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: Erythema nodosum
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 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

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 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 Feb;6(2):

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 / 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