Arrhythmogenic cardiomyopathy with left ventricle involvement/arrhythmogenic left ventricular cardiomyopathy: a clinical and MRI study

Abstract

Objective To investigate the characteristics of clinical and cardiovascular magnetic resonance imaging(CMR) of arrhythmogenic cardiomyopathy with left ventricular(ALVC) involvement. Methods Ten cases of arrhythmogenic cardiomyopathy with left ventricular involvement and sixteen randomly chosen cases of arrhythmogenic cardiomyopathy with right ventricular (ARVC) involvement were enrolled in this study. Clinical symptoms, cardiac electrophysiological changes as well as the cardiac morpharage, ventricular functions and delayed enhancement of myocardium assessed by CMR were compared between the two groups. The size of heart chambers, global ventricular functions and the fat/fibrosis infiltration were evaluated by turbo fast spin echo, ture FISP cine and delayed enhancement. These were statistically analyzed by independent samples t test, respectively. Results There was no significant difference in age and gender between two groups. All the patients in two groups presented non-specific clinical manifestations with no significant differences (P>0.05) except for short of breath(P=0.034). The end-diastolic diameter of left ventricular, left ventricular end-diastolic volume index, left and right ejection fraction in ALVC and ARVC group were (64.2±7.7), (49.2±5.9) mm (t=5.551 , P<0.001), (113.9±24.0), (69.2± 30.0) ml/m2 (t=3.962, P<0.001), (38.5±3.1)%, (56.0±8.4)%(t= -6.733,P<0.001), (42.0±5.5) %, (18.0±7.3)% (t=8.817, P<0.001) respectively. An medium of 11 and 14 segments of fat or fat/fibrosis were found in intramural wall of the LV myocardiumin patients with ALVC,while only 0 segment(all P<0.001)was found in patients with ARVC. Conclusions The clinical manifestations of arrhythmogenic cardiomyopathy with left ventricular involvement was similar to ARVC, however, with regarding to cardiac morphological, functional and myocardial lesions, these two diseases have different characteristics in CMR features.one-stop-shop MRI examination has high value in the diagnosis of arrhythmogenic cardiomyopathy with Left ventricular involvement. Key words: Ventricular dysfunction, left; Arrhythmogenic right ventricular dysplasia; Magnetic resonance imaging