Diagnosis of Arrhythmogenic Right Ventricular Dysplasia: A Review

Abstract

Arrhythmogenic right ventricular dysplasia (ARVD) is a myocardial disorder of primarily the right ventricle, with unknown cause and prevalence and with a frequent familial occurrence. The typical clinical manifestation consists of ventricular arrhythmias with a left bundle branch block (LBBB) pattern that occur predominantly in young adults. ARVD may result in sudden death. Other manifestations are electrocardiographic repolarization and depolarization changes, structural abnormalities that range from subtle wall aneurysms within the so-called "triangle of dysplasia" to biventricular regional or global dysfunction, and localized or widespread fibrofatty infiltration of the right ventricular myocardium. The diagnosis of ARVD is based on the presence of major and minor criteria encompassing genetic, electrocardiographic, pathophysiologic, and histopathologic factors. The imaging modalities used to evaluate right ventricular abnormalities include conventional angiography, echocardiography, radionuclide angiography, ultrafast computed tomography, and magnetic resonance (MR) imaging. Among these techniques, MR imaging allows the clearest visualization of the heart. Because MR imaging depicts both functional and structural abnormalities, positive MR imaging findings should be used as important additional criteria in the clinical diagnosis of ARVD. MR imaging appears to be the optimal technique for detection and follow-up of clinically suspected ARVD.

Displasia arritmogênica do ventrículo direito

“…Morphologically, it is characterized by RV myocardial infiltration with fibrofatty tissue 7 . The infiltration usually occurs in the diaphragmatic, apical, and infundibular region -known as the "dysplasia triangle" -and may develop to dilation or aneurysm 2 .…”

“…Some authors have suggested that MRI should be used to replace biopsy 9 . The endpoints that may be identified by MRI are: fibrofatty infiltration in RV with hyperintense signals, fibrofatty infiltration causing diffuse reduction of myocardial thickening, aneurysm or dilation of RV and of RV exit pathway, segmental contraction and global systolic or diastolic dysfunction 7 .…”

“…Pharmacological treatment is the first choice option, through the administration of antiarrhythmics such as sotalol, verapamil, amiodaron, betablockers, propafenone -with variable therapeutic responses 2,7 . Extenuating physical activity should be avoided 2 .…”

“…Suspicious genetic changes have also been identified in chromosomes 14 and 3 7 . This is the description of a clinical case of a young patient with ARVD.…”

Is cardiac MRI an effective test for arrhythmogenic right ventricular cardiomyopathy diagnosis?

“…In 2010, revised criteria were proposed to include quantitative parameters particularly for the imaging studies (Table 2) to improve diagnostic sensitivity whilst maintaining specificity [5] . CMR has an important role in the diagnosis of ARVC as it allows three-dimensional visualisation of the ventricles and is very useful in the assessment of functional and structural abnormalities [23] . Previous studies have demonstrated that cMR has high sensitivity and specificity for ARVC diagnosis [24,25] and also play an important role in the evaluation of ARVC even in patients who do not meet the Task Force Criteria [26] .…”