The accuracy of electrocardiogram-gated CT for diagnosing arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is uncertain.
We propose a comprehensive system for scoring characteristic CT findings to diagnose ARVC/D and discuss its utility.
Seventy-seven patients (mean age, 43.1 years; 48 male) diagnosed with ARVC/D or with suspected ARVC/D on the basis of ventricular tachyarrhythmias underwent CT with and without contrast enhancement. We retrospectively graded characteristic CT findings (fatty tissue, bulging appearance, and dilatation of the right ventricle) as minor (1 point) or major (2 points) and then validated our scoring system’s utility for diagnosing ARVC/D with the use of the modified 2010 Task Force criteria as the reference standard.
We diagnosed 27 of 77 patients with ARVC/D (23 definite, 4 borderline), observing each CT finding more often in patients with ARVC/D than in patients without ARVC/D: fatty tissue, 74.1% versus 12.0%; bulging appearance, 74.1% versus 34.0%; and RV dilatation, 92.6% versus 44.0%. However, observation of each finding in both groups could cause false positive diagnosis. Recursive partitioning analysis showed the superiority of our CT scoring system over methods that used each CT finding and CT findings without fatty tissue for distinguishing ARVC/D and non-ARVC/D. For overall (definite and borderline) and definite ARVC/D diagnosis, sensitivities were 77.8% and 87.0%, specificities were 96.0% and 94.4%, positive predictive values were 91.3% and 87.0%, negative predictive values were 88.9% and 94.4%, and accuracies were 89.6% and 92.2%, respectively.
Our CT scoring system showed excellent diagnostic ability and might aid differentiation of ARVC/D from ventricular tachyarrhythmias.
To read this article in full you will need to make a payment
Purchase one-time access:Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
Full access to the journal is a member benefit for SCCT Members, Login via the SCCT website to access all journal content.
Subscribe:Subscribe to Journal of Cardiovascular Computed Tomography
Already a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
- Arrhythmogenic right ventricular cardiomyopathy and fatty replacement of the right ventricular myocardium: are they different diseases?.Circulation. 1998; 97: 1571-1580
- Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study.J Am Coll Cardiol. 1997; 30: 1512-1520
- Arrhythmogenic right ventricular dysplasia: a cause of ventricular tachycardia in children with apparently normal hearts.Am Heart J. 1981; 102: 745-750
- Arrhythmogenic right ventricular cardiomyopathy.J Am Coll Cardiol. 2001; 38: 1773-1781
- Novel mutation of plakophilin-2 associated with arrhythmogenic right ventricular cardiomyopathy.Circ J. 2006; 70: 933-935
- Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Task Force of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology.Br Heart J. 1994; 71: 215-218
- Arrhythmogenic right ventricular dysplasia/cardiomyopathy: need for an international registry. Study Group on Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy of the Working Groups on Myocardial and Pericardial Disease and Arrhythmias of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the World Heart Federation.Circulation. 2000; 101: E101-E106
- Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria.Circulation. 2010; 121: 1533-1541
- Echocardiographic assessment of arrhythmogenic right ventricular cardiomyopathy.Heart. 2001; 86: 31-38
- Arrhythmogenic right ventricular dysplasia: evaluation with electron-beam CT.Radiology. 1993; 187: 723-727
- Usefulness of electron-beam computed tomography in arrhythmogenic right ventricular dysplasia. Relationship to electrophysiological abnormalities and left ventricular involvement.Circulation. 1996; 94: 437-444
- Helical CT features of arrhythmogenic right ventricular cardiomyopathy.Radiographics. 2002; 22: 1111-1124
- ACCF/SCCT/ACR/AHA/ASE/ASNC/NASCI/SCAI/SCMR 2010 appropriate use criteria for cardiac computed tomography. A report of the American College of Cardiology Foundation Appropriate Use Criteria Task Force, the Society of Cardiovascular Computed Tomography, the American College of Radiology, the American Heart Association, the American Society of Echocardiography, the American Society of Nuclear Cardiology, the North American Society for Cardiovascular Imaging, the Society for Cardiovascular Angiography and Interventions, and the Society for Cardiovascular Magnetic Resonance.J Am Coll Cardiol. 2010; 56: 1864-1894
- Evolving role of multidetector computed tomography in evaluation of arrhythmogenic right ventricular dysplasia/cardiomyopathy.Am J Cardiol. 2007; 100: 99-105
- Myocardial fat at cardiac imaging: how can we differentiate pathologic from physiologic fatty infiltration?.Radiographics. 2010; 30: 1587-1602
- CT detection of subendocardial fat in myocardial infarction.AJR Am J Roentgenol. 2009; 192: 532-537
- Right ventricular fat infiltration in asymptomatic subjects: observations from ECG-gated 16-slice multidetector CT.J Comput Assist Tomogr. 2007; 31: 22-28
- Epidemiology of fat replacement of the right ventricular myocardium determined by multislice computed tomography using a logistic regression model.Int J Cardiol. 2007; 119: 410-413
- Normal reference values for the adult right ventricle by magnetic resonance imaging.Am J Cardiol. 2006; 98: 1660-1664
- Role of magnetic resonance imaging in arrhythmogenic right ventricular dysplasia: insights from the North American arrhythmogenic right ventricular dysplasia (ARVD/C) study.Am Heart J. 2008; 155: 147-153
- Accuracy of guideline recommendations for two-dimensional quantification of the right ventricle by echocardiography.Int J Cardiovasc Imaging. 2008; 24: 691-698
- Reproducibility of MRI measurements of right ventricular size and function in patients with normal and dilated ventricles.J Magn Reson Imaging. 2008; 28: 67-73
- Controversial role of magnetic resonance imaging in the diagnosis of arrhythmogenic right ventricular dysplasia [letter].Am J Cardiol. 2003; 92: 649
- Magnetic resonance imaging findings in patients meeting task force criteria for arrhythmogenic right ventricular dysplasia.J Cardiovasc Electrophysiol. 2003; 14: 476-482
- Long-term follow up of patients with endomyocardial fibrosis: effects of surgery.Heart. 1998; 79: 362-367
- Prospective study of cardiac sarcoid mimicking arrhythmogenic right ventricular dysplasia.J Cardiovasc Electrophysiol. 2009; 20: 473-476
- Right ventricular wall motion abnormalities found in healthy subjects by cardiovascular magnetic resonance imaging and characterized with a new segmental model.J Cardiovasc Magn Reson. 2004; 6: 601-608
- Right ventricle shape and contraction patterns and relation to magnetic resonance imaging findings.J Comput Assist Tomogr. 2005; 29: 725-733
- Magnetic resonance and computed tomography imaging of arrhythmogenic right ventricular dysplasia.J Magn Reson Imaging. 2004; 19: 848-858
- Magnetic resonance imaging in individuals with cardiovascular implantable electronic devices.Europace. 2008; 10: 336-346
- Assessment of right ventricular function by 16-detector-row CT: comparison with magnetic resonance imaging.Eur Radiol. 2005; 15: 312-318
- Which concentration to choose in dual flow cardiac CT?: dual flow cardiac CT.Eur J Radiol. 2012; 81: e461-e466
- Comparison of mortality rates and progression of left ventricular dysfunction in patients with idiopathic dilated cardiomyopathy and dilated versus nondilated right ventricular cavities.Am J Cardiol. 1997; 80: 1583-1587
- Noninvasive detection of myocardial fibrosis in arrhythmogenic right ventricular cardiomyopathy using delayed-enhancement magnetic resonance imaging.J Am Coll Cardiol. 2005; 45: 98-103
- Adaptive statistical iterative reconstruction: assessment of image noise and image quality in coronary CT angiography.AJR Am J Roentgenol. 2010; 195: 649-654
Published online: June 03, 2013
Accepted: May 26, 2013
Received in revised form: April 15, 2013
Received: January 18, 2013
© 2013 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.