Ebstein anomaly in an adult presenting with wide QRS tachycardia: diagnostic and therapeutic dilemmas

A 51-year-old man presented to the emergency department with sustained hemodynamically unstable wide QRS tachycardia and was revived successfully by immediate direct current (DC) cardioversion. There was evidence of previous open heart surgery, possibly atrial septal defect closure. Transthoracic echocardiography showed severe Ebstein anomaly with severe tricuspid regurgitation, no residual atrial septal defect, but with severe right ventricular dysfunction. Subsequent electrocardiograms showed transient atrial fibrillation with no manifest Wolff-Parkinson-White (WPW) accessory pathway during sinus rhythm. The cause of wide QRS tachycardia in this patient may be WPW related or ventricular tachycardia. This case illustrates the diagnostic and therapeutic dilemmas in patients with wide QRS tachycardia and suspected WPW syndrome. In addition, this case demonstrates that unoperated Ebstein anomaly can present in late adult life with tachyarrhythmias.