Endomyocardial Fibrosis and Intracardiac Thrombus Occurring in Idiopathic Hypereosinophilic Syndrome

Abstract

Case Report A 59-year-old woman was examined to investigate weakness, fatigue, and abdominal pain. Clinical examination revealed vitiligo, raised jugular venous pressure, bilateral pleural effusion, splenomegaly, and mild peripheral edema. Laboratory studies showed an absolute eosinophilia level of 10%, thrombocytopenia, and abnormally elevated liver enzyme levels. A triphasic abdominal CT scan (not shown) revealed a nonenhancing thrombus in both ventricular cavities, a small pericardial effusion, and bilateral pleural effusion. Cardiac MRI was requested to exclude the presence of a pericardial mass suggested by the findings of transthoracic echocardiography performed on the same day as the CT. ECG-gated cardiac MRI was performed on a 1.5-T scanner (Sonata, Siemens). Multiple sequences were obtained, including singleshot and retrospective gated balanced gradient-echo (true fast imaging with steady-state precession [FISP]) cine; fat-suppressed HASTE; unenhanced and enhanced T1weighted gradient-echo; and delayed, enhanced viability (inversion recovery prepared turbo fast low-angle shot) obtained after a 10min delay following IV administration of double-dose gadolinium (0.2 mmol/kg). No pericardial mass was identified. The endocardium of both ventricles was more intense than the deeper myocardium on true FISP (Fig. 1A) and HASTE (Fig. 1B) sequences and showed intense linear hyperenhancement on the delayed viability sequences (Fig. 1C). Extensive nonenhancing thrombus was present in both ventricles (Fig. 1D). Both right and left ventricles showed qualitatively normal function on cine sequences, with no regional or global wall motion abnormalities. Large bilateral pleural effusions and a small pericardial effusion were present. After cardiac MRI, coronary angiography was performed and found no significant coronary artery disease in any vessel. Ventriculography revealed apical obliteration of both ventricles. A right ventricular biopsy was also performed; however, pathologic examination of the obtained specimens revealed mainly thrombus with some necrotic tissue. Despite the biopsy results, a diagnosis of endomyocardial fibrosis secondary to IHES was made on the basis of the imaging, clinical, and laboratory findings. The MRI findings of increased signal intensity and contrast enhancement not corresponding to a vascular territory involving the endocardium of both ventricles, together with the unremarkable coronary angiography findings, excluded prior infarction from coexistent ischemic heart disease. Because Loeffler’s syndrome more commonly affects the left ventricle in isolation, this syndrome was considered less likely than IHES. Unfortunately, despite corticosteroid and hydroxyurea therapy, the patient’s condition continued to decline and she died from septicemia 4 weeks after initial presentation.