Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is cumbersome. Stress or exercise imaging findings are not specifically included in the task force guidelines as major or minor criteria ; however, exercise plays a major role in right ventricular (RV) physiology and pathophysiology. Authentic ARVD/C gene carriers may demonstrate symptoms and signs that overlap substantially with those in subjects who have other reasons for RV impairment, including strenuous endurance training. However, even borderline forms (gene elusive) of ARVD/C carry a potentially poor prognosis, including risk for sudden death. Furthermore in patients with ARVD/C, the amount and intensity of exercise have been reported to be associated with decreased RV function and increased ventricular arrhythmias. Several studies have shown that the right ventricle is more prone to exercise-induced impairment than the left ventricle in athletes, and such RV alterations have been associated with ventricular arrhythmias.
When performing stress echocardiographic studies, physicians should focus on regional and global RV impairment, especially in patients being evaluated for ventricular arrhythmias or for possible ARVD/C. RV strain using speckle-tracking imaging, RV ejection fraction, and RV annular peak systolic velocity have moderate to good accuracy in discriminating patients with ARVD/C from healthy adults. A recent meta-analysis of the echocardiographic assessment of ARVD/C is available. Thus, stress echocardiography may play a role in the initial diagnosis of ARVD/C and for serial evaluation in at-risk first-degree adult relatives of patients with ARVD/C. Will stress or exercise imaging findings be part of future task force criteria? More studies are needed to assess the safety and accuracy of stress echocardiography (multimodal using several or other imaging modalities, including stress cardiac magnetic resonance) for the diagnosis of ARVD/C.
References
- 1. Marcus F.I., McKenna W.J., Sherrill D., Basso C., Bauce B., Bluemke D.A., et al: Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation 2010; 121: pp. 1533-1541
- 2. La Gerche A., Claessen G., Dymarkowski S., Voigt J.-U., De Buck F., Vanhees L., et al: Exercise-induced right ventricular dysfunction is associated with ventricular arrhythmias in endurance athletes. Eur Heart J 2015; 36: pp. 1998-2010
- 3. Oxborough D., Sharma S., Shave R., Whyte G., Birch K., Artis N., et al: The right ventricle of the endurance athlete: the relationship between morphology and deformation. J Am Soc Echocardiogr 2012; 25: pp. 263-271
- 4. Zaidi A., Sheikh N., Jongman J.K., Gati S., Panoulas V.F., Carr-White G., et al: Clinical differentiation between physiological remodeling and arrhythmogenic right ventricular cardiomyopathy in athletes with marked electrocardiographic repolarization anomalies. J Am Coll Cardiol 2015; 65: pp. 2702-2711
- 5. Sawant A.C., Bhonsale A., te Riele A.S.J.M., Tichnell C., Murray B., Russell S.D., et al: Exercise has a disproportionate role in the pathogenesis of arrhythmogenic right ventricular dysplasia/cardiomyopathy in patients without desmosomal mutations. J Am Heart Assoc 2014; 3: pp. e001471
- 6. Krahn A.D., Healey J.S., Chauhan V., Birnie D.H., Simpson C.S., Champagne J., et al: Systematic assessment of patients with unexplained cardiac arrest: Cardiac Arrest Survivors With Preserved Ejection Fraction Registry (CASPER). Circulation 2009; 120: pp. 278-285
- 7. Saberniak J., Hasselberg N.E., Borgquist R., Platonov P.G., Sarvari S.I., Smith H.-J., et al: Vigorous physical activity impairs myocardial function in patients with arrhythmogenic right ventricular cardiomyopathy and in mutation positive family members. Eur J Heart Fail 2014; 16: pp. 1337-1344
- 8. Ruwald A.-C., Marcus F., Estes N.A.M., Link M., McNitt S., Polonsky B., et al: Association of competitive and recreational sport participation with cardiac events in patients with arrhythmogenic right ventricular cardiomyopathy: results from the North American multidisciplinary study of arrhythmogenic right ventricular cardiomyopathy. Eur Heart J 2015; 36: pp. 1735-1743
- 9. James C.A., Bhonsale A., Tichnell C., Murray B., Russell S.D., Tandri H., et al: Exercise increases age-related penetrance and arrhythmic risk in arrhythmogenic right ventricular dysplasia/cardiomyopathy-associated desmosomal mutation carriers. J Am Coll Cardiol 2013; 62: pp. 1290-1297
- 10. Harper R.W., and Mottram P.M.: Exercise-induced right ventricular dysplasia/cardiomyopathy–an emerging condition distinct from arrhythmogenic right ventricular dysplasia/cardiomyopathy. Heart Lung Circ 2009; 18: pp. 233-235
- 11. Vitarelli A., Cortes Morichetti M., Capotosto L., De Cicco V., Ricci S., Caranci F., et al: Utility of strain echocardiography at rest and after stress testing in arrhythmogenic right ventricular dysplasia. Am J Cardiol 2013; 111: pp. 1344-1350
- 12. Alizade E., Yesin M., Tabakci M.M., Avci A., Bulut M., Acar G., et al: Utility of speckle tracking echocardiography imaging in patients with asymptomatic and symptomatic arrhythmogenic right ventricular cardiomyopathy. Echocardiography 2016; 33: pp. 1683-1688
- 13. Qasem M., Utomi V., George K., Somauroo J., Zaidi A., Forsythe L., et al: A meta-analysis for the echocardiographic assessment of right ventricular structure and function in ARVC: a study by the Research and Audit Committee of the British Society of Echocardiography. Echo Res Pract 2016; 3: pp. 95-104
- 14. te Riele A.S.J.M., James C.A., Rastegar N., Bhonsale A., Murray B., Tichnell C., et al: Yield of serial evaluation in at-risk family members of patients with ARVD/C. J Am Coll Cardiol 2014; 64: pp. 293-301
- 15. te Riele A.S.J.M., Tandri H., Sanborn D.M., and Bluemke D.A.: Noninvasive multimodality imaging in ARVD/C. JACC Cardiovasc Imaging 2015; 8: pp. 597-611
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
