ABC | Volume 112, Nº5, May 2019

Case Report Kilit et al WPW syndrome in a family with CFNS Arq Bras Cardiol. 2019; 112(5):594-596 Figure 1 – The 12-lead ECG of the patient showing Type AWolff-Parkinson-White pattern: PR interval < 120 ms, positive delta waves (black arrowheads) in all precordial leads (V1-V6) with R/S > 1 in V1. with affected subjects who had ventricular pre-excitation with conduction abnormalities and cardiac hypertrophy, mapped the PRKAG2 gene responsible for WPW to chromosome 7q34-q36. 6 A missense mutation, Arg531Gly, was identified in affected individuals who had ventricular pre-excitation and conduction system disease with childhood onset and absence of cardiac hypertrophy. 7 There are very few cases describing association of CFNS with heart defects such as atrial septal defect. 9,10 To date, there are no reported cases of CFNS with WPW syndrome, suggesting that this novel finding can be part of this condition. Approximately 100 different mutations have been reported in CFNS and Gly151Ser mutation in EFNB1 gene may cause familial WPW syndrome in this CFNS family. Conclusion To our knowledge, this is the first report of a family with WPW syndrome and CFNS. Genetic analyses are needed to explain this association between CFNS and WPW syndrome. Clinicians must be aware in patients with CFNS syndrome in terms of the presence of ventricular pre-excitation. Author contributions Conception and design of the research and analysis and interpretation of the data: Kilit C; writing of the manuscript and critical revision of the manuscript for intellectual contente: Kilit C, Kilit TP. Potential Conflict of Interest No potential conflict of interest relevant to this article was reported. Sources of Funding There were no external funding sources for this study. Study Association This study is not associatedwith any thesis or dissertationwork. 595