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Sarcoid: What on earth is it?

Dr David R. TomlinsonConsultant Cardiologist and Electrophysiologist

South West Cardiothoracic CentrePlymouth

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– 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 FUBogun 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