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Benson, D.W., Silberbach, G.M., Kavanaugh McHugh, A., Cottrill, C., Zhang, Y., Riggs, S., Smalls, O., Johnson, M.C., Watson, M.S., Seidman, J.G., Seidman, C.E., Plowden, J. and Kugler, J.D. (1999) Mutations in the Cardiac Transcription Factor NKX2.5 Affect Diverse Cardiac Developmental Pathways. Journal of Clinical Investigation, 104, 1567-1573.

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• TITLE: Ebstein's Anomaly—An Overview

  AUTHORS: Ramachandran Muthiah

  KEYWORDS: “Sail-Like” Deformity, “Atretic” Ebstein’s Anomaly, Inverted Ebstein’s Anomaly, Ebstein’s Mitral Valve, Kay Annuloplasty

  JOURNAL NAME: Case Reports in Clinical Medicine, Vol.7 No.2, February 7, 2018

  ABSTRACT: Aim: To present the various echocardiographic spectrum of Ebstein’s mal-formation in adolescence and adults. Introduction: Ebstein’s anomaly has different anatomic and hemodynamic variables with clinical manifestations of cyanosis, right-sided heart failure and arrhythmias. The leaflet tethering and dysplasia, together with dilatation of the tricuspid valve ring, constitute the anatomic cause of tricuspid regurgitation observed in this condition. Case Reports: The spectrum of leaflet tethering from mild to extreme with varying degrees of tricuspid regurgitation were documented by echocardiography in a 16-year-old cyanotic male with Ebstein’s anomaly associated with an atrial septal defect and mild low tricuspid regurgitation (TR), 22-year-old acyanotic male with right-sided heart failure due to severe tricuspid regurgitation and an intact atrial septum, 55-year-old asymptomatic female with moderate high tricuspid regurgitation. The extreme form was described as an “atretic” mem-brane in a 28-year-old cyanotic male and as a “blanket” of leaflet tissue in a 30-year-old cyanotic male. Conclusion: Ebstein’s anomaly has to be sus-pected clinically in presence of cyanosis with a WPW (Wolf-Parkinson-White) or atrioventricular (AV) block pattern of ECG and its management is complex and must be individualized. RV (right ventricular) exclusion procedures are preferred in advanced cases.