Non-ischemic Primary and Secondary Cardiomyopathy
Nisha Hosadurg, MD
Fellow Physician
University of Virginia Health System, United States
Nisha Hosadurg, MD
Fellow Physician
University of Virginia Health System, United States
Jeremy A. Slivnick, MD
Assistant Professor
University of Chicago Medicine
Chicago, Illinois, United States
Varun Subashchandran, MD
Resident Physician
University of Chicago Medicine, United States
Mohammad Abuannadi, MD
Assistant Professor
University of Virginia Health System, Virginia, United States
Catherine Bonham, MD
Assistant Professor
University of Virginia Health System, United States
Jamieson M. Bourque, MD
Associate Professor
University of Virginia Health System, United States
Christopher M. Kramer, MD
Chief
University of Virginia Health System
Charlottesville, Virginia, United States
Amit R. Patel, MD
Professor
University of Virginia Health System
Charlottesville, Virginia, United States
Late gadolinium enhancement (LGE) by cardiac magnetic resonance (CMR) imaging is increasingly used to diagnose cardiac sarcoidosis (CS) in patients with extracardiac sarcoidosis (ES), and its presence is associated with an increased risk of death and malignant arrhythmias1. Whether LGE in quiescent CS with preserved ejection fraction (EF) is associated with adverse myocardial remodeling and worsening left ventricular (LV) EF, is unknown. The goal of this study is to determine if the burden of LGE in individuals with quiescent CS and preserved LVEF is associated with deterioration in LV function.
Methods:
We retrospectively studied 36 consecutive sarcoidosis patients (19 with and 17 without CS) with a baseline LVEF >40% who had undergone two CMRs at least 6 months apart. Cardiac chamber dimensions and burden of late gadolinium enhancement (5-SD method) were quantified using suiteHeart (v 5.0.3, NeoSoft). We excluded patients with active CS [inflammation on CMR/PET, temporally related sustained arrhythmias, conduction system disease, increase in immunosuppression (IS)]. Change in parameters between the two CMRs were compared using the Wilcoxon signed-rank test or Fisher exact test (IBM SPSS Statistics, version 28.0.0.1) for patients with and without CS. Patients with CS were divided by LGE burden (≥ vs < 5%).
Results:
The mean age of CS patients was 54 ± 2 years. They were predominantly male (53%), black (68%), obese (mean body mass index 32 ± 1.7) and hypertensive (53%). Coronary disease and prior ventricular tachycardia were respectively present in 11% and 5%. The median interval between CMRs was 23 [interquartile range (IQR) 11-42] months. IS and heart failure guideline directed therapies were each used in 53% patients prior to the first CMR and continued in 47% prior to the second CMR.
Patients with and without CS had no significant baseline differences in LVEF, right ventricular (RV) EF, RV and LV indexed volumes, mass or LGE burden. There was a reduction in LVEF in CS patients compared to those without CS [-1% (IQR -7 to 4%) vs. +2% (IQR -0.5 to 4%); P=0.045] at the second CMR. No other parameters changed significantly between the two exams in either group (Table 1).
Among patients with CS who had an LGE burden ≥ 5%, (1) there was a significant interval decrease in LVEF [55.6% (IQR 51.6 to 58.6 %) vs. 51% (IQR 46.5 to 53%); P=0.03] and (2) the decrease was more pronounced than seen in patients with LGE < 5% [-5 % (IQR -9 to 1.8%) vs. -1 % (IQR -2 to 8.5%; P=0.04]. There was no difference in change in chamber volumes or mass (Table 2) between the LGE groups.
Conclusion: Among quiescent CS subjects with preserved EF, the presence of LGE burden ≥5% may be associated with a future decrease in LVEF and warrants further assessment in larger cohorts. The prognostic significance of this decrease and potential treatment implications for this scenario remain to be determined.