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Understanding Sarcoidosis: Insights from a Cardiologist on Cardiac Involvement and Treatment

Sarcoidosis is a multifaceted disorder characterized by non-caseating granulomas primarily affecting the lungs, lymph nodes, skin, and heart. In this overview, Dr. David R. Tomlinson, a consultant cardiologist, explores the implications of cardiac sarcoidosis, including its prevalence, diagnosis, and treatment options. He discusses the common clinical challenges posed by conduction disturbances, arrhythmias, and potential treatments such as corticosteroids and interventional strategies like VT ablation. Understanding this condition is essential for timely management in patients.

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Understanding Sarcoidosis: Insights from a Cardiologist on Cardiac Involvement and Treatment

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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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