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CMR Phenotypes and Outcomes in Patients With Suspected Cardiac Sarcoidosis
Quick Takes
- CMR phenotype incorporating the features of cardiac damage frequently seen on gross pathology images of patients who died of cardiac sarcoidosis (CS) or underwent cardiac transplant for CS identified patients at high risk of arrhythmic and heart failure events.
- Furthermore, the association between the pathology-frequent LGE phenotype and arrhythmic events was greater with an increasing number of pathology-frequent LGE features.
- These findings suggest that CMR phenotypes may be used to optimize clinical decision making for suspected CS, such as ICDs, and thus improve outcomes in patients with CS, but needs further validation.
Study Questions:
Are cardiovascular magnetic resonance (CMR) phenotypes based on left ventricular ejection fraction (LVEF) and late gadolinium enhancement (LGE) associated with long-term adverse outcomes in patients with suspected cardiac sarcoidosis (CS)?
Methods:
The investigators conducted a cohort study and included consecutive patients with histologically proven sarcoidosis who underwent CMR for the evaluation of suspected CS from 2004–2020 with a median follow-up of 4.3 years at one academic medical center. Demographic data, medical history, comorbidities, medications, and outcome data were collected and blinded to CMR data. 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. The main outcomes measures were a composite of ventricular arrhythmic events and composite of heart failure events.
Results:
Among 504 patients (mean [standard deviation] age, 54.1 [12.5] years; 242 [48.0%] female and 262 [52.0%] male; two [0.4%] American Indian or Alaska Native, six [1.2%] Asian, 90 [17.9%] Black or African American, 399 [79.2%] White, five [1.0%] of ≥2 races (including the above-mentioned categories and Native Hawaiian or Other Pacific Islander), and two [0.4%] of unknown race; four [0.8%] Hispanic or Latino, 498 [98.8%] not Hispanic or Latino, and two [0.4%] of unknown ethnicity), four 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% confidence interval [CI], 3.62-40.57; p < 0.001) independent of LVEF and extent of LV LGE. It was also associated with a high risk of heart failure events (HR, 2.49; 95% CI, 1.19-5.22; p = 0.02) independent of age, pulmonary hypertension, LVEF, right ventricular ejection fraction, and LV LGE 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:
The authors concluded 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.
Perspective:
This cohort study of patients with histology-proven sarcoidosis and cardiac involvement, reports that a CMR phenotype incorporating the features of cardiac damage frequently seen on gross pathology images of patients who died of CS or underwent cardiac transplant for CS identified patients at high risk of arrhythmic and heart failure events. Furthermore, the association between the pathology-frequent LGE phenotype and arrhythmic events was greater with an increasing number of pathology-frequent LGE features. Contrariwise, the absence of the pathology-frequent LGE phenotype was associated with a low risk of arrhythmic events, despite the presence of LGE or abnormal LVEF. These findings suggest that CMR phenotypes may be used to optimize clinical decision making for suspected CS, such as implantable cardioverter-defibrillators (ICDs), and thus improve outcomes in patients with CS, but needs further validation in other cohorts.
Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Diabetes and Cardiometabolic Disease, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Prevention, Pulmonary Hypertension and Venous Thromboembolism, Vascular Medicine, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Cardiac Surgery and Arrhythmias, Cardiac Surgery and Heart Failure, Acute Heart Failure, Heart Transplant, Pulmonary Hypertension, Interventions and Imaging, Interventions and Vascular Medicine, Magnetic Resonance Imaging, Hypertension
Keywords: Arrhythmias, Cardiac, Death, Sudden, Cardiac, Defibrillators, Implantable, Diagnostic Imaging, Ethnic Groups, Gadolinium, Heart Failure, Heart Transplantation, Hypertension, Pulmonary, Magnetic Resonance Imaging, Cine, Magnetic Resonance Spectroscopy, Phenotype, Sarcoidosis, Secondary Prevention, Stroke Volume, Ventricular Function, Left, Ventricular Function, Right
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