MRI-based Evidence for Myocardial Involvement in Women with Hypereosinophilic Syndrome

Abstract

Purpose: The aim of the study was to assess the presence and spectrum of cardiac abnormalities identified by cardiac magnetic resonance (CMR) in women with hypereosinophilic syndrome (HES) of undefined etiology, who present with normal electrocardiography (ECG) and transthoracic echocardiography (TTE) and no history of heart disease.Methods: Ten women (mean age, 48 « 14 years) with HES of undefined etiology, normal ECG and TTE, and no history of heart disease underwent CMR.Results: CMR showed cardiac abnormalities in 6 subjects. Five patients had nonischemic late gadolinium enhancement (LGE) lesions within the left ventricular (LV) myocardium, and 3 patients demonstrated CMR evidence of myocardial inflammation. The LV ejection fraction was 68.5 « 5.7%, and the end-diastolic volume index was 62.7 « 14.7 mL/m 2 . The maximum measured blood eosinophil count correlated with LVLGE volume (r = 0.80, P = 0.006) and was 11374 « 6242 cells/µL and 4114 « 2972 cells/µL (P = 0.047) in patients with and without LGE lesions, respectively. The actual blood eosinophil count in subjects with and without CMR evidence of myocarditis was 1058 « 520 cells/µL and 354 « 377 cells/µL (P = 0.04), respectively.Conclusions: Despite normal ECG, TTE, and absence of history of heart disease, women with HES of unknown etiology frequently demonstrate cardiac abnormalities on CMR, the presence and extent of which are related to blood eosinophil count.

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“…Cardiac MRI has higher sensitivity and specificity than any other imaging modalities and contrast-enhanced Cardiac MRI can identify inflammation and fibrosis in its early stages even when echocardiography findings may be normal [14][15][16][17]. Interestingly, the severity of eosinophilia was directly related with the severity of MRI findings in few case reports [17]. In our patient, Cardiac MRI showed circumferential apical endocardial thickening/ fibrosis after gadolinium contrast administration and LV apical thrombus, which is pathognomonic of EHD.…”

“…Myocardial fibrosis can result in restrictive cardiomyopathy in later stages which can be assessed with Doppler data. Cardiac MRI has higher sensitivity and specificity than any other imaging modalities and contrast-enhanced Cardiac MRI can identify inflammation and fibrosis in its early stages even when echocardiography findings may be normal [14][15][16][17]. Interestingly, the severity of eosinophilia was directly related with the severity of MRI findings in few case reports [17].…”

Intracardiac Thrombus in Leukemia: Role of Cardiac Magnetic Resonance Imaging in Eosinophilic Myocarditis

“… 10 found the extent of the lesion identified on CMR to be inversely proportional to the left ventricular ejection fraction, with another study by Miszalski-Jamka et al. 5 finding the maximum measured blood eosinophil count to correlate with the extent of myocardial damage seen on endomyocardial biopsy (EMB). Although EMB is the gold standard and may provide definitive diagnosis, this is typically reserved for those patients in whom there is uncertainty of eosinophilic-induced cardiac disease.…”

“…Women are less frequently affected than men and in turn have better outcomes. 5 The spectrum of presentation ranges from chest pain, dyspnea, and palpitations to cardiogenic shock, with the most common presentation being dyspnea and palpitations occurring in only 8% of patients. 6 , 7 Elevated troponin concentrations are evidence of eosinophil-mediated cardiac cell damage that involves three stages: an acute necrotic stage, an intermediate stage involving damaged endocardium and thrombus formation, and finally a fibrotic stage with altered cardiac function.…”

“…CMR is more sensitive and specific than either transthoracic or transesophageal echocardiography for detection of ventricular thrombi. 5 , 7 T 2 -weighted sequences detect myocardial inflammation during the acute phase noted by myocardial hyperintensity, while postgadolinium inversion recovery delayed enhancement sequences can detect thrombotic and fibrotic stages. 10 Cine CMR can detect the functional impact of the thrombus and eosinophilic infiltrate, while late enhancement detects myocardial fibrosis and inflammation.…”