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(Circulation. 2002;106:e16.)
© 2002 American Heart Association, Inc.

Images in Cardiovascular Medicine

Cardiac Imaging in Isolated Noncompaction of Ventricular Myocardium

Magnus Baumhäkel; Ingrid Janzen, MD; Michael Kindermann, MD; Günther Schneider, MD; Benno Hennen, MD; Michael Böhm, MD

From Medizinische Klinik und Poliklinik, Innere Medizin III (M. Baumhäkel, I.J., M.K., B.H., M. Böhm), and Abteilung fur Radiodiagnostik (G.S.), Universitätskliniken des Saarlandes, Homburg/Saar, Germany.

Correspondence to Magnus Baumhäkel, Universitätskliniken des Saarlandes, Medizinische Klinik und Poliklinik, Innere Medizin III, Kirrbergstr, D-66421 Homburg/Saar, Germany. E-mail magnus{at}baumhaekel.de

A42-year-old man with previously diagnosed dilated cardiomyopathy due to alcohol abuse was referred to our clinic for further diagnostic procedures. Cardiac catheterization (cine loop 1: Figure 1 and Movie I) demonstrated left ventricular dilatation with obviously hypokinetic basal and apical segments, a restrictive filling pattern ("square root sign"), moderate mitralic regurgitation, and no obstruction of the left ventricular outflow tract. Impaired systolic function was confirmed by an ejection fraction of 28%. After injection of a contrast medium, the basal and apical segments showed remarkable opacification, as evidenced by a loosened, spongy myocardium without indication of ventricular blood communication with the epicardial coronary artery system. Along with previously performed echocardiographic imaging, hereditary isolated noncompaction of ventricular myocardium (INVM) was diagnosed.

View larger version (139K): [in this window] [in a new window]   Figure 1. Cine loop 1. Left ventricular angiography, 30° right anterior oblique projection. There is a permanent ventricular demand pacing pacemaker lead in the right ventricle.

Additional screening of the relatives also revealed INVM in an asymptomatic half-sister (age, 40 years). Her echocardiographic imaging showed a regular-sized left chamber (left ventricular end-diastolic diameter, 54 mm) with hypertrophied apical segments. The myocardium appeared loosened, with deep intramyocardial recesses and prominent trabeculations comparable to those seen in her brother’s echocardiography images. Color Doppler imaging of the apical myocardium (cine loop 2: Figure 2 and Movie II) demonstrated blood flow throughout the trabeculations. Fractional shorten-ing was calculated as 22%. Additionally, her cardiac MRI (cine loop 3: Figure 3 and Movie III) showed a typical myocardial appearance of both ventricles in this rare congenital disorder.

View larger version (42K): [in this window] [in a new window]   Figure 2. Cine loop 2. Color Doppler imaging of the left ventricle in an apical 4-chamber view.

View larger version (165K): [in this window] [in a new window]   Figure 3. Cine loop 3. MRI of longitudinal section of the heart (cine true fast imaging with steady-state precision).

In conclusion, diagnosis of INVM is made mainly on the basis of sufficient cardiac imaging, including echocardiographic imaging, catheterization, and MRI or CT.

Footnotes

Movies I, II, and III are available in an online-only Data Supplement at http://www.circulationaha.org.

The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.

Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke’s Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.

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This Article Full Text (PDF) Data Supplement Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Baumhäkel, M. Articles by Böhm, M. Search for Related Content PubMed PubMed Citation Articles by Baumhäkel, M. Articles by Böhm, M. Related Collections Other heart failure Cardiovascular imaging agents/Techniques CT and MRI Echocardiography