Ebstein Anomalisine Eşlik Eden Atriyal Septal Defekt

Abstract

Ebstein anomalisi 1866 yılında Wilhelm Ebstein tarafından tanımlanmıştır. Ebstein anomalisi tüm konjenital kalp anomalilerinin % 1 ini oluşturmaktadır. Sıklıkla Pulmoner stenoz veya atrezi, Atriyal Septal Defekt, Ventriküler Septal Defekt ve Fallot Tetrolojisi eşlik eder. Hastalarda genel olarak 3 temel patofizyolojik durum vardır. Sağ ventrikül anomalileri,trikuspit kapak anomalileri,aksesuar ileti yolları WPW sendromu . Medikal tedavisinde konjestif kalp yetmezliği, aritmiler ve bakteriyel endokardit proflaksisi yapılmaktadır. Cerrahi tedavide ise mekanik veya bioprotez kapak replasmanı, kapağın plastik rekonstriksiyonu veya replasman ile rekonstriksiyon yapılabilir.Ebstein anomalisi nadir konjenital kalp hastalıklarından biri olması nedeniyle, kliniğimizde tedavi olan bir olgu literatür bilgileri verilerek sunulmaktadır

Keywords

• Ebstein Anomali,Atrial Septal Defekt

Atrıal Septal Defect Wıth Ebsteın’s Anomaly

Abstract

Ebstein anomally described by Willhelm Ebstein in 1866. Ebstein anomaly accounting for 1/100 of all cases of congenital heart disease. Pulmoner stenosis or atresia is most common accompaying defect. Others include ASD,VSD,TOF,PDA.Three primary pathophisiologic features predominant patients with anomaly. Right ventricle abnormalities, tricuspidal valve abnormalities, accessory conduction pathways WPW syndrome . Medical treatment will consist of the treatment of the congestive heart failure, arrhytmias and prevention of bacterial endocarditis. Mechanical or bioprothesis valve replacement or valve plastic reconstruction, or reconstruction with replacement have been used in surgical treatments. We report one case of this rare Ebstein’s anomaly with the review of the literature

Keywords

• Ebstein's Anomaly,Atrial Septal Defects.

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