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Magnetic Resonance Imaging in the Assessment of the Risk of Sudden Death in Cardiac Sarcoidosis: What Is Extensive or
Pauli Pöyhönen1,2, Jukka Lehtonen1, Suvi Syväranta2
1Heart and Lung Center, Helsinki University Hospital and University of Helsinki, Finland (P.P., J.L., D.V., H.M., P.S., H.-K.N., M.K.).
Insights
Cardiac sarcoidosis patients with myocardial late gadolinium enhancement (LGE) covering ≥9.9% of left ventricular mass or ≥6 segments face a high risk of sudden cardiac death (SCD). These findings help define risk stratification for these patients.
Area of Science:
- Cardiology
- Cardiovascular Imaging
- Sarcoidosis Research
Background:
- Cardiac sarcoidosis presents a significant, yet poorly defined, risk for sudden cardiac death (SCD).
- Current guidelines suggest implantable cardioverter defibrillators for patients with extensive myocardial late gadolinium enhancement (LGE) on cardiac MRI, but "extensive" lacks precise definition.
Purpose of the Study:
- To define quantitative thresholds for myocardial late gadolinium enhancement (LGE) in cardiac sarcoidosis.
- To assess the association of LGE quantitative measures with the risk of sudden cardiac death (SCD) and ventricular tachycardia (VT).
Main Methods:
- Analysis of a nationwide cardiac sarcoidosis registry including cardiac MRI data.
- Quantification of LGE mass (% of LV mass) and LGE segments (17-segment model).
- Cumulative incidence analyses for SCD/VT, considering competing events, using Fine and Gray modeling and ROC analysis.
Main Results:
- In 305 patients, LGE mass and number of LGE segments predicted SCD/VT (P<0.001).
- Discriminative thresholds identified: LGE mass ≥9.9% and ≥6 LGE segments.
- Patients with LGE mass <9.9% or <6 segments had a 5-year SCD/VT rate of ~6.5%, versus ~21% for those exceeding these thresholds.
Conclusions:
- Myocardial LGE ≥9.9% of LV mass or ≥6 LV segments indicates significant involvement in cardiac sarcoidosis.
- These thresholds suggest a high risk for sudden cardiac death (SCD) in affected patients.
- Prospective validation of these quantitative LGE thresholds is recommended.
Background:
Cardiac sarcoidosis involves a significant but difficult-to-define risk of sudden cardiac death (SCD). Current guidelines recommend consideration of an implantable cardioverter defibrillator for patients with extensive or significant myocardial late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging. However, extensive/significant LGE is not defined.
Methods:
A nationwide cardiac sarcoidosis registry was screened for patients entered before 2020 with cardiac magnetic resonance imaging done before or <3 months after diagnosis. Available studies were re-analyzed for LGE mass as a percentage of left ventricular (LV) mass and the number of LGE-positive LV segments in a 17-segment model. The occurrence of fatal or aborted SCD and ventricular tachycardia (VT) prompting therapy was recorded until the end of 2020 and subjected to cumulative incidence analyses, including competing events (LV assist device implantations, heart transplantations, and fatalities other than SCD). The predictors of SCD/VT were assessed using Fine and Gray modeling and time-dependent receiver operating characteristic analysis.
Results:
Altogether, 305 patients (66% women, median age 51) with clinically manifest, definite (45%) or probable cardiac sarcoidosis (55%) were analyzed. On follow-up (median, 4.0 years), 21 SCDs, 60 VTs, and 14 competing events were noted. Both LGE mass and the number of LGE segments predicted the composite of SCD/VT (P<0.001), with receiver operating characteristic analyses identifying LGE mass ≥9.9% and ≥6 LGE segments as discriminative thresholds. At presentation, 70 patients were free of class I and class IIa implantable cardioverter defibrillator indications unrelated to LGE. Their 5-year rate of SCD/VT was 6.3% (0.0-14.8%) with LGE mass <9.9% versus 21.5% (6.5-36.6%) with higher LGE mass, and 6.9% (0.0-16.3%) with <6 LGE segments versus 20.5% (5.9-35.2%) with ≥6 segments.
Conclusions:
In cardiac sarcoidosis, myocardial LGE making up ≥9.9% of LV mass or affecting ≥6 LV segments may suggest prognostically significant LV involvement and a high risk of SCD. However, prospective validation of the thresholds is needed.
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