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(Circulation. 2007;115:e412-e414.)
© 2007 American Heart Association, Inc.

Images in Cardiovascular Medicine

Magnetic Resonance Imaging Guiding Pacemaker Implantation for Severe Sinus Node Dysfunction Due to Cardiac Involvement in Erdheim-Chester Disease

Thomas Elgeti, MD; Michael Schlegl, MD; Aischa Nitardy; Dietmar E. Kivelitz, MD; Martin Stockburger, MD

From the Department of Radiology (T.E., D.E.K.), the Department of Cardiology (A.N., M. Stockburger), Charité-Universitäetsmedizin Berlin, and Kardiologische Praxis Westend (M. Schlegl), Berlin, Germany.

Correspondence to Dr Thomas Elgeti, Department of Radiology, Charité- Universitätsmedzin Berlin, Charitéplatz 1, 10117 Berlin, Germany. E-mail thomas.elgeti@charite.de

An extract of the first 250 words of the full text is provided, because this article has no abstract.

A 64-year-old woman was referred to the arrhythmia outpatient clinic after she had experienced syncope without preceding symptoms and frequent paroxysmal, near syncopal episodes over the last 8 months. The ECG revealed alternation of sinus bradycardia and frequent ectopic atrial beats, normal PR interval and right bundle branch block with consecutive repolarization abnormalities. Holter ECG showed frequent periods of asystole up to 4.3 seconds. Laboratory findings were normal. Pacemaker therapy for symptomatic sinus node dysfunction was clearly indicated.

The patient’s past history revealed the diagnosis of Erdheim-Chester disease with osseous, cutaneous, mesenteric, and right atrial involvement 22 years ago. This disease belongs to a rare group of non-Langerhans cell histiocytosis of unknown origin. Tissue is infiltrated by foamy histiocytes.¹ The patient showed nearly pathognomonic radiographic changes in both tibiae and femora with bilateral symmetrical osteosclerosis of metaphyseal and diaphyseal regions with sparing of the epiphyses (Figure 1A).²

View larger version (28K):   Figure 1. A, X-rays of both knees showing bilateral symmetric osteosclerosis of metaphysis with sparing of the epiphysis. B, echocardiography from an atypical apical view showing thickening of the right atrial wall (see measurements) and sparing of the lateral wall.

Over the last 5 years the patient has been stable through daily oral administration of 5 mg prednisolone.

Cardiac magnetic resonance imaging was performed to guide placement of the pacemaker electrodes and visualize the overall and local extent of tumor due to the known right atrial involvement by Erdheim-Chester disease. Echocardiographic findings are shown in Figure 1B.

Nearly complete and . . . [Full Text of this Article]

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