek^b, Štěpánka Šindelářová^b, Aleš Mokráček^c, Pavla Vítková^d, Jiří Kubále^e

^a Interní oddělení, Nemocnice Tábor, a.s., Tábor

^b Kardiologické oddělení, Kardiocentrum, Nemocnice České Budějovice, a. s., České Budějovice

^c Kardiochirurgické oddělení, Kardiocentrum, Nemocnice České Budějovice, a. s., České Budějovice

^d Patologické oddělení, Nemocnice České Budějovice, a. s., České Budějovice

^e Radiologické oddělení, Nemocnice České Budějovice, a. s., České Budějovice

Hypereosinophilic syndrome (HES) is a rare disorder defined by a marked and persistent hypereosinophilia (HE) (˃1,5 × 10⁹/l), evidence of eosinophil-mediated organ damage, that is not the result of secondary (reactive) HE. HES is a part of a heterogeneous group of hypereosinophilic disorders. Cardiovascular involvement is present in 40-50% of patients with HES and is a major source of morbidity and mortality. Among its manifestations there are heart failure, intracardiac thrombosis, myocarditis, arrhythmias, and valve diseases. Fatal cardiogenic shock may be the first manifestation of the disease. We present a case report of a patient who presented with severe mitral regurgitation. It was after mitral valve valvuloplasty, permanent pacemaker implantation for complete atrioventricular block, that idiopathic HES was diagnosed and treated with corticosteroids. Furthermore, dilated cardiomyopathy developed, and an upgrade to cardiac resynchronisation therapy was performed. Timely differential diagnosis and targeted treatment are of the utmost importance whenever HE is detected in both asymptomatic patients and those undergoing or after treatment of organ complications.

Keywords: Hypereosinophilia, Hypereosinophilic disorders, Hypereosinophilic syndrome

Received: January 3, 2022; Revised: January 3, 2022; Accepted: February 19, 2022; Published: November 1, 2022  Show citation