Ebstein's malformation presenting with tricuspid stenosis: 1-year follow-up after surgical repair.
This is a case of a young woman previously completely healthy, with two uneventful pregnancies and deliveries, who presented with bloating, shortness of breath, and signs of right heart failure. A thorough clinical evaluation, along with a work-up including an echocardiogram and a magnetic resonance imaging revealed the diagnosis of Ebstein's anomaly malformation with tricuspid stenosis. A right ventricular outflow tract obstruction due to thrombus formation was thought to exacerbate her symptoms and lead to the diagnosis. Surgery in the form of right ventricular thrombectomy, right atrial reduction, porcine tricuspid valve replacement and bidirectional cavopulmonary anastomosis "bidirectional Glenn", was successfully performed. The patient has been stable clinically more than one year after surgery. Discussion about this rare condition and operative details are provided.
Khouzam
Rami N
RN
Farmington Heart Center, Farmington, New Mexico, USA. khouzamrami@yahoo.com
Dearani
Joseph A
JA
Julsrud
Paul R
PR
D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000601:surgery; D000328:Adult; D006348:Cardiac Surgical Procedures / Q000379:methods; D003131:Combined Modality Therapy; D004437:Ebstein Anomaly / Q000175:diagnosis* / Q000601:surgery; D004452:Echocardiography / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D018610:Heart Bypass, Right; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000601:surgery; D019918:Heart Valve Prosthesis Implantation / Q000379:methods; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D018570:Risk Assessment; D017131:Thrombectomy / Q000379:methods; D013927:Thrombosis / Q000175:diagnosis / Q000601:surgery; D016896:Treatment Outcome; D014261:Tricuspid Valve / Q000002:abnormalities* / Q000601:surgery; D014264:Tricuspid Valve Stenosis / Q000175:diagnosis* / Q000601:surgery; D014694:Ventricular Outflow Obstruction / Q000175:diagnosis* / Q000601:surgery