Korean Circ J.  2012 Aug;42(8):571-574. 10.4070/kcj.2012.42.8.571.

Cardiac Sarcoidosis Presenting With Complete Atrioventricular Block and Sustained Monomorphic Ventricular Tachycardia

• Lee JM
• Oh IY
• Choi DJ

• KMID: 2225010
• DOI: http://doi.org/10.4070/kcj.2012.42.8.571

Abstract

Sarcoidosis is a rare but potentially fatal multisystem granulomatous disease of unknown etiology. While a number of clinical manifestations may develop, cardiac involvement (prior to or coincident with sarcoidosis of other organs) is an important prognostic factor. Recently, we encountered a patient with cardiac sarcoidosis who presented with complete atrioventricular (AV) block and sustained ventricular tachycardia. An implantable cardioverter-defibrillator was inserted as a precautionary measure for ventricular tachycardia and symptomatic complete AV block. 18F-fluoro-2-deoxyglucose positron emission tomography confirmed a dramatic response to high-dose steroid at four weeks, as demonstrated by a marked decrease in cardiac sarcoid activity from baseline status.

Keyword

Sarcoidosis; Heart failure; Magentic resonance imaging; Tachycardia, ventricular

MeSH Terms

Atrioventricular Block
Defibrillators, Implantable
Heart Failure
Humans
Positron-Emission Tomography
Sarcoidosis
Tachycardia, Ventricular

Figure

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  Fig. 1 Initial presentation of the patients. A: admission electrocardiogram: complete atrioventricular block (heart rate, 27 beats per minute). B: echocardiogram: RV and right atrium enlargement and severe RV dysfunction, without pulmonary hypertension (pulmonary artery systolic pressure, 27.64 mm Hg). C: chest X-ray: mild cardiomegaly (cardiothoracic ratio 55%); no hilar lymph node enlargement. D: Gadolinium-dietylene triamine penta-acetic acid-enhanced cardiac MRI: delayed hyper-enhancement of the entire RV wall and subepicardial (apical) or transmural (apical, basal) portions of the left ventricle. E: sustained ventricular tachycardia during endomyocardial biopsy. RV: right ventricle.

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  Fig. 2 Pathologic finding of lymph nodes and results of initial and follow-up 18F-FDG-PET. A and B: right paratracheal (17.27×8.35 mm) and subcarinal (18.63×11.97 mm) lymph node enlargement discovered incidentally in coronary CT angiography six months prior. C and D: lymph node aspirate: noncaseating epitheloid granulomata with giant cells. E and F: baseline 18F-FDG-PET: inhomogeneous uptake, accentuated in left ventricular myocardium {maximum standardized uptake value (SUVMAX)=7.9 g/mL}. G and H: 18F-FDG-PET at one-month follow-up; marked decrease in myocardial hypermetabolism (SUVMAX=3.1 g/mL). 18F-FDG-PET: 18F-fluoro-2-deoxyglucose positron emission tomography.

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