Endomyocardial fibrosis in Churg–Strauss syndrome assessed by cardiac magnetic resonance imaging

Churg–Strauss Syndrome

“…Infrequently reported cardiac involvement includes impaired myocardial perfusion leading to ischemic cardiomyopathy, cardiac wall motion anomalies, constrictive pericarditis, endomyocardial fibrosis, eosinophilic coronary arteritis, coronary dissection, valvular regurgitation, conduction disturbances and arrhythmias, and isolated ventricular septum involvement. Pulmonary hypertension is rare (Hunsaker et al 1992;Davies 2005;Alter and Maisch 2006;Pela et al 2006;Lhote et al 2008;Wassmuth et al 2008;Mavrogeni et al 2009;Rosato et al 2009;Dennert et al 2010;Sauvetre et al 2010). …”

“…It is mainly observed in the apical territory and mid-cavity left ventricular side wall but does not relate to a major coronary artery distribution. Impaired myocardial perfusion is related to inflammation and fibrotic changes in small vessels and the myocardium which may be seen even in patients with preserved systolic function as measured by current imaging methods (Alter and Maisch 2006;Vignaux et al 2007;Wassmuth et al 2008;Mavrogeni et al 2009;Neumann et al 2009;Sauvetre et al 2010). Fibrotic changes are predominantly subendocardial, whereas nonischemic cardiomyopathies or viral myocarditis is often found in the middle or in the subepicardial locations of the myocardial wall (Wassmuth et al 2008).…”

“…Fibrotic changes are predominantly subendocardial, whereas nonischemic cardiomyopathies or viral myocarditis is often found in the middle or in the subepicardial locations of the myocardial wall (Wassmuth et al 2008). Subendocardial fibrosis following myocardial infarction is similar but is regionally limited and associated with local wall motion disturbances (Alter and Maisch 2006). CMRI can detect transient markers of myocardial inflammation such as edema (increased T2 myocardial signal), hyperemia, and capillary leakage, which is demonstrated by early contrast enhancement (Wassmuth et al 2008;Mavrogeni et al 2009).…”

Loeffler's Endocarditis due to Idiopathic Hypereosinophilic Syndrome

“…In addition, the intensive inflammatory changes result in superimposed thrombus formation, which subsequently occupies much of the ventricular cavity. [7][8][9] The presence of asymptomatic endomyocardial lesions may precede overt cardiac disease by a substantial period of time.…”

Fatal Loeffler's endocarditis due to hypereosinophilic syndrome

“…Any organ is vulnerable to eosinophilia-associated tissue damage, although the major tissue targets are the heart [3], the nervous system, the skin, and the upper and lower respiratory tract including the lung parenchyma [2]. Loeffler's endocarditis, the classical endocardial manifestation of HES, is characterized by fibrous thickening of the endocardium, leading to apical obliteration and restrictive cardiomyopathy, resulting in heart failure, thromboembolic events, or atrial fibrillation [4,5]. HES is a potentially fatal disease with a less than 50% reported 10-year survival, especially in corticosteroid-resistant cases with cardiac involvement [2,6].…”