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Right Ventricular MR Abnormalities in Myotonic Dystrophy and Relationship with Intracardiac Electrophysiologic Test Findings: Initial Results¹

¹ From the Departments of Radiology (O.V., P. Legmann), Cardiology (J.V., D.D., S.W.), and Biostatistics (J.C.), Université René Descartes, Hôpital Cochin, 27 rue du Fg Saint Jacques, 75014 Paris, France; InParys, St Cloud, France (A.L.); Service Hospitalier Frederic Joliot, Orsay, France (P.C.); General Electric Medical Systems, Buc, France (C.A.); and Myology Institute, Hôpital Salpétrière, Paris, France (P.C., P. Laforet). Supported by an AFM grant. Received June 1, 2001; revision requested July 12; revision received September 24; accepted November 12. Address correspondence to O.V. (e-mail: olivier.vignaux@cch.ap-hop-paris.fr).

View larger version (127K): [in a new window]   Figure 1. Transverse MR image obtained in a 29-year-old inducible patient with myotonic dystrophy shows a focal area of increased signal intensity due to fatty infiltration in the RV free wall (arrow). ECG-triggered T1-weighted non-breath-hold spin-echo sequence: 740/30; matrix, 192 x 256; section thickness, 5 mm; four signals acquired; imaging time, 5 minutes 25 seconds.

View larger version (120K): [in a new window]   Figure 2a. Transverse MR images obtained in an inducible 49-year-old patient with myotonic dystrophy. (a) Conventional T1-weighted non-breath-hold spin-echo MR image shows myocardial thinning of the RV free wall. ECG-triggered T1-weighted spin-echo sequence: 710/30; matrix, 192 x 256; section thickness, 5 mm; four signals acquired; imaging time, 4 minutes 54 seconds. (b) Image obtained with a gradient-echo breath-hold double-angulated left ventricular long-axis sequence (systolic phase) shows focal dyskinesis of the RV free wall (arrows). Segmented k-space gradient-echo sequence: 5.6/3.6; matrix, 128 x 256; flip angle, 20°; section thickness, 10 mm; number of views per segment, six; imaging time, 18 seconds (one section per breath hold).

View larger version (127K): [in a new window]   Figure 2b. Transverse MR images obtained in an inducible 49-year-old patient with myotonic dystrophy. (a) Conventional T1-weighted non-breath-hold spin-echo MR image shows myocardial thinning of the RV free wall. ECG-triggered T1-weighted spin-echo sequence: 710/30; matrix, 192 x 256; section thickness, 5 mm; four signals acquired; imaging time, 4 minutes 54 seconds. (b) Image obtained with a gradient-echo breath-hold double-angulated left ventricular long-axis sequence (systolic phase) shows focal dyskinesis of the RV free wall (arrows). Segmented k-space gradient-echo sequence: 5.6/3.6; matrix, 128 x 256; flip angle, 20°; section thickness, 10 mm; number of views per segment, six; imaging time, 18 seconds (one section per breath hold).