First-pass myocardial perfusion abnormalities in Churg-Strauss syndrome with cardiac involvement

This study sought to evaluate whether representative abnormalities can be identified by first-pass perfusion (FPP) studies in patients with Churg-Strauss Syndrome (CSS), a rare disease characterized by small-vessel vasculitis. Seven patients with CSS (3 men, 4 women; mean age 55 +/- 7 years) were investigated. Echocardiography was performed in all patients and coronary angiography in 5 patients. Magnetic resonance imaging (MRI) was performed with a 1.5 T whole body scanner in all patients. Functional cardiac imaging, T2-weighted turbo inversion recovery magnitude images, and T2-weighted turbo spin echo sequences with fat saturation were also performed. Cardiac viability was assessed by myocardial FPP imaging at rest and by delayed contrast enhancement (DCE) images. The FPP was abnormal in 5 of the 7 CSS patients. The abnormality was localized in the subendocardium of the left ventricle, particularly in areas of myocardial inflammatory edema. All patients showed subendocardial DCE abnormalities in the left ventricle, and 2 patients also in the right ventricle. The localization was typically subendocardial. This study describes a myocardial FPP deficit at rest in patients with CSS. The deficit is localized in areas of inflammatory myocardial edema. This diagnostic technique and image evaluation is simple, quick, and harmless. Due to the rarity of CSS, further studies are necessary to evaluate the impact of these findings.