Objective. Churg-Strauss syndrome (CSS) is a rare form of systemic vasculitis. Previous studies showing cardiac involvement in CSS patients were limited in the number of patients and were often based solely on clinical manifestations. The aim of the present study was to determine in detail the incidence of cardiac involvement in a large population of ambulatory CSS patients.Methods. Thirty-two consecutive patients with CSS in remission (mean ؎ SD duration of disease between diagnosis and enrollment 6.1 ؎ 5.8 years, mean ؎ SD age 61 ؎ 10 years) who were previously unaware of cardiac involvement were compared with 32 randomly selected age-and sex-matched control subjects, using clinical evaluation, electrocardiography (EKG), echocardiography, and cardiac magnetic resonance imaging (MRI).Results. Detailed cardiac evaluation revealed a 62% prevalence of cardiac involvement in CSS patients compared with 3% in controls (P < 0.001), with clinical symptoms in 26% and 3%, respectively (P ‫؍‬ 0.009), EKG abnormalities in 66% and 3%, respectively (P < 0.001), and echocardiographic defects in 50% and 3%, respectively (P < 0.001). Cardiac MRI detected cardiac manifestations in 62% of CSS patients. In the presence of cardiac MRI abnormalities, echocardiography could detect cardiac involvement with a sensitivity of 83% and a specificity of 80%. The absence of symptoms or EKG abnormalities did not exclude cardiac involvement, because abnormalities could still be detected in 38% of these patients at the time of echocardiography or cardiac MRI.Conclusion. These results demonstrate a high incidence of cardiac involvement in CSS patients. Systematic cardiac evaluation including detailed imaging is required to properly identify CSS patients with cardiac involvement.

The present pilot study demonstrates that IVIg significantly reduces viral load and improves cardiac function in patients with DCM related to increased PVB19 viral load in the heart.

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This study aimed to investigate whether pulmonary shunt grade on transthoracic contrast echocardiography (TTCE) predicts the size of pulmonary arteriovenous malformations (PAVMs) on chest computed tomography (CT) and subsequent feasibility for transcatheter embolotherapy.We prospectively included 772 persons with possible or definite hereditary haemorrhagic telangiectasia, who underwent both TTCE and chest CT for screening of PAVMs. A quantitative three-point grading scale was used to classify the pulmonary shunt size on TTCE (grade 1-3). Transcatheter embolotherapy was performed for PAVMs deemed large enough for endovascular closure on chest CT.TTCE documented pulmonary shunting in 510 (66.1%) patients. The positive predictive value of a pulmonary shunt grade 1, 2 and 3 on TTCE for presence of PAVMs on chest CT was 13.4%, 45.3% and 92.5%, respectively (p,0.001). None of the 201 persons with a pulmonary shunt grade 1 on TTCE had PAVMs on chest CT large enough for transcatheter embolotherapy, while 38 (25.3%) and 123 (77.4%) individuals with a pulmonary shunt grade 2 and 3 on TTCE, respectively, underwent endovascular closure of PAVMs.Pulmonary shunt grade on TTCE predicts the size of PAVMs on chest CT and their feasibility for subsequent transcatheter embolotherapy. Chest CT can be safely withheld from all persons with a pulmonary shunt grade 1 on TTCE, as any PAVM found in these subjects will be too small for transcatheter embolotherapy. @ERSpublications Chest CT can be safely withheld from persons with pulmonary shunt grade 1 on TTCE