[The arrhythmogenic right ventricular cardiomyopathy (ARVC)]

The following report illustrates the case of a 40-year old patient, who was transferred to our hospital because of a symptomatic sustained ventricular tachycardia (VT). The documented VT showed typical left bundle branch block morphology with an inferior axis, which was accompanied by repolarization abnormalities in the right precordial leads, as revealed by the surface ECG. While coronary angiography allowed the exclusion of an obstructive coronary heart disease and confirmed an intact left ventricular function, the right ventricular angiography as well as the MRI demonstrated not only reduced right ventricular function but also regional abnormalities of contraction in the inferior and diaphragmal regions and typical "outpouchings" and "bulgings". The MRI also provided evidence for sporadic fatty infiltrations of the right ventricle. In the electrophysiological study, sustained VT could be reproducibly induced, while showing the typical RVOT configuration, which corresponded to the clinically apparent VT. Since these examination results matched with the criteria of McKenna et al., arrhythmogenic right ventricular cardiomyopathy was diagnosed. The patient underwent implantation of a cardioverter-defibrillator (ICD) and medical treatment with metoprolol. This particular case report was selected because it shows a typical presentation of an ARVC. Furthermore, the present example offers the possibility of demonstrating that in the case of an ARVC in the regions of the right ventricle, arrhythmogenic areals can also be found which seem morphologically inconspicuous.