Transcript Slide 1
Sarcoid: What on earth is it? Dr David R. Tomlinson Consultant Cardiologist and Electrophysiologist South West Cardiothoracic Centre Plymouth 1 Sarcoidosis: What is it? • It depends on who you are... – Medical student: Noncaseating 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 – 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 reentry (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 – Cardiac involvement – Symptomatic • VT prevalence • Making some assumptions... • 1 patient per year 10.9 / 100,000 25% 5%, or 5.49 p.m. 23%, or 1.3 p.m.