Assessment of Right Ventricular Dysplasia

Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/145229/1/cpmia1002.pd

Role of cardiovascular magnetic resonance imaging in arrhythmogenic right ventricular dysplasia

Arrhythmogenic right ventricular dysplasia (ARVD) is a genetic cardiomyopathy characterized clinically by ventricular arrhythmias and progressive right ventricular (RV) dysfunction. The histopathologic hallmark is fibro-fatty replacement of RV myocardium. It is inherited in an autosomal pattern with variable penetrance. ARVD is unique in that it most commonly presents in young, otherwise healthy and highly athletic individuals. The cause of ARVD is not well-known but recent evidence suggests strongly that it is a disease of desmosomal dysfunction. The disease involvement is not limited only to the RV as left ventricle (LV) has also been reportedly affected. Diagnosis of ARVD is challenging and is currently based upon a multi-disciplinary work-up of the patient as defined by the Task Force. Currently, implanted cardioverter defibrillators (ICD) are routinely used to prevent sudden death in patients with ARVD. Cardiovascular MR is an important non-invasive diagnostic modality that allows both qualitative and quantitative evaluation of RV. This article reviews the genetics of ARVD, current status and role of CMR in the diagnosis of ARVD and LV involvement in ARVD

Obesity and right ventricular structure and function: the MESA-RV study

Introduction: The relationship between obesity and right ventricle (RV) has been inadequately studied due to difficulty in imaging its more complex geometry by echocardiography. While obesity associated RV diastolic dysfunction has been shown, inconclusive data exists regarding systolic function. We aimed to determine the association between obesity and RV structure and function by cardiac magnetic resonance imaging (MRI) in a large multi-ethnic cohort. Purpose: We hypothesized that obesity would be associated with greater RV mass, larger RV end-diastolic volume (EDV), and lower RV ejection fraction (EF). Methods: Cardiac MRIs were analyzed from 1973 participants in the Multi-Ethnic Study of Atherosclerosis, which included individuals aged 45-84 years without clinical cardiovascular disease. Participants were divided into 3 categories based on BMI: normal (18.5-24.9 kg/m2), overweight (25-29.9 kg/m2) and obese (= 30 kg/m2). Associations with RV measures were determined using multivariable regression. Results: The mean age was 62 ± 10 years, 47% were men. 43% were white, 28% African American, 20% Hispanic, and 9% Asian. In multivariable analyses adjusted for age, ethnicity, gender, cardiovascular risk factors and height, obesity was associated with higher RV mass, larger RVEDV (3.1 g/17% higher & 22.7 ml/20% higher respectively, p < 0.0001) and lower RVEF, mass/EDV ratio (-0.9%, p < 0.05; -8.1 mg/ml, p < 0.0001) as compared to normal BMI category participants. These findings persisted after adjusting for the respective left ventricle (LV) parameter. Within each BMI category, RV mass and EDV were positively associated with BMI while mass/EDV was negatively associated with BMI only in the normal BMI category Figures 1 and 2. Figure 1 Figure 2 Conclusion: In a cohort free of clinical cardiovascular disease, obesity was significantly associated with higher RV mass, RVEDV and lower RVEF even after adjustment for the LV. Future studies should examine the mechanism of this effect on the RV