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Sarcoid: What on earth is it?

Sarcoid: What on earth is it?. Dr David R. Tomlinson Consultant Cardiologist and Electrophysiologist South West Cardiothoracic Centre Plymouth. Sarcoidosis: What is it?. It depends on who you are... Medical student: Non-caseating granulomas SHO: MRCP answer

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Sarcoid: What on earth is it?

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  1. Sarcoid: What on earth is it? Dr David R. Tomlinson Consultant Cardiologist and Electrophysiologist South West Cardiothoracic Centre Plymouth

  2. Sarcoidosis: What is it? • It depends on who you are... • Medical student: Non-caseating granulomas • SHO: MRCP answer • Cardiologist: Uncommon cause of CHB, CHF and arrhythmias • AHP: Occasional patient with ICD

  3. Sarcoidosis: What is it? • Multisystem disorder • Granuloma formation in lung, lymph nodes, skin, eye, CNS, heart • Fibrotic reaction • Commonly affects young adults • Symptoms • Lung: Cough, SOB • Skin: Erythemanodosum • Other: Fatigue, weight loss, lymphadenopathy • Acute, self-limiting versus chronic disease

  4. Sarcoidosis: Cause • Unknown • Theories • Genetics • Immune reaction to environmental agents

  5. Pathogenesis

  6. Sarcoidosis: Cardiac involvement • ~25% prevalence • ~5% symptomatic • Common sites: • Myocardium • LV free wall and papillary muscles; basal septum; atria • Pericardium • Endocardium

  7. Cardiac sarcoidosis: Clinical sequelae • Conduction disturbances • CHB: 23-30% • First degree AV block or BBB • Arrhythmias • VT in 23% • AF / flutter / FAT in 15-17% (secondary to pulmonary involvement) • SCD • Terminal event in 67% • CHF • Progressive and cause of death in 25% • Other • Pericarditis (constriction), effusion (3-19%), tamponade rarely

  8. Cardiac sarcoidosis: Diagnosis

  9. Cardiac sarcoidosis: Treatment (I) • Disease modifying agent: Corticosteroids • Mechanism of action: Unknown • May improve prognosis • Do not reduce the incidence of VT • May reduce the incidence of VT during arrhythmia flare: Contradictory data • Disease activity monitoring required to allow dose reduction

  10. Treatment (II) • Treat secondary effects • Antiarrhythmics • No systematic studies • May exacerbate (bradyarrhythmias) • PPM • Frequently required • ICD • Recommended in patients with VT, regardless of LVEF • Cardiac transplantation • Rarely performed: Disease may recur in transplanted organ

  11. Cardiac sarcoidosis: VT ablation (I) • N=98 NIDCM referred: Sarcoidosis in 8% • VT presenting feature in 5/8: 7/8 had impaired LV • Note: 2 had presumptive ARVC • EPS / ablation: Scar-related re-entry • 4 +/- 2 VTs per patient • Low voltage scar in RV (8/8) and LV (5/8) • 1 or more VTs abolished in 75% • Recurrent VT in 6/8 at 6 months • Transplant required in 5/8 long term (VT in 4, CHF in 1) Stevenson WG Heart Rhythm. 2006 Aug;3(8):924-9.

  12. Cardiac sarcoidosis: VT ablation (II) • Multicentre registry, N=42 patients • VT refractory to medical therapy in 9 • Age 47 +/- 9 yrs, LVEF 42 +/- 14% • EPS / ablation • 44 VTs induced (mean TCL 348 +/- 78ms) • Endocardial RF in 8 (RV in 5, LV in 3), epicardial in 1 • 4 of 5 patients with right ventricular VTs had a peritricuspid re-entry (ablation success 100%) • 31 (70%) of 44 VTs eliminated • Outcome • Decrease (n = 4) or complete elimination (n = 5) of VT during 20 +/- 20 mo FU Bogun F et al Heart Rhythm. 2009 Feb;6(2):189-95.

  13. Cardiac sarcoidosis: Prognosis • Not certain! • Early studies: 2 yrs • Later studies: 40-60% 5 yr survival • NYHA status • LVEDD • VT

  14. Cardiac sarcoidosis: In perspective • How many cardiac sarcoid patients is the average AHP performing ICD follow up likely to review annually? • Sarcoid prevalence 10.9 / 100,000 • Cardiac involvement 25% • Symptomatic 5%, or 5.49 p.m. • VT prevalence 23%, or 1.3 p.m. • Making some assumptions... • 1 patient per year

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