Multimodality Imaging in Arrhythmogenic Right Ventricular Cardiomyopathy

Abstract

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare, heritable myocardial disease associated with the development of ventricular arrhythmias, heart failure, and sudden cardiac death in early adulthood. Multimodality imaging is a central component in the diagnosis and evaluation of ARVC. Diagnostic criteria established by an international task force in 2010 include noninvasive parameters from echocardiography and cardiac magnetic resonance imaging. These criteria identify right ventricular structural abnormalities, chamber and outflow tract dilation, and reduced right ventricular function as features of ARVC. Echocardiography is a widely available and cost-effective technique, and it is often selected for initial evaluation. Beyond fulfillment of diagnostic criteria, features such as abnormal tricuspid annular plane excursion, increased right ventricular basal diameter, and abnormal strain patterns have been described. 3-dimensional echocardiography may also expand opportunities for structural and functional assessment of ARVC. Cardiac magnetic resonance has the ability to assess morphological and functional cardiac features of ARVC and is also a core modality in evaluation, however, tissue characterization of the right ventricle is limited by spatial resolution and low specificity for detection of pathological changes. Nonetheless, the ability of cardiac magnetic resonance to identify left ventricular involvement, offer high negative predictive value, and provide a reproducible structural evaluation of the right ventricle enhance the ability and scope of the modality. In this review, the prognostic significance of multimodality imaging is outlined, including the supplemental value of multidetector computed tomography and nuclear imaging. Strengths and weaknesses of imaging techniques, as well as future direction of multimodality assessment, are also described.

Undiagnosed cardiomyopathy: An incidental finding in a recreational soccer player—A case report and literature review

“…RV factional area change (FAC) has limitations, although it is a useful indictor of global RV function 4,18,19,23,26,27 . A value of ≤30% has been demonstrated in ARVC patients with high specificity 22,23 .…”

“…[21][22][23][24] RV factional area change (FAC) has limitations, although it is a useful indictor of global RV function. 4,18,19,23,26,27 A value of ≤30% has been demonstrated in ARVC patients with high specificity. 22,23 In advanced disease, reduced RV FAC and TAPSE are strong ECHO predictors of arrhythmic risk and adverse outcomes.…”

Case report: Multiple biventricular aneurysms in arrhythmogenic cardiomyopathy

“…Although echocardiography is the most commonly used imaging technique, cardiac magnetic resonance (CMR) is the preferred initial test for patients with suspected ARVC due to its accuracy, availability, safety, and low cost ( 7 ). Among the multimodality imaging of the ACM, late gadolinium enhancement on CMR is accepted as a more objective indicator of myocardial fibrosis.…”

Role of Imaging in Cardiomyopathies

“…First, the diagnostic criteria have been mostly validated on adult subjects with well-differentiated phenotypes, therefore, their diagnostic performance at a younger age may be lower. 125 Second, echocardiography is the preferred tool for screening, but is unable to identify subtler structural abnormalities, so it currently remains to be defined which methods and timing are the most appropriate for family screening of inherited CMPs. For example, the 2014 ESC guidelines on the diagnosis and management of HCM recommend clinical evaluation with ECG and echocardiography and long-term follow-up in first-degree relatives who have the same defined pathological mutation as the proband; when no definite genetic mutation is identified in the proband, ECG and echocardiography should be considered in first-degree adult relatives and repeated every 2-5 years (or 6-12 months if nondiagnostic abnormalities are present).…”