Myocardial delayed enhancement by magnetic resonance imaging in a patient with Duchenne muscular dystrophy and ventricular tachycardia

Abstract

Received 17 December 2012; revision accepted for publication 27 February 2013. Cardiomyopathy is a significant component in Duchenne muscular dystrophy (DMD), and it is not necessarily related to the degree of skeletal myopathy. Cardiac death can result from progressive heart failure, due to ventricular dysfunction, or from sudden death, presumably caused by heart block or malignant arrhythmias. Although electrical abnormalities may occur in the absence of overt cardiac histopathology, electrical and functional abnormalities have been attributed to cardiac fibrosis1. As a representative example of this, we present the cardiovascular magnetic resonance (CMR) finding in a 19-year-old patient with DMD. Myocardial delayed enhancement showed multifocal fibrosis in the septal and lateral wall of the mid left ventricle. This patient had presented several syncopes and spells, with electrocardiographic documentation of repetitive episodes of ventricular tachycardia (VT). The ECG shows a monomorphic wide QRS tachycardia (240 bpm), with RBBB morphology, inferior axis and QS complex in lead I and aVL, suggestive of a basal lateral LV exit site, with, possibly, epicardial origin, compatible with the CMR findings mentioned above. Baseline ECG was representative of cardiac involvement, showing a short PR interval, R/S ratio > 1 in lead V1 V2 and Q waves in the lateral leads2. Myocardial delayed enhancement by magnetic resonance imaging in a patient with Duchenne muscular dystrophy and ventricular tachycardia