Cardiac Sarcoidosis
Hearth Rhythm Society Criteria Cardiac Sarcoidosis 2015. Probable Cardiac Sarcoidosis if:
Other Causes of Cardiac Manifestations Excluded AND
Histologic Diagnosis of Extracardiac Sarcoidosis AND 1 of the following:
Unexplained LVEF < 40%
LGE pattern consistent with cardiac sarcoidosis
PET pattern consistent with cardiac sarcoidosis
Ga-111 pattern consistent with cardiac sarcoidosis
Steroid/Immunosuppressant responsive block or CMP
Unexplained sustained VT
Mobitz type II or 3rd Degree heart block
Imaging of Sarcoidosis
CXR - 50% of patients with biopsy proven sarcoid have abnormal CXR
HRCT - 95% of patients with biopsy proven sarcoid have abnormal HRCT
Unclear what above numbers are if patient has silent or clinically manifest cardiac sarcoidosis.
CMR
LGE - Patchy, multifocal, sparing subendocardium
Basal (common if silent) IVS, lateral wall.
Mid/Apical (may have block, VT, Failure) IVS, lateral wall
Intramural, subepicardial, transmural can occur
RV free wall
T2 - Active inflammation
ECHO
Basal thinning more common than thickened myocardium
May simulate LVH, HCM
Isolated WMA
Aneurysm
Systolic Diastolic dysfunction LV/RV
HRS Expert Consensus- Diagnosis and Management of Arrhythmias Associated with Cardiac Sarcoidosis - 2014
1 - Diagnosis and Screening
CMR/PET IIa
Abnormal symptoms/ECG/ECHO
Unexplained Block in patient < 60 yo
2 - Risk Stratification for Sudden Cardiac Death
CMR IIb
Coleman 2017 JACCi - Patients with known/suspected CS, +LGE
Odds of Composite (SCD, ASCD, Appropriate ICD Discharge, all Cause Mortality)
LVEF > 50% - OR 19
LVEF < 50% - OR 2 (higher prevalence of LGE+, no significant difference b/w LGE+ and LGE-)
Adverse Events - LGE Stratifies beyond LVEF assessment alone
73% - LGE+ (27% - LGE-)
Murtagh 2015 - LGE HR 30
Other studies - LVEF/LGE - LEG best independent variable HR 29
Odds of All Cause Mortality LGE+ - OR 3
3 - ICD Implantation
CMR (LVEF 36-49%, RVEF < 40%)
Coleman 2017 JACCi - Meta Analysis (+LGE, OR 3 All Cause Mortality) may justify
Skip EP. Get ICD.
Clinical Cardiac Sarcoidosis (CS)
The first manifestation of sarcoidosis may be CS
Of those presenting with AV block, VT of unknown etiology, 16-35% has previously undiagnosed CS
1/3 of CS is isolated CS (and most of CS has minimal extracardiac disease)
Most symptoms of clinically silent CS are due to extracardiac sarcoidosis
Lungs are not affected in 10% of sarcoidosis
5% of sarcoidosis is CS
Of known extracardiac sarcoidosis, 25% have clinically silent CS
Biopsy at LVAD placement for HF showed 3.4% had incidental CS
Jean Jeudy, Allen P. Burke, Charles S. White, Gerdien B. G. Kramer, and Aletta Ann Frazier
RadioGraphics 2015 35:3, 657-679