Modified criteria of arrhythmogenic right ventricular dysplasia do not correspond to the clinical scenario. Case study

Authors: S.A. Aleksandrova 1; O.V. Blagova 2; E.Yu. Glazkova 1; V.N. Makarenko 1;

Company: 1 A.N. Bakoulev Scientific Center for Cardiovascular Surgery of Russian Academy of Medical Sciences; Rublevskoe shosse, 135, Moscow, 121552, Russian Federation; 2 I.M. Sechenov Fierst Moscow Medical State University of Ministry of Health of the Russian Federation; ulitsa Trubetskaya, 8, stroenie 2, Moscow, 119991, Russian Federation


For citation: Makarenko V.N., Aleksandrova S.A., Blago-va O.V., Glazkova E.Yu. Modified criteria of arrhythmogenic right ventricular dysplasia do not correspond to the clinical scenario. Case study. Creative Cardiology. 2014; 1: 70-75

Keywords: магнитно-резонансная томография аритмогенная дисплазия правого желудочка частичный аномальный дренаж легочных вен

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Abstract

This article presents a rare case of magnetic resonance imaging (MRI) of congenital heart disease in patient with suspected arrhythmogenic right ventricular dysplasia (ARVD). Nonspecificyty clinical features of ARVC made the diagnosis clinical challenge. MRI is important in management of these patients; the method allows determining the volume and ventricular function, to evaluate the changes of regional contractility right ventricular wall. Magnetic resonance imaging showed two features corresponding to one major criteria for the diagnosis of ARVD, by International Task Force criteria: increased normalized end systolic volume index of the right ventricle in women over 100 ml/m2 and regional right ventricular wall dyskinesia. Additionally, it was revealed a twofold increase in the pulmonary-to-systemic blood flow ratio, the atrial septal defect and partial anomalous pulmonary venous connection. All findings were confirmed intraoperatively. Magnetic resonance imaging diagnosis of partial anomalous pulmonary venous connection will be successful if the correct protocol is selected and detailed analysis of the anatomy and function of the heart chambers are made.

References

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Chief Editor

Leo A. Bockeria, MD, PhD, DSc, Professor, Academician of Russian Academy of Sciences, President of Bakoulev National Medical Research Center for Cardiovascular Surgery