title: Ebstein's anomaly: one and a half ventricular repair.
abstract: Patients with Ebstein's anomaly can present after childhood or adolescence with cyanosis, arrhythmias, severe right ventricular dysfunction and frequently with left ventricular dysfunction secondary to the prolonged cyanosis and to the right ventricular interference. At this point conventional repair is accompanied by elevated mortality and morbidity and poor functional results. We report our experience with three patients (8, 16 and 35 years of age) with Ebstein's anomaly, very dilated right atrium, severe tricuspid valve regurgitation (4/4), bi-directional shunt through an atrial septal defect and reduced left ventricular function (mean ejection fraction = 58%, mean shortening fraction = 25%). All underwent one and a half ventricular repair consisting of closure of the atrial septal defect, tricuspid repair with reduction of the atrialised portion of the right ventricle and end-to-side anastomosis of the superior vena cava to the right pulmonary artery. All patients survived, with a mean follow-up of 33 months. In all there was complete regression of the cyanosis and of the signs of heart failure. Postoperative echocardiography showed reduced degree of tricuspid regurgitation (2/4) and improvement of the left ventricular function (mean ejection fraction = 77%, mean shortening fraction = 40%). In patients with Ebstein's anomaly referred late for surgery with severely compromised right ventricular function or even with reduced biventricular function, the presence of a relatively hypoplastic and/or malfunctioning right ventricular chamber inadequate to sustain the entire systemic venous return but capable of managing part of the systemic venous return, permits a one and a half ventricular repair with good functional results.
authors.affiliation: Cardiovascular Surgery, Centre Hospitalier Universitaire Vandois, Lausanne. antonio.corno@chuv.hospvd.ch
authors.forename: Antonio F
authors.initials: AF
authors.lastname: Corno
authors.forename: Pierre-Guy
authors.initials: PG
authors.lastname: Chassot
authors.forename: Maurice
authors.initials: M
authors.lastname: Payot
authors.forename: Nicole
authors.initials: N
authors.lastname: Sekarski
authors.forename: Piergiorgio
authors.initials: P
authors.lastname: Tozzi
authors.forename: Ludwig K
authors.initials: LK
authors.lastname: Von Segesser
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D003490:Cyanosis / Q000209:etiology; D004437:Ebstein Anomaly / Q000000981:diagnostic imaging / Q000503:physiopathology* / Q000601:surgery*; D018618:Echocardiography, Doppler, Color; D005260:Female; D006325:Heart Atria / Q000601:surgery; D006346:Heart Septum / Q000601:surgery; D006801:Humans; D008297:Male; D013997:Time Factors; D016896:Treatment Outcome; D014262:Tricuspid Valve Insufficiency / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D018487:Ventricular Dysfunction, Left / Q000209:etiology; D018497:Ventricular Dysfunction, Right / Q000209:etiology