Dr. Jonna presented a case of a 74 yo M who presented after a pulseless Vtach arrest which deteriorated to Vfib. He achieved ROSC after 13 minutes. He had a hx of 2:1 AV block s/p DC PPM (2015). He was managed for cardiogenic shock. After an extensive workup (including MRI, endomyocardial biopsy) and it was determined that this patient had isolated Cardiac Sarcoidosis!
Vocab:
Sudden Cardiac Arrest - rapidly developing pulselessness with return of spontaneous circulation (ROSC) after code
Sudden Cardiac Death - rapidly developing pulselessness without ROSC (death).
Two Diagnostic Schemas for Sudden Cardiac Arrest / Sudden Cardiac Death:
Cardiac Sarcoidosis = sarcoidosis of the endocardium, myocardium, pericardium, or coronary arteries. Most common area of involvement = myocardium. Recall, sarcoidosis is a systemic dz of unknown etiology resulting in autoimmune-mediated Non-caeseating granulomas (no necrosis).
In Cardiac Sarcoidosis specifically:
- Most common is Ventricular Myocardium
- Atria, papillary muscles, and valves
- Array of clinical pictures arise from the amount and location of inflammation
Epi:
•Average age of presentation is 50
•20-27 % of patients with systemic sarcoidosis
•Isolated Cardiac Sarcoidosis (25% of CS cases)
Most Common Presentations of Cardiac Sarcoid
•Conduction System Abnormalities
–First Degree AV block > 2nd degree > Complete Heart Block
•Supraventricular Tachycardia (granulomas in SA node)
•Ventricular Tachycardia (granulomas in myocardium disrupting activation and repolarization)
•Dilated (reduced EF) > Restrictive Cardiomyopathy (preserved)
Diagnosis
suspect if unexplained:
1. AV Block
2. Arrhythmogenic cardiomyopathy
3. Sustained VT
Image w/ MRI. If inconclusive can use PET.
Cardiac MR in Sarcoid:
- multi focal (late gadolinium enhancement) LGE
- mid-myocardial LGE
- extension of LGE from LV, across intraventricular segment into RV
- biopsy reveals: non-caseating granulomas
Treatment
Increased risk of CAD -> use statin
Guideline-directed HFrEF therapy
Immunosuppression
Indications: active inflammation (from PET), conduction abnormalities, RV involvement
Why you use it: prevents progression of LV dysfunction
Options: glucocorticoids #1, MTX, AZA, INF, MMF
PPM for certain conduction abnormalities
High risk VT or SCD - ICD Implantation.
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