Rapid Fire Abstracts
Bharath Ambale Venkatesh, PhD
Research Faculty, Instructor
Johns Hopkins University
Shadi Afyouni, MD
Postdoctoral fellow
Johns Hopkins medicine
Emma Enriquez, BSc
Medical Student
Johns hopkins medicine
Ghazal Zandieh, MD
Postdoctoral fellow
Johns hopkins medicine
Muhammad Umair, MD
Assistant professor of radiology
Johns Hopkins Medicine
Julie Paik, MD
Associate Professor of Medicine
Johns Hopkins medicine
Stefan L. Zimmermann, MD
Professor
Johns Hopkins University School of Medicine
John Hopkins
Bharath Ambale-Venkatesh, PhD
Physicist
The John Hopkins Hospital
Background/Purpose: Idiopathic inflammatory myopathies (IIM) often involve development of myocarditis, and increasing morbidity and mortality. CMR is crucial for non-invasive assessment of subclinical cardiac involvement. Anti-Ku antibodies are found in some IIM patients, and typically involve a more severe disease course. Our goal was to elucidate the precise interplay of CMR parameters, clinical myocarditis, and anti-Ku antibodies in IIMs.
Methods:
Methods: This study included patients with IIM (excluding inclusion body myositis) who underwent CMRI at Johns Hopkins Hospital from 2019-2024. Imaging data included late gadolinium enhancement (LGE) assessment of scar, T1/T2 mapping, extracellular volume fraction ECV (Fig. 1), left and right atrial (LA, RA) and ventricular (LV, RV) function and strain analysis. All data were extracted using Circle CVI42 software. Myocarditis was defined according to the 2013 European Society of Cardiology criteria and confirmed using the 2018 Lake Louise Criteria (LLC) on CMR. Incident hospitalizations due to cardiac conditions (4 heart failure, 3 arrhythmic events) occurred in seven patients within 342 to 1566 (median=690) days after imaging. The association of CMR parameters with anti-Ku status was analyzed using student’s t-test, Mann-Whitney U test, and Fisher’s exact test as appropriate. Logistic regression analysis with adjustment for age, gender, body mass index, and anti-Ku status was used to assess association of imaging parameters with outcomes.
Results:
Results: Among 29 patients (median age 53 years; 79% female), 8 (27%) met criteria for myocarditis, and 7 (24%) had incident cardiac hospitalization (after the CMR. Six patients were anti-Ku positive. Patients with cardiac hospitalizations (Table 1) had higher LV end-diastolic and end-systolic volumes, and worse LV global circumferential strain (GCS). They also had higher RV volumes and lower RV ejection fraction (p < 0.001). Additionally, LA function (ejection fraction and reservoir strain) was impaired. The presence of anti-Ku antibodies was more frequent in those who had events (57.14% vs. 9%, p = 0.03). LV global longitudinal strain and LVGCS were significantly worse in anti-Ku positive patients, even if they did not meet the LLC. In anti-Ku positive patients, LV volumes were higher, LGE was more prevalent, and LV ejection fraction was lower. Multivariable logistic regression analysis revealed LV end-systolic volume (coefficient = 0.10, p = 0.03) and LV GCS (coefficient = 0.31, p = 0.03) were independently associated with adverse outcomes.
Conclusion:
Conclusions: This pilot study highlights the potential role of LV strain and detailed CMR phenotyping in IIM patients with and without clinical myocarditis. The presence of anti-Ku antibodies was linked to poor cardiac function and increased focal fibrosis. These findings underscore the importance of early and thorough cardiac assessment in myositis patients to guide clinical management and reduce cardiac hospitalization risk.
