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European Heart Journal Advance Access originally published online on April 19, 2006
European Heart Journal 2006 27(21):2510; doi:10.1093/eurheartj/ehi874
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© The European Society of Cardiology 2006. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org

ARVC with left ventricular involvement in a young woman

Thomas Thouet1,*, Julia J. Krueger2,3, Ingo Paetsch1, Sebastian Kelle1 and Eike Nagel1

1 Department of Internal Medicine/Cardiolgy, German Heart Institute Berlin, Augustenburger Platz 1, 13353 Berlin, Germany
2 Department of Congenital Heart Disease, German Heart Institute Berlin, Berlin, Germany
3 Department of Pediatric Cardiology, German Heart Institute Berlin, Berlin, Germany

* Corresponding author. Tel: +49 30 45932400; fax: +49 30 45932458. E-mail address: thouet{at}dhzb.de

A 22-year-old patient was sent to our institution with recurrent palpitations and echocardiographic findings of an isolated right ventricular (RV) dilation. Holter-ECG demonstrated frequent, polymorphic ventricular ectopia and an asymptomatic non-sustained ventricular tachycardia. Twelve-lead ECG showed inverted T-waves in right precordial leads. Cardiac MR imaging revealed pronounced thinning of the dilated RV myocardium and regional akinesia of the basal and equatorial RV free wall with a global RV function of 25%. No direct visualization of intramyocardial fatty tissue was possible despite high-resolution MRI most likely due to the thinning of the free wall. However, fibrofatty tissue was found in the inferolateral wall of the left ventricle (LV) using a series of T1 (Panel A) and T2 weighted images with and without fat saturation pre-pulse (Panel B) and an inversion recovery technique 15 min after Gd-DTPA injection (Panel C). Hypoperfusion during dynamic perfusion imaging could be observed (Panel D).

We diagnosed an arrhythmogenic right ventricular cardiomyopathy (ARVC) with left ventricular involvement according to the modified McKenna criteria. The present case shows the necessity of a vigilant and detailed observation of the LV myocardium in patients with suspected ARVC, especially because histopathological studies have shown LV involvement in 76% of patients.

Panel A. The T1-weighted blackblood images revealed two localized areas of hyperintense signal in the inferolateral LV-wall.

Panel B. Using a T1-weighted blackblood sequence with fat saturation prepulse (STIR) the hyperintense signal in the LV wall was suppressed and identified as fatty tissue.

Panel C. 15 minutes after administration of Gd-DTPA (0.2 mmol/kg/bodyweight) fibrous tissue with hyperintnese signal in both areas could be detected using an inversion recovery technique.

Panel D. During injection of Gd-DTPA, hypoperfusion of both areas could be observed using a balanced turbo field echo imaging sequence dedicated for dynamic first pass perfusion.

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This Article
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27/21/2510    most recent
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