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 arrhythmo- with the iLAB intravascular system (Boston Scientiﬁc) was genic right ventricular cardiomyopathy/dysplasia by electro- used for imaging of the so-called ‘triangle’ of ARVD: inﬂow cardiographic features (presence of epsilon wave in lead V1–V3), tract-outﬂow tract and apex of the right ventricle. Visualization echocardiography (right ventricular dilation) and cardiac res- of the pulmonary artery and right ventricular outﬂow tract onance magnetic imaging. He presented a syncope and mul- was saved on videotape during pulling back of the catheter. tiple episodes of ventricular tachycardia with a left-bundle By intracardiac ultrasound a focal aneurysm of the antero- branch block morphology and therefore was implanted septal wall of RVOT was identiﬁed, corresponding with that of with an internal cardiac deﬁbrillator. A contrast-enhanced contrast-enhanced computed tomography. Absence of trabe- computed tomography showed a focal aneurysm of the culations in the RVOT could be demonstrated at this level right ventricular outﬂow tract which was further conﬁrmed (Fig. 1). Catheter ablation of the right ventricular VT was per- by intracardiac ultrasound. A 6F, 9 MHz catheter in combination formed 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 firstname.lastname@example.org Downloaded from https://academic.oup.com/omcr/article-abstract/2018/1/omx088/4824928 by Ed 'DeepDyve' Gillespie user on 16 March 2018 36 C. Gabriel et al. trabeculations is a typical ﬁnding in patients with ARVD/C . Our report indicates that intracardiac ultrasound can demon- strate 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 Dr Cismaru Gabriel. Figure 1: Intracardiac ultrasound of the right ventricular outﬂow tract and right ventricular apex in a patient with arrhythmogenic right ventricular dysplasia/ cardiomyopathy. (A) At the level of the RVOT an aneurysm is present (red REFERENCES arrow) that was also identiﬁed during contrast-enhanced computed tomog- 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, trabeculations are present (yellow arrow); in this particular patient the apex et al. Intravascular pulmonary venous ultrasound imaging was not affected by dysplasia. for catheter ablation of atrial ﬁbrillation. Expert Rev Med Devices 2017;14:309–14. programmed ventricular stimulation. There were no femoral 2. Marcus FI, Mc Kenna WJ, Sherrill D, Basso C, Bauce B, or cardiac post-procedural complications. Bluemke DA, et al. Diagnosis of arrhythmogenic right ven- The ESC diagnosis of ARVD/C is based on morphological and tricular cardiomyopathy/dysplasia: proposed modiﬁcation of functional changes of the right ventricle. In early stages patients the task force criteria. Circulation 2010;121:1533–41. are often asymptomatic but with a risk of death during effort 3. Pop-Mandru D, Cismaru G, Pop D, Zdrenghea D. which is still present. In late stages patients present symptom- Arrhythmogen right ventricular tachycardia masquerading atic arrhythmias and structural changes of the right ventricle right ventricular outﬂow tract tachycardia. Int J Case Rep and RVOT are present. Structural changes include presence of Images 2014;5:314–9. aneurysm or motion abnormalities, and segmental or global 4. Peters S, Brattstrom A, Gotting B, Trummel M. Value of intra- dilation of the right ventricle . Transthoracic echocardiog- cardiac ultrasound in the diagnosis of arrhythmogenic right raphy is the ﬁrst line imaging technique but it has a low ability ventricular dysplasia-cardiomyopathy. Int J Cardiol 2002;83: to demonstrate localized RVOT aneurysm [3, 4]. The ‘gold stand- 111–7. ard’ in the diagnosis is selective right ventricular angiography, 5. Lobo FV, Heggtveit HA, Butany J, Silver MD, Edwards JE. Right but tissue characterization is not possible with this technique. ventruicular dysplasia: morphological ﬁndings in 13 cases. From a pathological point of view it is known that loss of Can J Cardiol 1992;8:261–8. Downloaded from https://academic.oup.com/omcr/article-abstract/2018/1/omx088/4824928 by Ed 'DeepDyve' Gillespie user on 16 March 2018
Oxford Medical Case Reports – Oxford University Press
Published: Jan 1, 2018
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