The Indian Practitioner q Vol.69 No.8. August 2016

Case Report

Introduction

Sarcoidosis 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 - A Second thought

ABSTRACTCardiac 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 ReportA 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 - MedicineCorresponding author: Adukia S A - Senior Resident, Bharati Hospital And Research Center, Katraj, Dhankawadi,

Pune-Satara Road, Pune-411043

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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.

DiscussionSarcoidosis 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-

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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.

TreatmentCorticosteroids 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, infliximab is poorly defined 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.

References1. 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)

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