Cardiac Magnetic Resonance for Arrhythmogenic Right Ventricular Cardiomyopathy | Journal Scan

Study Questions:

What is the contribution of cardiac magnetic resonance (CMR) to the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) using the revised Task Force Criteria (rTFC) in children and adolescents?

Methods:

A retrospective review was performed of existing CMR data obtained from patients evaluated for ARVC between 2005 and 2009. Using the rTFC, patients were categorized into “definitive,” “borderline,” “possible,” or “no” ARVC diagnostic groups. A c-statistic model was used to determine the extent to which each element of the rTFC contributed to the diagnosis of ARVC.

Results:

A total of 213 patients were referred for first-time imaging evaluation for ARVC during the study period. Of these studies, 71 were excluded for various reasons, including lack of complete CMR data, concomitant congenital heart disease, and unavailable rTFC components. Applying the rTFC to the remaining 142 patients, 23 (16%) had definite, 32 (23%) borderline, 37 (26%) possible, and 50 (35%) no ARVC. RV end-diastolic volumes ranged from 92 ± 18 cc/m2 for those with no ARVC to 118 ± 31 cc/m2 for those with definite ARVC. The prevalence of regional wall motion abnormalities ranged from 16% in those with no ARVC, to 83% in those with ARVC by rTFC. RV fatty infiltration was detected in one patient, while RV fibrosis was detected in three patients, all of whom had definitive ARVC. Of the rTFC major criteria, CMR had the largest c-statistic decline (c -0.163). Eleven of the 23 patients with definitive ARVC would not have met criteria to be in this group without CMR.

Conclusions:

CMR data contribute significantly to the diagnosis of ARVC in children using the rTFC. Fatty infiltration and myocardial fibrosis provide limited value in the pediatric population.

Perspective:

Diagnosis of ARVC can be a challenge, both in adults and children. This study assessed the role of CMR in the diagnosis of ARVC in the pediatric population. CMR-derived data including RV volumes and the presence of regional wall motion abnormalities proved important in the diagnosis of ARVC. Intra- and inter-rate reproducibility was only moderate for the assessment of wall motion abnormalities in this study. The primary limitation is lack of an independent gold standard for the diagnosis of ARVC, as MRI criteria are inherently a part of the criteria. Interestingly, fatty infiltration and myocardial fibrosis were not important contributors to the diagnosis of ARVC in children.

Clinical Topics: Arrhythmias and Clinical EP, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Genetic Arrhythmic Conditions, SCD/Ventricular Arrhythmias, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Imaging, Magnetic Resonance Imaging

Keywords: Arrhythmogenic Right Ventricular Dysplasia, Cardiomyopathies, Magnetic Resonance Imaging, Diagnostic Imaging, Magnetic Resonance Spectroscopy, Heart Defects, Congenital, Child, Adolescent, Fibrosis


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