Cardiac_Sarcoidosis_Summary.pptxCardiac_Sarcoidosis_

Cardiac_Sarcoidosis_Summary.pptx

1. Cardiac Sarcoidosis — Summary & Key Management Definition • Diagnosis • Imaging • Prognosis • Treatment • Recommendations

2. Definition & Pathophysiology Multisystem granulomatous disease with noncaseating granulomas; myocardium most commonly affected. Course: inflammation → resolution or fibrosis; can involve pericardium/endocardium. Cardiac manifestations: conduction disease, VT, aneurysm, global ↓ LVEF; ↑ risk of HF and SCD as disease becomes overt.

3. Epidemiology & Clinical Impact Autopsy: 20–50% of sarcoidosis has cardiac involvement; many clinically silent early. ≈5% present with cardiac‑predominant disease (no classic extracardiac signs). Cardiac sarcoid is a leading cause of sarcoidosis‑related mortality—often preventable with appropriate therapy.

4. Presentation & ECG/Holter Clues Symptoms: palpitations, syncope, dyspnea, chest pain; HF when advanced. ECG: BBB, new AV block (often complete at younger age), PVCs, VT, pathologic Q waves, ST‑T changes. Holter: frequent ventricular ectopy (>100/24h proposed threshold) supports further evaluation.

5. Diagnostic Workup (Stepwise) Initial: H&P, ECG, chest radiograph → if abnormal or symptomatic, proceed to TTE + Holter. Advanced imaging if suspicion persists: CMR with LGE and/or FDG‑PET. Consider extracardiac biopsy to establish systemic sarcoid; cardiac involvement inferred with imaging. EMB: confirms if noncaseating granulomas found, but sensitivity limited due to patchy disease.

6. Echocardiography Findings Systolic/diastolic dysfunction; regional wall‑motion abnormalities. Abnormal septal thickness; abnormal Doppler filling patterns. Essential first‑line test in suspected cardiac involvement.

7. Advanced Imaging — CMR & FDG‑PET CMR: LGE in mid‑myocardium; basal septum & lateral walls commonly involved; T2 signals indicate edema/inflammation. FDG‑PET: detects active inflammation; helpful for diagnosis and treatment response; prognostic value reported.

8. Endomyocardial Biopsy (EMB) Diagnostic when noncaseating granulomas seen. Sensitivity limited (RV EMB + patchy disease → ~25–50% in autopsy‑proven cases). Absence of granulomas does not exclude cardiac sarcoid.

9. Prognosis & Predictors Historic survival poor once cardiac symptoms develop; contemporary cohorts show improved outcomes with therapy. Five‑year survival improved with steroids; survival ~89% when EF ≥50%. Predictors of mortality: ↑ LVEDD, higher NYHA class, sustained VT.

10. Treatment — Immunosuppression Corticosteroids = mainstay (e.g., prednisone 40–60 mg/day, slow taper); start during active inflammation and before LV dysfunction. Close follow‑up for relapse after taper/cessation. Steroid‑sparing/add‑on agents if intolerance or progression: methotrexate, azathioprine, mycophenolate, cyclophosphamide, pentoxifylline, thalidomide (multidisciplinary management).

11. Treatment — HF & Arrhythmias Apply guideline‑directed medical therapy for HFrEF when LVEF reduced. High arrhythmic risk (25–65% of deaths): evaluate for pacemaker/ICD. ICD reasonable even with relatively preserved LVEF if arrhythmic risk is high; Class IIa per ACCF/AHA/HRS (2012).

12. Advanced Therapies Refer for advanced HF care: MCS and/or transplant when refractory (assess extracardiac disease burden). Transplant outcomes favorable; recurrences may occur but usually steroid‑responsive. Surgical options (e.g., aneurysm resection, ventricular exclusion) reported with variable success.

13. Key Recommendations — Strong Consensus Perform echocardiogram in HF symptoms to assess LVEF (LoE C). Refer advanced HF for transplant/MCS when appropriate (LoE C). Treat with corticosteroids (LoE B). Use guideline‑directed HF therapy for reduced EF (LoE B).

14. Key Recommendations — Moderate Consensus Use CMR and/or FDG‑PET for diagnosis and to follow response (LoE B). EMB can confirm but negative biopsy does not exclude disease (LoE C). Consider other immunosuppressants if steroid‑intolerant or refractory (LoE C). Coordinate immune‑modulating therapy with pulmonology/rheumatology (LoE C). ICD implantation is reasonable (LoE C).

15. Practical Algorithm (Condensed) Suspect in sarcoid patients with arrhythmia, AV block, HF, or unexplained LV dysfunction. Baseline: ECG + CXR → TTE + Holter. If suspicious: CMR (LGE) and/or FDG‑PET (active inflammation). Seek extracardiac biopsy for systemic confirmation; consider EMB if diagnosis uncertain and will change management. Treat with steroids ± steroid‑sparing agents; apply GDMT; assess for ICD; refer advanced cases for transplant/MCS.