cardiac sracoidosis presenting as STEMI with cardiac symptoms

1. The Indian Practitioner q Vol.69 No.8.August 2016
Case Report
Introduction
S
arcoidosis is a multisystem disease of variable eti-
ology (genetic, infectious, environmental) which af-
fects lungs, skin, liver, eyes, brain and other organs.1
Infrequently, cardiac involvement is seen; the patient may
be completely asymptomatic or may have complaints at-
tributable to conduction disturbances or congestive heart
failure or even sudden death. EMB is the gold standard in-
vestigation for the diagnosis of CS; echocardiography and
various other scans might help in diagnosis. However, CS
can be easily missed if not for a high index of suspicion.
It is to be considered in any young patient with the above
clinical presentation, in the absence of any other obvi-
ous cause. ECG may be normal, may show arrhythmias
like ventricular ectopics, varying atrio-ventricular blocks,
ventricular and atrial arrhythmias, or non-specific ST-T
changes.2
ST segment elevation mimicking STEMI is an
Fibrinolysis in STEMI -ASecond thought
ABSTRACT
Cardiac sarcoidosis (CS) is an infrequent element of systemic sarcoidosis, manifesting in upto 2% cases of
sarcoidosis, but found in upto 25% of these on autopsy. Despite a plethora of tools such as ECG, echocardiog-
raphy, cardiac MRI, PET scan, endomyocardial biopsy (EMB), accurate antemortem diagnosis of CS remains a
challenge. Pathological hallmark of CS is a noncaseating epitheloid granuloma, either microscopic or macro-
scopic. It causes conduction defects manifesting on ECG as a variety of arrhythmias or non-specific ST-T seg-
ment changes. Uncommon amongst these is an ST segment elevation mimicking myocardial infarction (MI). This
triggers an urge for fibrinolysis if a proper history and other confirmatory biochemical markers are not sought.
We report a previously healthy male presenting with chest discomfort and progressive breathlessness, and with
ECG suggestive of ST segment elevation myocardial infarction (STEMI). Fibrinolytic therapy was spared owing to
a high index of suspicion for CS against a background of detailed history and incoherent biochemistry. CS was
confirmed by cardiac MRI and endomyocardial biopsy. Partial remission of symptoms was observed after steroid
therapy alongwith anti-arrhythmics.
uncommon but misleading finding on ECG.3
In such a
case, if incoherence exists between history, biochemistry
and ECG, the patient must be subjected to a more detailed
evaluation before injudicious fibrinolysis.
Case Report
A 30 year old, previously healthy male, presented with
episodic, non-exertional, reterosternal discomfort, poorly
localised, self limited (lasting around 30 seconds), associ-
ated with breathlessness. Both these symptoms had pro-
gressed over two months. No other cardio-respiratory or
systemic symptoms. He had been treated locally, but with
no relief. No similar complaints in the family. Physical
examination was completely unremarkable. On admis-
sion 12 lead ECG showed ST elevation in leads V1
to V4
suggesting an anteroseptal MI. However, troponin-T spot
test was negative, cardiac enzymes were normal (CPK-
20 IU/L, CPK-MB- 84 IU/L) alongwith other routine bio-
Adukia S A1
, Diwan A G2
, Chavan C, Jagade N3
1
Senior Resident, Medicine, 2
Professor and Head, Medicine, 3
Post Graduate Student - Medicine
Corresponding author: Adukia S A - Senior Resident, Bharati Hospital And Research Center, Katraj, Dhankawadi,
Pune-Satara Road, Pune-411043
37

2. Case Report
The Indian Practitioner q Vol.69 No.8.August 2016
chemistry. Chest radiograph revealed a mildly enlarged
cardiac silhouette. Echocardiography showed mild left
ventricular dilatation with left ventricular ejection frac-
tion 50% and mild diastolic dysfunction; no regional wall
abnormalities, pericardial effusion or asymmetric septal
hypertrophy were seen. Fibrinolysis was withheld and
causes of ST elevation other than STEMI were considered.
Serial ECG’s revealed persistent ST elevation which ruled
out transient coronary occlusion followed by spontane-
ous reperfusion. Serial cardiac enzyme levels were nor-
mal, thus ruling out myocarditis. Cardiac sarcoidosis was
considered and investigated. Holter monitoring showed
130 ventricular ectopics in 24 hours. Serum Angiotensin
converting enzyme (ACE) level was elevated-445 µmol/L
(normal=5 to 15 µmol/L). Cardiac MRI revealed increased
signal intensity on T2-weighted images and gadolinium-
Fig 1: Cardiac MRI showing myocardial
edema over ventricular free walls (white
arrow)
Fig 2: Cardiac MRI showing myocardial edema
over ventricular free walls (white arrows)
Fig 3: Endomyocardial biopsy (with
Haematoxylin and Eosin staining)
showing epitheloid non-caseating
granulomas (white arrow)
enhanced images in the basal septal and
lateral walls of the left ventricle (Fig. 1
and Fig. 2). These were suggestive of in-
flammation associated with myocardial
edema in CS. Occasional nodular struc-
tures were also seen in these regions,
probably representative of sarcoid
granulomas. MRI guided EMB revealed
epitheloid non-caseating granulomas
(Fig. 3) consistent with cardiac sarcoid-
osis. Thus, CS was confirmed. Patient
was stared on oral prednisolone 30 mg
OD and discharged. One month later,
on follow up, there was partial remis-
sion of symptoms with near normaliza-
tion of serum ACE level and attenua-
tion of ST-segment elevation.
Discussion
Sarcoidosis is a multisystem dis-
ease characterized histologically by the
formation of granulomas in many tis-
sues. Cardiac involvement manifests
in upto 2% of diagnosed sarcoidosis. However, during
autopsy of diagnosed sarcoidosis upto 25% show CS.1
Underdiagnosis can be attributed to low suspicion in-
dex, variable sensitivity and specificity of diagnostic tests
(Table 1), and lack of an updated consensus for diagnosis.
Clinically, patients of CS have variable manifestations
from asymptomatic state to progressive congestive heart
failure and arrhythmias to sudden death.2
Similarly,
ECG may be normal or may show variable abnormali-
ties: Atrio-ventricular block 26%-62%, Bundle branch
block 12%-61%, Supra-ventricular tachycardia 0%-15%,
Ventricular tachycardia 2%-42%.2
However, ST elevation
suggestive of MI in CS is extremely uncommon. ST el-
evation may be due to abnormal wall motion (including
ventricular aneurysm) and/or myocardial fibrosis. It may
be present during phase of active inflammation in CS, as
it subsided following steroid use in our patient and in one
other case report. In both cases, attenuation of ST segment
elevation was concomitant with alleviation of symptoms.3
Causes for ST elevation other than STEMI include a nor-
mal variant in healthy individuals, acute myocarditis,
hyperkalemia, hypothermia, acute cor pulmonale, pul-
monary embolism, Brugada syndrome, cardiac tumor or
cardiac sarcoidosis.4,5
If there is incoherence between patient’s symptoms and
ECG, and if his condition permits; rapid work up must
be arranged to either diagnose or rule out above entities.
If indeed CS is strongly suspected, confirmatory evidence
must be sought using serum ACE level, echocardiogra-
phy, EMB, and imaging modalities as per merit as out-
38

3. The Indian Practitioner q Vol.69 No.8.August 2016
Case Report
lined in table 1. Use of 67Ga
scintigraphy with MRI, 201Tl,
or 99mTc may assist in better
diagnosis and prognostica-
tion. Cardiac MRI or PET scan
are preferred to others be-
cause of their relatively high
sensitivity and better correla-
tion with clinical disease ac-
tivity.2
Needless to say, coro-
nary angiography is always
valid in appropriate clinical
settings.
Treatment
Corticosteroids suppress
inflammation and granuloma
formation in CS. Longterm
treatment with corticosteroids
is recommended from two
years to lifelong, especially in
patients with severe ventricu-
lar dysfunction. Role of other
immunosuppressants like
methotrexate, azathioprine,
hydroxychloroqine, cyclo-
phosphamide, cyclosporine
A,infliximabispoorlydefined
in CS. Anti-arrhythmics have
an empirical role. Indications
of implantable defibrillators
and heart transplantation are
not yet concrete.2,6
Cardiac involvement in sarcoidosis is of prognostic
value. Early treatment with corticosteroids prevents ir-
reversible damage to the heart and improves prognosis.
The first step is developing a high suspicion index and
appropriately investigating the clinical presentation like
arrhythmias in the young.
References
1. Dubrey SW, Rodney H, Falk. Diagnosis and
Management of Cardiac Sarcoidosis. Progress in
Cardiovascular Diseases. 2010;52(4):336–346.
2. Kim JS, Judson MA, Donnino R, Gold M, et al;
Cardiac sarcoidosis. American Heart Journal. January
Diagnostic modality Sensitivity Specificity Merits
ECG Low Low Detects conduction disturba-
nces
Echocardiography Low to moder-
ate
Low Useful early screening test,
widely available
Thallium 201 (201Tl
and technetium 99m
(99mTc) scintigraphy
Moderate Moderate Improvement of defects related
to CS after exercise or vasodila-
tor infusion, called “reverse dis-
tribution” pattern can be picked
up. It may indicate potential re-
sponsiveness to steroids.
Gallium 67 (67 Ga)
scintigraphy
Low High Detects areas of suspected infla-
mmation related to CS. Useful
in acute phase of CS.
Positron emission
tomography with
18F-fluorodeoxyglucose
(18F-FDG PET)
High Moderate to
high
Provides a measure of both,
disease activity using 18F-FDG
uptake, and of fibrogranuloma-
tous replacement of myocar-
dium using perfusion imaging.
However, radiation exposure
and limited availability is a ma-
jor drawback.
Cardiac MRI Moderate to
high
High Segmental wall motion abnor-
malities or regions of focal wall
thickening /thinning /scarring
can be detected. Easily avail-
able and no added radiation
risk.
Table 1: Diagnostic modality with their sensitivity, specificity and merits while
investigating a case of cardiac sarcoidosis.(2)
2009;157(1):9-21.
3. Iijima K, Chinushi M, Furushima H, Aizawa Y.
Intramural inflammation as a cause of transient ST-
segment elevation in a patient of cardiac sarcoidosis.
Europace 2012;14(2): 300-302.
4. Wang K, Asinger RW, Marriott HJL. ST-Segment
Elevation in Conditions Other Than Acute Myocardial
Infarction. N Engl J Med. 2003;349:2128-35.
5. Goldberger, Ary. Myocardial Infarction:
Electrocardiographic Differential Diagnosis, 4th edi-
tion. Mosby-Year Book, 1991.
6. Deng JC, Baughman RP, Lynch JP. Cardiac
Involvement in Sarcoidosis. Semin Respir Crit Care
Med. 2002;23(6)
X
39