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Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D)
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Author: Tarique Hussain

Publish Date: 11/1/2017

Indications and purpose of the scan

  • In patients with suspected ARVC/D, CMR is an important test to help confirm or refute diagnosis. It is a safe, fast, and cost-efficient and is an important test to rule out other conditions that may mimic ARVC/D.

Description

  • Cardiac cine images acquired without contrast for ventricular volumetric assessment are required for diagnostic purposes. Standard short-axis cine stack, 2ch, 3ch, and 4ch cines should be combined with a transverse (axial) cine stack and right-ventricular outflow tract cines. Cardiac images acquired late after contrast administration (myocardial late gadolinium enhancement images) may be helpful to suggest other cardiomyopathies in the differential diagnosis. Additional 3D imaging or phase-contrast flow imaging may be required if a shunt or congenital heart disease is suspected in the differential as a cause of right ventricular dilation (e.g. atrial septal defect, anomalous pulmonary venous drainage).

Why CMR (Specific Advantages)

  • CMR is the gold-standard for assessment of right ventricular volume due to its ability to clearly image RV in unrestricted planes. It is not limited by the acoustic windows of ultrasound and it is radiation-free. It is part of the recommended diagnostic work-up for this disease.1
  • Other cardiomyopathies and congenital defects can mimic ARVC/D. Myocardial late gadolinium enhancement is a useful adjunct to volumetric imaging to diagnose cardiomyopathies; the combination of cine imaging, phase contrast flow imaging, and magnetic resonance angiography is useful to help diagnose congenital defects.

Evidence

  • Revised Task Force Criteria (108 Patients).1 CMR assessment of right ventricular volume and wall motion have 96 to 100% specificity for ARVC/D. The accepted major criterion for diagnosis is regional RV akinesia or dyskinesia or dyssynchronous RV contraction, and 1 of the following:

-          Ratio of RV end-diastolic volume to BSA >110 mL/m2 (male) or >100 mL/m2 (female)

-          or RV ejection fraction <40%

  • CMR has been shown to be an important diagnostic discriminant in children and adults also (142 patients).2

Contraindications

  • Reasons to consider a test other than CMR include the presence of contraindications to MRI, and severe renal failure (eGFR<30).

Appropriateness

  • CMR is appropriate as part of a diagnostic work-up of suspected ARVC/D.3, 4 

References:

  1.  Marcus FI, McKenna WJ, Sherrill D, Basso C, Bauce B, Bluemke DA, Calkins H, Corrado D, Cox MG, Daubert JP, Fontaine G, Gear K, Hauer R, Nava A, Picard MH, Protonotarios N, Saffitz JE, Sanborn DM, Steinberg JS, Tandri H, Thiene G, Towbin JA, Tsatsopoulou A, Wichter T, Zareba W. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation. 2010;121(13):1533-41. doi: 10.1161/CIRCULATIONAHA.108.840827. PubMed PMID: 20172911; PMCID: PMC2860804.
  2. Etoom Y, Govindapillai S, Hamilton R, Manlhiot C, Yoo SJ, Farhan M, Sarikouch S, Peters B, McCrindle BW, Grosse-Wortmann L. Importance of CMR within the Task Force Criteria for the diagnosis of ARVC in children and adolescents. Journal of the American College of Cardiology. 2015;65(10):987-95. doi: 10.1016/j.jacc.2014.12.041. PubMed PMID: 25766945.
  3. Shen WK, Sheldon RS, Benditt DG, Cohen MI, Forman DE, Goldberger ZD, Grubb BP, Hamdan MH, Krahn AD, Link MS, Olshansky B, Raj SR, Sandhu RK, Sorajja D, Sun BC, Yancy CW. 2017 ACC/AHA/HRS Guideline for the Evaluation and Management of Patients With Syncope: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation. 2017;136(5):e25-e59. doi: 10.1161/CIR.0000000000000498. PubMed PMID: 28280232.
  4. European Heart Rhythm A, Heart Rhythm S, 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, Priori SG, Blanc JJ, Budaj A, Camm AJ, Dean V, Deckers JW, Despres C, Dickstein K, Lekakis J, McGregor K, Metra M, Morais J, Osterspey A, Tamargo JL, Zamorano JL, American College of C, American Heart Association Task F, European Society of Cardiology Committee for Practice G. 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). Journal of the American College of Cardiology. 2006;48(5):e247-346. doi: 10.1016/j.jacc.2006.07.010. PubMed PMID: 16949478.
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