5 key points
- The hotspot region in ARVC is the subtricuspid region (basal segment). This is the most and earliest affected RV region.
- Regional deformation characteristics are: 1) reduction of peak systolic strain below -18%, 2) a prolonged time to onset shortening >100 ms, and/or 3) post-systolic shortening >15%.
- RV mechanical dispersion ≥37 ms (SD of regional timing peak shortening) is associated to ventricular arrhythmias.
- Left ventricular involvement is most often seen in the posterolateral region.
- Normal deformation imaging findings does not completely rule out this disease. However, deformation abnormalities might precede conventional abnormalities
Cases
References:
- Mast TP, Teske AJ, Walmsley J, van der Heijden JF, van Es R, Prinzen FW, Delhaas T, van Veen TA, Loh P, Doevendans PA, Cramer MJ, Lumens J. Right Ventricular Imaging and Computer Simulation for Electromechanical Substrate Characterization in Arrhythmogenic Right Ventricular Cardiomyopathy. J Am Coll Cardiol. 2016 Nov 15;68(20):2185-2197. PMID: 27855808
- Leren IS, Saberniak J, Haland TF, Edvardsen T, Haugaa KH.Combination of ECG and Echocardiography for Identification of Arrhythmic Events in Early ARVC. JACC Cardiovasc Imaging. 2016 Oct 14. PMID: 27771401
- Mast TP, Teske AJ, Te Riele AS, Groeneweg JA, van der Heijden JF, Velthuis BK, Loh P, Doevendans PA, VAN Veen TA, Dooijes D, DE Bakker JM, Hauer RN, Cramer MJ. Prolonged Electromechanical Interval Unmasks Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy in the Subclinical Stage. J Cardiovasc Electrophysiol. 2016 Mar;27(3):303-14. PMID: 26585103. 1
- Mast TP, Teske AJ, vd Heijden JF, Groeneweg JA, Te Riele AS, Velthuis BK, Hauer RN, Doevendans PA, Cramer MJ. Left Ventricular Involvement in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Assessed by Echocardiography Predicts Adverse Clinical Outcome. J Am Soc Echocardiogr. 2015 Sep;28(9):1103-13.e9. PMID: 26025724. 2
- Mast TP, Teske AJ, Doevendans PA, Cramer MJ. Current and future role of echocardiography in arrhythmogenic right ventricular dysplasia/cardiomyopathy. Cardiol J. 2015;22(4):362-74. PMID: 25786767. 3
- Teske AJ, Cox MG, Te Riele AS, De Boeck BW, Doevendans PA, Hauer RN, Cramer MJ. Early detection of regional functional abnormalities in asymptomatic ARVD/C gene carriers. J Am Soc Echocardiogr. 2012 Sep;25(9):997-1006. PMID: 22727198. 4
- Teske AJ, Cox MG, De Boeck BW, Doevendans PA, Hauer RN, Cramer MJ. Echocardiographic tissue deformation imaging quantifies abnormal regional right ventricular function in arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Am Soc Echocardiogr. 2009 Aug;22(8):920-7. 19553080. 5