Oxford Medical Case Reports, 2018;1, 35–36 doi: 10.1093/omcr/omx088 Clinical Image
CLINICAL IMAGE
Intracardiac ultrasound to detect aneurysm in arrhythmogenic right ventricular dysplasia/cardiomyopathy Cismaru Gabriel1,*, Mihai Puiu1, Radu Rosu1, Lucian Muresan1, Raluca Rancea2, Gabriel Gusetu1, Dana Pop1 and Dumitru Zdrenghea1 1
Cardiology-Rehabilitation, 5th Department of Internal Medicine, ‘Iuliu Hatieganu’ University of Medicine and Pharmacy, Cluj-Napoca, Romania, and 2Heart Institute, 5th Department of Internal Medicine, ‘Iuliu Hatieganu’ University of Medicine and Pharmacy, Cluj-Napoca, Romania
*Correspondence address. Cardiology Department, Rehabilitation Hospital, Viilor 46-50 Street, 400437, Cluj-Napoca, Romania. Tel: +40-72-19-26-230; E-mail:
[email protected]
Abstract Arrhythmogenic right ventricular dysplasia/cardiomyopathy is a genetic disorder characterized by fibrofattty replacement of the right ventricular myocardium. In the revised 2010 Task Force Criteria, a major criteria for ARVD/C is the presence of RV aneurysm by 2D echo. Our report demonstrates that intracardiac ultrasound can detect RV aneurysms and also focal absence of trabeculations which brings additional value to the diagnosis of ARVD/C. A 26-year-old male patient suffering from multiple sustained episodes of ventricular tachycardia was implanted with an internal cardiac defibrillator after confirmation of the disease by cardiac magnetic resonance imaging. Intracardiac ultrasound was performed using a 6F, 9 MHz catheter and the iLAB intravascular system (Boston Scientific). Images of right ventricular inflow tract, outflow tract, apex and pulmonary artery were taken and saved on videotape. ICE revealed loss of trabecular structure at the right ventricular outflow tract with an antero-septal aneurysm at this level. Intravascular ultrasound provides useful information on the presence of aneurysms and of tissue characterization.
A 26-year-old male patient was diagnosed with arrhythmogenic right ventricular cardiomyopathy/dysplasia by electrocardiographic features (presence of epsilon wave in lead V1–V3), echocardiography (right ventricular dilation) and cardiac resonance magnetic imaging. He presented a syncope and multiple episodes of ventricular tachycardia with a left-bundle branch block morphology and therefore was implanted with an internal cardiac defibrillator. A contrast-enhanced computed tomography showed a focal aneurysm of the right ventricular outflow tract which was further confirmed by intracardiac ultrasound. A 6F, 9 MHz catheter in combination
with the iLAB intravascular system (Boston Scientific) [1] was used for imaging of the so-called ‘triangle’ of ARVD: inflow tract-outflow tract and apex of the right ventricle. Visualization of the pulmonary artery and right ventricular outflow tract was saved on videotape during pulling back of the catheter. By intracardiac ultrasound a focal aneurysm of the anteroseptal wall of RVOT was identified, corresponding with that of contrast-enhanced computed tomography. Absence of trabeculations in the RVOT could be demonstrated at this level (Fig. 1). Catheter ablation of the right ventricular VT was performed with no-inducibility at the end of the procedure by
Received: July 21, 2017. Revised: October 3, 2017. Accepted: November 5, 2017 © The Author 2018. Published by Oxford University Press. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact
[email protected]
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trabeculations is a typical finding in patients with ARVD/C [5]. Our report indicates that intracardiac ultrasound can demonstrate focal absence of trabeculations which adds additional value for ARVD/C diagnosis.
ACKNOWLEDGEMENTS None to declare.
CONFLICT OF INTEREST None to declare.
FUNDING This work was supported by research grants from the internal Grant number (4994/1/08.03.2016) of the Iulu Hatieganu University of Medicine and Pharmacy Cluj-Napoca, Romania.
ETHICS APPROVAL Not required.
CONSENT The patient provided consent for the case presentation and intravascular ultrasound images to be published.
GUARANTOR Figure 1: Intracardiac ultrasound of the right ventricular outflow tract and right
Dr Cismaru Gabriel.
ventricular apex in a patient with arrhythmogenic right ventricular dysplasia/ cardiomyopathy. (A) At the level of the RVOT an aneurysm is present (red arrow) that was also identified during contrast-enhanced computed tomog-
REFERENCES
raphy. Lack of trabeculations is a marker of ARVD/C. (B) At the level of the apex,
1. Cismaru G, Schiau S, Muresan L, Rosu R, Puiu M, Gusetu G, et al. Intravascular pulmonary venous ultrasound imaging for catheter ablation of atrial fibrillation. Expert Rev Med Devices 2017;14:309–14. 2. Marcus FI, Mc Kenna WJ, Sherrill D, Basso C, Bauce B, Bluemke DA, et al. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation 2010;121:1533–41. 3. Pop-Mandru D, Cismaru G, Pop D, Zdrenghea D. Arrhythmogen right ventricular tachycardia masquerading right ventricular outflow tract tachycardia. Int J Case Rep Images 2014;5:314–9. 4. Peters S, Brattstrom A, Gotting B, Trummel M. Value of intracardiac ultrasound in the diagnosis of arrhythmogenic right ventricular dysplasia-cardiomyopathy. Int J Cardiol 2002;83: 111–7. 5. Lobo FV, Heggtveit HA, Butany J, Silver MD, Edwards JE. Right ventruicular dysplasia: morphological findings in 13 cases. Can J Cardiol 1992;8:261–8.
trabeculations are present (yellow arrow); in this particular patient the apex was not affected by dysplasia.
programmed ventricular stimulation. There were no femoral or cardiac post-procedural complications. The ESC diagnosis of ARVD/C is based on morphological and functional changes of the right ventricle. In early stages patients are often asymptomatic but with a risk of death during effort which is still present. In late stages patients present symptomatic arrhythmias and structural changes of the right ventricle and RVOT are present. Structural changes include presence of aneurysm or motion abnormalities, and segmental or global dilation of the right ventricle [2]. Transthoracic echocardiography is the first line imaging technique but it has a low ability to demonstrate localized RVOT aneurysm [3, 4]. The ‘gold standard’ in the diagnosis is selective right ventricular angiography, but tissue characterization is not possible with this technique. From a pathological point of view it is known that loss of
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