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 Cardiologist: Uncommon cause of CHB, CHF and arrhythmias AHP: Occasional patient with ICD

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: Erythema nodosum Other: Fatigue, weight loss, lymphadenopathy Acute, self-limiting versus chronic disease

Sarcoidosis: Cause Unknown Theories Genetics Immune reaction to environmental agents

Pathogenesis

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

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

Cardiac sarcoidosis: Diagnosis

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

Treatment (II) Treat secondary effects Cardiac transplantation 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

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.

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.

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

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