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For Physicians - Symptoms and Diagnosis
- Nonsustained VT
- Sustained VT of left bundle morphology
- Heart failure
- Sudden cardiac arrest
There is no single test that can either establish or exclude ARVD/C. The criteria that is used to determine ARVD/C is a physical exam, family history, various cardiac tests, and genetic information. Tests may include
- Signal Averaged Electrocardiogram
- 24-hour Holter Monitor
- Exercise Stress Test
- Cardiac Magnetic Resonance Imaging (MRI)
- Cardiac Computed Tomography (CT)
- Genetic Testing
- Electrophysiology Study
- Right Ventriculogram (RV angiogram)
- Cardiac Biopsy
In 1994, an International Task Force proposed criteria for the clinical diagnosis of ARVD/C, based on structural, electrocardiographic, arrhythmic, histological and familial characteristics of ARVD/C. These criteria were very specific but lacked sensitivity. These diagnostic criteria were revised in 2010 and now incorporate the advances in both technology and genetics. View a comparison chart of the ARVD diagnosis criteria for 1994 and 2010.
A definite diagnosis of ARVD/C consists of the following criteria options from different categories:
- 2 major criteria, or
- 1 major and 2 minor criteria, or
- 4 minor criteria.
A borderline diagnosis consists of the following criteria options from different categories:
- 1 major and 1 minor criteria, or
- 3 minor criteria
A possible diagnosis consists of the following criteria options from different categories:
- 1 major criteria, or
- 2 minor criteria
This classification is for probands. There is an online guide to help you evaluate your patients for ARVD/C. Simply click on the criteria that are present in your patient, then click on the summary.
A standard 12-lead electrocardiogram (ECG) is one of most useful diagnostic tools in evaluating a patient for ARVD/C. With respect to the ECG and ARVD/C diagnostic criteria, there are 5 main characteristics one should be assessing: T wave inversions, epsilon waves, TAD, and the presence of a right bundle branch block.
We suggest obtaining a 12-lead ECG using standard recording techniques (paper speed: 24 mm/s; 1 mm = 40 ms; 1 mm = 1 mV; 40 Hz filtering). ECGs recorded at double speed double amplitude (paper setting: 50 mm/s; 1 mm = 20 ms; 1mm = 0.5 mV; 40 Hz filtering) are not necessarily more helpful in our experience.
The presence of T wave inversions in leads V1-V3 or beyond in the absence of a complete right bundle branch block (QRS≥120 ms) represents a major criterion according to the 2010 Diagnostic Task Force Criteria. T wave inversions in leads V1 and V2 in the absence of a complete right bundle branch block or in leads V1-V4 in the presence of a complete right bundle branch block represent a minor criterion.
We find that T wave inversions are a very important parameter as they are easy to interpret and require no special measurement.
A more difficult ECG characteristic to identify is the epsilon wave which also represents a major criterion. The epsilon wave is defined as “distinct waves of small amplitude that occupies the ST segment in the right precordial leads and are distinct from the QRS complex.” (Rahul’s paper) In our experience, epsilon waves are seen infrequently and generally only in patients with severe disease where the diagnosis is rarely uncertain.
Terminal Activation Delay (TAD) is defined as “the longest value V1-V3 from the nadir of the S wave to the end of all depolarization. A TAD of ≥55ms in the absence of a complete right bundle branch block constitutes a minor criterion. Although we agree that this is a valuable addition to the diagnostic criteria, the error with which precise time measurements are made on ECG tracings results in the criterion being less reproducible and harder to apply.
In addition, a Signal Averaged Electrocardiogram (SAECG) is another diagnostic tool used to assess for depolarization abnormalities in ARVD/C in whom the QRS duration is ≤110ms on standard ECG. Late potentials observed in 1 or more parameters (filtered QRS, LAS duration or RMS voltage of terminal 40ms) represent a minor criterion. We recommend averaging 350 beats at a filter setting of 40-250 Hz with a noise level < 0.3uV.
If a patient has a complete RBBB pattern none of the parameters listed above can be used to make the diagnosis of ARVD/C. We have found that patients with ARVD/C have rather unusual RBBB patterns with more extensive T wave inversions across the precordium and an R’/S ratio < 1 in lead V1. Only one of these criteria (epsilon wave, TAD or SAECG) can be applied toward the diagnostic criteria.
Jain R, Tandri H, Daly A, Tichnell C, James C, Abraham T, Judge D, Calkins H, Dalal D. Reader- and Instrument-Dependent Variability in the Electrocardiograpy Assessment of Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy. J Cardiovasc Electrophysiol 2011; 22:561-568.
Jain R, Dalal D, Daly A, Tichnell C, James C, Evenson A, Jain R, Abraham T, Tan BY, Tandri H, Russell SD, Judge D, Calkins H. Circulation 2009; 120(6):477-87.
Arrhythmias observed in ARVD/C may be captured by a 12-lead ECG, exercise stress test, Holter or event monitor. A 24 hour Holter can be quite helpful in assessing arrhythmia burden, even in asymptomatic patients.
Arrhythmia criteria can be met by the presence of >500 ventricular extrasystoles within a 24 hour time period (minor criteria) or ventricular tachycardia (VT). Morphology of VT is important to assess as this can account for a major or minor criteria. VT of a left bundle superior axis (nonsustained or sustained) OR sustained VT with a negative or indeterminate QRS in II, III, and aVF and positive in VL is considered a major criteria. Any other morphology of nonsustained VT, sustained RVOT VT, sustained VT of left bundle inferior axis, or sustained VT of unknown axis is considered a minor criterion.
The right ventricle is of primary interest when assessing for ARVD/C. The right ventricle can be observed through echocardiogram, MRI, and/or angiogram. The presence of akinesis/dyskinesia/aneurysm on echo or MRI is not sufficient by itself to meet any of the diagnostic criteria for ARVD/C. There are other specific measurements that also need to be met. See Diagnostic Checklist. Specific protocols have been developed to pay special attention to the right ventricle:
The major condition which needs to be differentiated from ARVD/C is idiopathic ventricular tachycardia arising from the outflow tract. The ventricular tachycardia can be exactly the same, but there is no structural abnormality of the heart, unlike the situation in ARVD/C where commonly there is dilation of the ventricle, abnormal contraction, or reduced function. Right ventricular outflow tract tachycardia (RVOT) is more common than ARVD/C and occurs in young, otherwise healthy people. The treatment is either with medications or with catheter ablation. View a comparison of RVOT and ARVD/C.
To request an appointment with the Johns Hopkins ARVD/C Program, please contact Crystal Tichnell, MGC at 410-502-7161 or firstname.lastname@example.org.