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Role of cardiovascular magnetic resonance imaging in arrhythmogenic right ventricular dysplasia.

Jain A, Tandri H, Calkins H, Bluemke DA - J Cardiovasc Magn Reson (2008)

Bottom Line: It is inherited in an autosomal pattern with variable penetrance.The disease involvement is not limited only to the RV as left ventricle (LV) has also been reportedly affected.Cardiovascular MR is an important non-invasive diagnostic modality that allows both qualitative and quantitative evaluation of RV.

View Article: PubMed Central - HTML - PubMed

Affiliation: Russell H, Morgan Department of Radiology and Radiological Science, Johns Hopkins University, School of Medicine, Baltimore, MD, USA. ajain18@jhmi.edu

ABSTRACT
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.

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Axial bright-blood image from a patient with ARVD showing a thinned lateral wall of the LV (arrow) due to fatty replacement.
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Figure 7: Axial bright-blood image from a patient with ARVD showing a thinned lateral wall of the LV (arrow) due to fatty replacement.

Mentions: LV involvement in classic ARVD is characterized by extension of T-wave inversion in the ECG lateral leads (V5, V6, L1, aVL), arrhythmia of LV or biventricular origin, and/or isolated LV dilatation and impairment [36]. ARVD fatty/fibro-fatty changes of LV myocardium can extend across varying thickness of myocardial circumference, with a predilection for the sub-epicardial and mid-ventricular wall (Figure 7). Fibro-fatty changes can affect both the septum and more often, the LV free wall, either diffusely or regionally with a predilection for postero-septal and postero-lateral areas [77]. Myocardial replacement of LV by adipose or fibro-adipose tissue, like the RV, is believed to progress inwards from the subepicardium to the trabecular myocardium [82].


Role of cardiovascular magnetic resonance imaging in arrhythmogenic right ventricular dysplasia.

Jain A, Tandri H, Calkins H, Bluemke DA - J Cardiovasc Magn Reson (2008)

Axial bright-blood image from a patient with ARVD showing a thinned lateral wall of the LV (arrow) due to fatty replacement.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2483704&req=5

Figure 7: Axial bright-blood image from a patient with ARVD showing a thinned lateral wall of the LV (arrow) due to fatty replacement.
Mentions: LV involvement in classic ARVD is characterized by extension of T-wave inversion in the ECG lateral leads (V5, V6, L1, aVL), arrhythmia of LV or biventricular origin, and/or isolated LV dilatation and impairment [36]. ARVD fatty/fibro-fatty changes of LV myocardium can extend across varying thickness of myocardial circumference, with a predilection for the sub-epicardial and mid-ventricular wall (Figure 7). Fibro-fatty changes can affect both the septum and more often, the LV free wall, either diffusely or regionally with a predilection for postero-septal and postero-lateral areas [77]. Myocardial replacement of LV by adipose or fibro-adipose tissue, like the RV, is believed to progress inwards from the subepicardium to the trabecular myocardium [82].

Bottom Line: It is inherited in an autosomal pattern with variable penetrance.The disease involvement is not limited only to the RV as left ventricle (LV) has also been reportedly affected.Cardiovascular MR is an important non-invasive diagnostic modality that allows both qualitative and quantitative evaluation of RV.

View Article: PubMed Central - HTML - PubMed

Affiliation: Russell H, Morgan Department of Radiology and Radiological Science, Johns Hopkins University, School of Medicine, Baltimore, MD, USA. ajain18@jhmi.edu

ABSTRACT
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.

Show MeSH
Related in: MedlinePlus