Abstract
Background
Clinically manifest cardiac sarcoidosis (CS) has a prevalence of 5%, but is more frequent in autoptic series (25%). Diagnosis is multiparametric and relies on clinical criteria and imaging findings, although a certain diagnosis, especially in the case of isolated CS (ICS), can only be based on endomyocardial biopsy. Cardiovascular magnetic resonance (CMR) has a comprehensive role in the assessment of CS: left ventricular (LV) dysfunction and extent of late gadolinium enhancement (LGE)are important predictors of prognosis, T2 mapping provides information on disease activity and global longitudinal strain (GLS) analysis can uncover subclinical LV impairment.
Purpose
To assess the prevalence of CS by CMR in patients with biopsy-proven extracardiacsarcoidosis (ECS); to describe the imaging characteristics of patients with ECS and those with high clinical suspicionof ICS; to investigate the contribution of more recent techniques to the diagnosis of CS alongside traditional LGE assessment.
Methods
We retrospectively enrolled 84 patients (66% males, mean age 59±13 years) referred to our centreforsuspected CS (biopsy-proven ECS, n=61; clinical presentation suggestive of CS,, n=23). CMR was performed on a 1.5T scanner, with a protocol comprehensive of biventricular functional assessment and post-contrast images; T2-STIR images (n=30), native myocardial T1 mapping (n=24) and T2 mapping (n=19) were also performed in selected patients. Tissue tracking analysis was perfomed in all patients using a dedicated software.
Results
Based on CMR findings, 35 patients (42%) with ECS did not show cardiac involvement (SS), 26 (31%) showed both cardiac and systemic involvement (CS-SS) and 23 (27%) had evidence of ICS (ICS). 43% of patients had history of arrhythmias, but life-threatening tachyarrhythmiaswere more frequent in patients with CS (p=0.02).Patients with CS had significantly lower LVEF (p<0,01), larger LV volumes (p<0,01) and greater LV mass (p<0,01). GLS values were impaired in all the groups but significantly more in patients with CS (p<0,01). With regards to LGE distribution, ICS patients showed a higher number of segments involved (p=0,011) as compared to CS patients. T2-STIRimages were positive in 3 out of 30 patients; T2 mapping detected myocardial oedema in 1 patient with negative T2- STIR and was positive in 7 who did not undergo traditional oedema evaluation. T1 mapping mainly confirmed the results provided by LGE, but was altered in 1 patient who could not receive gadolinium.
Conclusions
CMR findings consistent with CS were found in 49 patients referred for suspected CS. Patients with cardiac involvement, particularly if isolated, had significantly lower LVEF, greater LV volumes and more impaired GLS. Patients with SS, despite a normal LV function, showed mildly impaired GLS, subtending subclinical cardiac involvement.
Funding Acknowledgement
Type of funding source: None
Early myocardial damage and microvascular dysfunction in asymptomatic patients with systemic sclerosis: A cardiovascular magnetic resonance study with cold pressor test
