Sudden Death Due to Eosinophilic Endomyocardial Diseases

Abstract

Eosinophils are associated with various disorders, such as allergic or hypersensitivity reactions, parasitic diseases, connective tissue diseases, certain neoplastic diseases (Hodgkin's disease, lymphomas, and carcinomas), and various immune deficiency states. Eosinophils can infiltrate any tissue and can cause tissue damage. Heart, has been demonstrated to be the most extensively involved and toxicity of eosinophils is well-established on cardiac tissue. We describe 3 cases with extensive eosinophilic infiltration without endomyocardial fibrosis. All patients died after a short clinical course with rapidly progressive heart failure. Bronchial asthma, hydatid disease and drug reaction were considered as possible etiologies of eosinophilia in case 1 and case 2. Case 3 was considered to fall into the "idiopathic hypereosinophilic syndrome" in which no underlying causes for eosinophilia could be identified.

An autopsy case of necrotizing eosinophilic myocarditis causing left ventricular wall rupture

“…Thus, any cluster of eosinophils in the myocardial interstitium should be considered pathologic and suggest the diagnosis of eosinophilic myocarditis [16]. Macroscopically, the entity is characterized by a flabby heart, irregular tannish areas extending transmurally through the myocardium, areas of diffuse yellow-white discoloration (mottling) and mural thrombi [1,10]. Also aneurysm formation of right ventricular wall in a case of acute myocarditis has been described [17].…”

“…Histological examination revealed marked myocardial damage with massive eosinophilic infiltration. The abrupt onset of fulminant eosinophilic myocarditis in the acute necrotizing phase occurring in the absence of extracardiac disease has been rarely reported [1,4].…”

“…The toxicity of eosinophils toward the myocardium is well established and dominates the patient's prognosis [1][2][3].…”

Eosinophilic myocarditis: two case reports and review of the literature

“…Clinical disorders that may result in both hypereosinophilia and endomyocardial injury include idiopathic hypereosinophilic syndrome, malignancies, parasitic infections, vasculitic and granulomatous diseases, tropical endomyocardial diseases, drug reactions, and transplant rejections [9]. A paramount diagnostic feature of EM on endomyocardial biopsy includes the detection of myocardial eosinophils; these inflammatory cells are rarely detected in normal myocardial interstitium thus their presence is considered pathologic for EM [10]. Regardless of the underlying etiology of EM, whether related to the HES or a parasitic infection, eosinophil-mediated cardiac injury occurs in a similar fashion and ranges from early necrosis to subsequent thrombosis and fibrosis [11].…”

“…Regardless of the underlying etiology of EM, whether related to the HES or a parasitic infection, eosinophil-mediated cardiac injury occurs in a similar fashion and ranges from early necrosis to subsequent thrombosis and fibrosis [11]. On a macroscopic level this type of myocardial injury can translate into variable degrees of focal or diffuse structural abnormalities including systolic or diastolic dysfunction, mural thrombi, micro-abscesses, ventricular wall aneurysms, and rupture [10,12,13]. The sensitivity of endomyocardial biopsy based on autopsy specimens was estimated to be around 54% given the often patchy nature of the disease and this is likely even lower in the beating heart due to the technical difficulties of the biopsy procedure [14].…”