 Sarcoidosis is a multisystem granulomatous

disease of unknown etiology characterized by

the presence of non caseating granulomas in
the involved organs

 Virtually any body tissue may be involved

 Cardiac involvement in sarcoidosis occurs in 20-
30%

 Cardiac involvement a poor prognosis.

 Cardiac involvement 58% in

 Japanese patients with sarcoidosis and may be

responsible for 85% of deaths of Japanese
patients with sarcoidosis [6, 7].

 Only 5% of patients with sarcoidosis have clinical

manifestations of cardiac disease,
 Exact etiology unknown.
 Environmental
 Occupational
 Infectious
 3 major events: exposure to antigen;
acquired cellular immunity directed against
the antigen mediated through antigen
presenting cells and antigen specific T
lymphocytes; and the appearance of immune
effector cells that promote a more
nonspecific inflammatory response
 Thorough clinical evaluation

 A syndrome consistent with sarcoidosis with

a biopsy non caseating granulomas
 A definite diagnosis by endomyocardial
biopsy.

 Sensitivity less than 20% , patchy myocardial

involvement which frequently involves the
intraventricular septum and the left ventricle

 Helps to differentiate - idiopathic giant cell

myocarditis

 Both have giant cells and are associated with

ventricular tachycardia and heart block.
 Cardiac amyloidosis
 Alcohol related cardiomyopathy
 Lyme disease
 Rheumatoid arthritis
 Dermatomyositis
 Sinus tachycardia
 Non specific changes but should be clinically
correlated
 24-h Holter monitoring
 Sensitivity of 67% and a specificity of 62%
 When ventricular ectopic beats numbered
more than 100/day
 QT dispersion maximal interlead difference in
QT interval on the surface 12 lead ECG,
Predictor of sudden cardiac death
Carvedilol is protective
 Abnormal septal thickening or thinning,
 Dilatation of the left ventricle
 Diastolic dysfunction - early sign of
granulomatous involvement of the
myocardium .
 Cycle dependent variation of myocardial
integrated backscatter (CV-IB.
◦ Decreased in the basal septum
 Fibrogranulomatous lesions in the
myocardium display segmental areas of
decreased uptake in nuclear imaging.
 Improvement or complete resolution with
dipyridamole differentiates cardiac
sarcoidosis from coronary artery disease
 Sestamibi single-photon emission computer
tomography (SPECT)
 A zone of increased intramyocardial signal
intensity – suggests granuloma

 Delayed enhanced MRI is considered a useful

method for the early identification of cardiac
sarcoidosis

 The area with delayed enhancement

represents myocardium replaced by the
fibrogranulomatous tissue of sarcoidosis
 Safer in pateients with pacemakers and ICD

 Diagnosis and monitoring of patients with

cardiac sarcoidosis

 After steroids show reduced uptake

 In the presence of normal coronary arteries,
the perfusion defects on thallium scanning in
a patient with sarcoidosis strongly point
toward the existence of cardiac sarcoidosis.
 Immunosuppressive therapy
◦ indication for treatment because of increased risk
of sudden death.
 Definite or strong probability of cardiac
sarcoidosis on different imaging studies, even
with a negative myocardial biopsy
 Start with prednisone 60-80 mg a day and
taper the dose over 6 months to a dose of
10-mg a day.
 Consider second line in refractory cases
 Methotrexate, azathioprine, or
cyclophosphamide can be used
 Case report – infliximab is useful
 Implantable cardioverter defibrillator should
be considered as primary therapy
◦ Beta blockers can worsen conduction block
◦ Amiodarone causes pulmonary fibrosis
 Resistant ventricular tachyarrhythmias
 Severe intractable heart failure
 Young patients
 Can recur in transplanted heart
 Severity of congestive heart failure is the
most powerful prognostic predictor