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Biventricular involvement in cardiac sarcoidosis
  1. Y Nakada,
  2. T Date,
  3. S Mochizuki

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A 50 year old woman was admitted to our department to undergo an assessment of recurrent wide QRS tachycardia. An electrophysiologic study was performed. Ventricular tachycardia (VT) of three different morphologies, with left and right bundle branch block configurations, were induced with double extrastimuli from the right ventricle (left panel), thus suggesting that arrhythmogenic substrates existed in both the right and left ventricles. Endocardial mapping study showed that the site of earliest activation was found around the lower right ventricular outflow tract in VT 2. A thallium-scintigram showed a low uptake of agent from the anterior wall to the basal septum wall (panel A). Gadolinium enhanced magnetic resonance imaging (MRI) (T1) showed myocardial hyperenhancement in not only the left ventricular anteroseptum but also in the right ventricle (panel C). Non-caseating epithelioid granuloma with multinuclear giant cells and intracytoplasmic inclusions were detected in a right ventricular endomyocardial biopsy (panel B). We diagnosed cardiac sarcoidosis and administered prednisolone acetate and amiodarone. Recent reports have demonstrated cardiac sarcoidosis to affect not only the left ventricle but also the right ventricle. Although thallium-201 myocardial scintigrams have been proposed as a non-invasive diagnostic tool for detecting cardiac sarcoidosis, detecting abnormalities in the right ventricle using thallium-201 scintigraphy tends to be difficult. In the present patient, gadolinium enhanced MRI showed myocardial hyperenhancement in the culprit lesion of the VT in the right ventricle, as demonstrated by the electrophysiological study, thus suggesting that gadolinium enhanced MRI is a useful tool for detecting cardiac sarcoidosis involving both left and right ventricular infiltration.

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ECG at baseline (SR) and during the tachycardia (VT 1–3), revealing a first degree atrioventricular (AV) block with sinus rhythm and ventricular tachycardias of three different morphologies with left and right bundle branch block configurations, respectively. VT, ventricular tachycardia; SR, sinus rhythm.

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Panel A: rest myocardial thallium-201 scan (short axis) showing a clear anteroseptal defect. Panel B: biopsy specimen of endocardial tissue revealing many non-caseating granulomas with asteroid giant cells. Panel C: magnetic resonance imaging examination with a short axis view (gadolinium-DTPA delayed enhanced T1 weighted image) showing myocardial hyperenhancement in the anteroseptal wall of the left ventricle and part of the right ventricle wall.