CARDIOVASCULAR JOURNAL OF AFRICA • Vol 21, No 3, May/June 2010
162
AFRICA
Treatment
The mainstay of cardiac sarcoidosis treatment is corticoster-
oids, although most supporting data are anecdotal and studies
that have been performed are retrospective.
9,10
Treatment with
methotrexate, azathioprine, chloroquine, cyclophosphamide and
infliximab has been described in the literature for patients intol-
erant of corticosteroids or with progression of cardiac dysfunc-
tion despite corticosteroids.
9,32-34
Medical therapy for heart failure and pacemakers/implantable
cardiodefibrillators are indicated according to current guidelines.
Radiofrequency ablation has been successful in treating ventricu-
lar tachycardia (VT) refractory to medical therapy.
35
Transplant
is the ultimate therapy in end-stage heart failure or intractable
ventricular arrhythmias refractory to both medical therapy and
devices. In one series of 65 patients with cardiac sarcoidosis
who underwent heart transplant, one-year survival was better
than for other transplant indications (87.7 vs 84.5%,
p
=
0.030).
The five-year survival was estimated at 80%.
11
The incidence of
recurrence of cardiac sarcoidosis post-transplant is unknown,
however, it has been described in the literature.
36
Transmission of
sarcoidosis has been hypothesised based on case reports (‘donor-
acquired sarcoidosis’),
37
and has been described in a patient post
heart transplantation.
38
Prognosis
Originally,survivalafteronsetofcardiacmanifestationswasrough-
ly two years based on autopsy data,
6
however, more recent studies
in patients receiving treatment have the five-year survival at 40 to
60%.
10,39
The cause of death in cardiac sarcoidosis is, again, most-
ly secondary heart block or ventricular fibrillation. Concomitant
pulmonary and cardiac involvement carries a worse prognosis.
8
A multivariate analysis by Yazaki
et al
. identified NYHA class,
left ventricular end-diastolic diameter, and a history of sustained
ventricular tachycardia as independent predictors of mortality.
10
Conclusion
Subclinical cardiac involvement in patients with sarcoidosis
is common. ECG abnormalities are the commonest presenta-
tion of cardiac sarcoidosis. Abnormalities can also be detected
on echocardiogram, myocardial perfusion scans, cardiac MRI
and PET. Screening of patients with sarcoidosis for cardiac
involvement with CMR or PET, especially with abnormal ECG
or echocardiograms, should be performed. Cardiac dysfunction
secondary to sarcoid infiltration responds to steroid therapy
and transplant is an option in patients with severe, symptomatic
cardiomyopathy. Patients should also be evaluated for implant-
able defibrillator, certainly in those with low ejection fraction
or a history of ventricular arrhythmia or sudden cardiac death.
Lastly, although the five-year survival of patients with cardiac
sarcoidosis is roughly 50% with steroid treatment, aggressive
medical management of independent predictors of mortality,
namely signs and symptoms of heart failure and ventricular
tachycardia, may also improve survival.
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