Clinical vignette
‘Hissing snake’ left ventricle thrombotic phase of hypereosinophilic endomyocardial disease
Maurizio Pieroni, Cristina Chimenti, and Andrea Frustaci*
Cardiology Department, Catholic University, Largo A. Gemelli 8, 00168 Rome, Italy
*Corresponding author. E-mail address: biocard@rm.unicatt.it
A 33-year-old girl with chronic allergic state and history of a recent travel in South-East Asiatic countries was admitted because of acute heart failure with pulmonary oedema. ECG revealed sinus tachycardia with diffuse T-wave abnormalities, whereas two-dimensional echocardiography in apical four-chamber view showed thickening of the mid-apical portion of left ventricular (LV) walls with almost complete apex obliteration and a restrictive LV filling pattern. Laboratory tests showed remarkable hypereosinophilia with cationic protein elevation. Cardiac catheterization revealed a marked increase in LV end-diastolic pressure and angiography showed obliteration of the LV apex by a mass centrally infiltrated by contrast medium (Panel A and Movie) resembling the shape of a snake head with forked tongue (Panel B). Multiple LV endomyocardial biopsies showed the presence of extensive eosinophil-rich endocardial thrombi (Panel C) with inner areas of organization (arrows), suggesting the thrombotic phase of an eosinophilic endomyocardial disease. Combination of steroids and anticoagulant therapy provided a rapid and significant improvement of clinical and echocardiographic picture. Hypereosinophilia, whatever its origin, causes a severe endomyocardial damage with endocardial thrombotic apposition when eosinophil degranulation with release of the cationic protein and factor X activation occurs.
End-diastolic frame from LV angiography (30° right anterior oblique view) (Panel A) showing significant obliteration of ventricular apex by a mass centrally infiltrated by contrast medium, resembling the shape of a snake head with forked tongue (Panel B). LV endomyocardial biopsy (Panel C) showed an extensive eosinophil-rich thrombus with inner areas of organization (arrows). (Masson's trichrome, 40X).
Movie. LV angiography (30° right anterior oblique view) showing massive obliteration of ventricular apex by a huge mass centrally infiltrated by contrast medium, resembling the shape of a hissing snake with a forked tongue. For the movie, see online supplementary material available at European Heart Journal online.
[Table of Contents]