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. 2022 Oct 1;7(10):1057-1066.
doi: 10.1001/jamacardio.2022.2981.

Cardiovascular Magnetic Resonance Imaging Phenotypes and Long-term Outcomes in Patients With Suspected Cardiac Sarcoidosis

Pal Satyajit Singh Athwal  1 , Sanya Chhikara  1 , Mohamed F Ismail  1 , Khaled Ismail  1 , Fredrick M Ogugua  1 , Felipe Kazmirczak  1 , Parag H Bawaskar  1 , Andrew C Elton  1 , Jeremy Markowitz  1 , Lisa von Wald  1 , Henri Roukoz  1 , Maneesh Bhargava  2 , David Perlman  2 , Chetan Shenoy  1
Affiliations

Cardiovascular Magnetic Resonance Imaging Phenotypes and Long-term Outcomes in Patients With Suspected Cardiac Sarcoidosis

Pal Satyajit Singh Athwal et al. JAMA Cardiol. .

Abstract

Importance: In patients with sarcoidosis with suspected cardiac involvement, late gadolinium enhancement (LGE) on cardiovascular magnetic resonance imaging (CMR) identifies those with an increased risk of adverse outcomes. However, these outcomes are experienced by only a minority of patients with LGE, and identifying this subgroup may improve treatment and outcomes in these patients.

Objective: To assess whether CMR phenotypes based on left ventricular ejection fraction (LVEF) and LGE in patients with suspected cardiac sarcoidosis (CS) are associated with adverse outcomes during follow-up.

Design, setting, and participants: This cohort study included consecutive patients with histologically proven sarcoidosis who underwent CMR for the evaluation of suspected CS from 2004 to 2020 with a median follow-up of 4.3 years at an academic medical center in Minnesota. Demographic data, medical history, comorbidities, medications, and outcome data were collected blinded to CMR data.

Exposures: CMR phenotypes were identified based on LVEF and LGE presence and features. LGE was classified as pathology-frequent or pathology-rare based on the frequency of cardiac damage features on gross pathology assessment of the hearts of patients with CS who had sudden cardiac death or cardiac transplant.

Main outcomes and measures: Composite of ventricular arrhythmic events and composite of heart failure events.

Results: Among 504 patients (mean [SD] age, 54.1 [12.5] years; 242 [48.0%] female and 262 [52.0%] male; 2 [0.4%] American Indian or Alaska Native, 6 [1.2%] Asian, 90 [17.9%] Black or African American, 399 [79.2%] White, 5 [1.0%] of 2 or more races (including the above-mentioned categories and Native Hawaiian or Other Pacific Islander), and 2 [0.4%] of unknown race; 4 [0.8%] Hispanic or Latino, 498 [98.8%] not Hispanic or Latino, and 2 [0.4%] of unknown ethnicity), 4 distinct CMR phenotypes were identified: normal LVEF and no LGE (n = 290; 57.5%), abnormal LVEF and no LGE (n = 53; 10.5%), pathology-frequent LGE (n = 103; 20.4%), and pathology-rare LGE (n = 58; 11.5%). The phenotype with pathology-frequent LGE was associated with a high risk of arrhythmic events (hazard ratio [HR], 12.12; 95% CI, 3.62-40.57; P < .001) independent of LVEF and extent of left ventricular late gadolinium enhancement (LVLGE). It was also associated with a high risk of heart failure events (HR, 2.49; 95% CI, 1.19-5.22; P = .02) independent of age, pulmonary hypertension, LVEF, right ventricular ejection fraction, and LVLGE extent. Risk of arrhythmic events was greater with an increasing number of pathology-frequent LGE features. The absence of the pathology-frequent LGE phenotype was associated with a low risk of arrhythmic events, even in the presence of LGE or abnormal LVEF.

Conclusions and relevance: This cohort study found that a CMR phenotype involving pathology-frequent LGE features was associated with a high risk of arrhythmic and heart failure events in patients with sarcoidosis. The findings indicate that CMR phenotypes could be used to optimize clinical decision-making for treatment options, such as implantable cardioverter-defibrillators.

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Conflict of interest statement

Conflict of Interest Disclosures: Ms von Wald has received speaking fees from Medtronic. Dr Roukoz has received consulting fees from Boston Scientific and speaking fees from Medtronic. Dr Shenoy has received consulting fees from Medtronic outside the submitted work and grants from National Institutes of Health, University of Minnesota Clinical and Translational Science Institute, and Lillehei Heart Institute during the conduct of the study. No other disclosures were reported.

Figures

Figure 1.
Figure 1.. Identification of Cardiovascular Magnetic Resonance Phenotypes Based on Left Ventricular Ejection Fraction (LVEF), Late Gadolinium Enhancement (LGE) Presence, and LGE Features
Figure 2.
Figure 2.. Representative Examples of Patients With Each of the 4 Distinct Cardiovascular Magnetic Resonance (CMR) Phenotypes
ATP indicates antitachycardia pacing; ICD, implantable cardioverter-defibrillator; LGE, late gadolinium enhancement; LVEF, left ventricular ejection fraction; RVEF, right ventricular ejection fraction; VT, ventricular tachycardia.
Figure 3.
Figure 3.. Kaplan-Meier Cumulative Incidence Curves for the Arrhythmic and Heart Failure End Points for Patients Stratified by Cardiovascular Magnetic Resonance (CMR) Phenotype
LGE indicates late gadolinium enhancement; LVEF, left ventricular ejection fraction.
See this image and copyright information in PMC

References

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