Document Detail

Ebstein's anomaly: one and a half ventricular repair.
MedLine Citation:
PMID:  12458449     Owner:  NLM     Status:  MEDLINE    
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.
Antonio F Corno; Pierre-Guy Chassot; Maurice Payot; Nicole Sekarski; Piergiorgio Tozzi; Ludwig K Von Segesser
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Swiss medical weekly     Volume:  132     ISSN:  1424-7860     ISO Abbreviation:  Swiss Med Wkly     Publication Date:  2002 Aug 
Date Detail:
Created Date:  2002-11-29     Completed Date:  2003-03-21     Revised Date:  2011-02-15    
Medline Journal Info:
Nlm Unique ID:  100970884     Medline TA:  Swiss Med Wkly     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  485-8     Citation Subset:  IM    
Cardiovascular Surgery, Centre Hospitalier Universitaire Vandois, Lausanne.
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MeSH Terms
Cyanosis / etiology
Ebstein Anomaly / physiopathology*,  surgery*,  ultrasonography
Echocardiography, Doppler, Color
Heart Atria / surgery
Heart Septum / surgery
Time Factors
Treatment Outcome
Tricuspid Valve Insufficiency / physiopathology,  surgery*,  ultrasonography
Ventricular Dysfunction, Left / etiology
Ventricular Dysfunction, Right / etiology

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