Arrhythmogenic Right Ventricular Cardiomyopathy: Usefulness of Imaging in Prognostic Stratification and Choice of Treatment


Subgroups

Risk markers

Recommendations

Follow-up

ICD indication

Definite ARVC: high risk

Aborted SD

Reduce physical exercise

Annually, including:

Recommended

Sustained VT

Avoid competitive sport

 Electrocardiography

Unexplained syncope

β-blockers

 Cardiac imaging (echocardiography vs. CMR)

 Holter

 Exercise stress testing

Definite ARVC: moderate risk

Extensive disease (severe RV dysfunction, large LV involvement)

Reduce physical exercise

Annually including:

Consider

Nonsustained VT

Avoid competitive sport

 Electrocardiography

β-blockers

 Cardiac imaging (echocardiography vs. CMR)

 Holter

 Exercise stress testing

Definite ARVC: low risk

Remaining patients with definite diagnosis of ARVC

Reduce physical exercise

Annually including:

Not recommended

Avoid competitive sport

 Electrocardiography

β-blockers

 Cardiac imaging (echocardiography vs. CMR)

 Holter

 Exercise stress testing

Asymptomatic mutation carriers

Asymptomatic mutation-carrying relatives of ARVC

Reduce physical exercise

Annually including:

Not recommended

Avoid competitive sport

 Electrocardiography

 Cardiac imaging (echocardiography vs. CMR)

 Holter

 Exercise stress testing


Modified from Epstein et al. [3]

CMR cardiac magnetic resonance, LV left ventricle, RV right ventricle, SD sudden death, VT ventricular tachycardia





References



1.

Marcus FI, Fontaine GH, Guiraudon G, Frank R, Laurenceau JL, Malergue C, Grosgogeat Y (1982) Right ventricular dysplasia: a report of 24 adult cases. Circulation 65(2):384–398PubMedCrossRef


2.

Hauer RN, Aliot E, Block M, Capucci A, Luderitz B, Santini M, Vardas PE (2001) Indications for implantable cardioverter defibrillator (ICD) therapy. Study Group on Guidelines on ICDs of the Working Group on Arrhythmias and the Working Group on Cardiac Pacing of the European Society of Cardiology. Eur Heart J 22(13):1074–1081. doi:10.​1053/​euhj.​2001.​2584 PubMedCrossRef


3.

Epstein AE, DiMarco JP, Ellenbogen KA, Estes NA 3rd, Freedman RA, Gettes LS, Gillinov AM, Gregoratos G, Hammill SC, Hayes DL, Hlatky MA, Newby LK, Page RL, Schoenfeld MH, Silka MJ, Stevenson LW, Sweeney MO, Smith SC Jr, Jacobs AK, Adams CD, Anderson JL, Buller CE, Creager MA, Ettinger SM, Faxon DP, Halperin JL, Hiratzka LF, Hunt SA, Krumholz HM, Kushner FG, Lytle BW, Nishimura RA, Ornato JP, Riegel B, Tarkington LG, Yancy CW (2008) ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices): developed in collaboration with the American Association for Thoracic Surgery and Society of Thoracic Surgeons. Circulation 117(21):e350–e408. doi:10.​1161/​CIRCUALTIONAHA.​108.​189742 PubMedCrossRef


4.

Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M, Gregoratos G, Klein G, Moss AJ, Myerburg RJ, Priori SG, Quinones MA, Roden DM, Silka MJ, Tracy C, Smith SC Jr, Jacobs AK, Adams CD, Antman EM, Anderson JL, Hunt SA, Halperin JL, Nishimura R, Ornato JP, Page RL, Riegel B, Blanc JJ, Budaj A, Dean V, Deckers JW, Despres C, Dickstein K, Lekakis J, McGregor K, Metra M, Morais J, Osterspey A, Tamargo JL, Zamorano JL (2006) ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death). J Am Coll Cardiol 48(5):e247–e346. doi:10.​1016/​j.​jacc.​2006.​07.​010 PubMedCrossRef


5.

Epstein AE, DiMarco JP, Ellenbogen KA, Estes NA 3rd, Freedman RA, Gettes LS, Gillinov AM, Gregoratos G, Hammill SC, Hayes DL, Hlatky MA, Newby LK, Page RL, Schoenfeld MH, Silka MJ, Stevenson LW, Sweeney MO, Smith SC Jr, Jacobs AK, Adams CD, Anderson JL, Buller CE, Creager MA, Ettinger SM, Faxon DP, Halperin JL, Hiratzka LF, Hunt SA, Krumholz HM, Kushner FG, Lytle BW, Nishimura RA, Ornato JP, Riegel B, Tarkington LG, Yancy CW (2008) ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices) developed in collaboration with the American Association for Thoracic Surgery and Society of Thoracic Surgeons. J Am Coll Cardiol 51(21):e1–e62. doi:10.​1016/​j.​jacc.​2008.​02.​032 PubMedCrossRef


6.

Schinkel AF (2013) Implantable cardioverter defibrillators in arrhythmogenic right ventricular dysplasia/cardiomyopathy: patient outcomes, incidence of appropriate and inappropriate interventions, and complications. Circ Arrhythm Electrophysiol 6(3):562–568. doi:10.​1161/​CIRCEP.​113.​000392 PubMedCrossRef


7.

Saxon LA, Bristow MR, Boehmer J, Krueger S, Kass DA, De Marco T, Carson P, DiCarlo L, Feldman AM, Galle E, Ecklund F (2006) Predictors of sudden cardiac death and appropriate shock in the Comparison of Medical Therapy, Pacing, and Defibrillation in Heart Failure (COMPANION) Trial. Circulation 114(25):2766–2772. doi:10.​1161/​CIRCULATIONAHA.​106.​642892 PubMedCrossRef


8.

Buxton AE, Lee KL, Fisher JD, Josephson ME, Prystowsky EN, Hafley G (1999) A randomized study of the prevention of sudden death in patients with coronary artery disease. Multicenter Unsustained Tachycardia Trial Investigators. N Engl J Med 341(25):1882–1890. doi:10.​1056/​NEJM199912163412​503 PubMedCrossRef


9.

Pires LA, Hafley GE, Lee KL, Fisher JD, Josephson ME, Prystowsky EN, Buxton AE (2002) Prognostic significance of nonsustained ventricular tachycardia identified postoperatively after coronary artery bypass surgery in patients with left ventricular dysfunction. J Cardiovasc Electrophysiol 13(8):757–763PubMedCrossRef

Mar 14, 2016 | Posted by in CARDIOVASCULAR IMAGING | Comments Off on Arrhythmogenic Right Ventricular Cardiomyopathy: Usefulness of Imaging in Prognostic Stratification and Choice of Treatment

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