ARVC-致心律失常性右室心肌病

ARVC/D is a rare genetic cardiomyopathy characterized by structural abnormalities of the right ventricle and ventricular arrhythmias. In ARVC/D there is replacement of right ventricular myocardium with fatty and fibrous tissue, which leads to progressive RV failure. The most common area of tissue change is the triangle of dysplasia which consists of the inferior-sub tricuspid area RV apex and the RV infundibulum . The disease presents between the second and fifth decades of life either with symptoms of palpitations and/or syncope associated with ventricular tachycardia or with SCD. The prevalence of ARVC/D is relatively uncommon but may account for up to 20 percent of cases of sudden death among young individuals and is an important cause of sudden cardiac death in young athletes. This picture shows the most common area of tissue change of ARVC/D, it’s the triangle of dysplasia which consists of the inferior-sub tricuspid area RV apex and the RV infundibulum. The diagnosis of ARVC/D relies on the demonstration of structural, functional, and electrophysiological abnormalities, and Family medical history.

Technical advances in MRI have improved the capability to image the RV with reproducible measurements of volume and systolic function, which permits classification of severity and differentiation from normality. Previous diagnostic reliance on subjective assessment of RV wall thinning, wall motion abnormalities, and fatty infiltration of the myocardium by MRI.

There also have been recent developments to quantify the extent of RV wall motion abnormalities by angiography with

computer-based analysis, as well as to determine RV volumes. In addition, commercial software is available to determine RV volumes and ejection fraction. The RV angiogram obtained in multiple views is considered to be a reliable imaging test to assess wall motion abnormalities.

So MRI change of ARVC/D include Functional and Morphologic Abnormalities.

The pictures show the end diastolic and systolic frames of a short axis cine MRI. There is an area of dyskinesia in the right ventricular free wall due to a focal aneurysm.

This is a Axial black blood image from a patient with ARVC/D , showing enlarged and dysmorphic outflow tract with focal bulging anteriorly .

This is a Axial black blood image from a patient with ARCV/D showing lack of demarcation between epicardial fat and myocardium .

This is a Fat suppressed axial black blood image from a patient with ARVC/D showing focal abrupt thinning of the anterior wall of the RV.

At last let’s see a case of suspect of ARVC in our department. This is a 57 years old man, with pulmonary hypertension 8 years. ECG show aurial fibrillation and inverted T waves.

This picture show the right ventricular enlargement. These two pictures show the right ventricular outflow tract （RVOT） widen.

This picture show fatty infiltration at the RV apex and the RV infundibulum.

This film show there is an area of dyskinesia in the right ventricular apex.