Ebstein's anomaly: one and a half ventricular repair
Autor: | M. Payot, N. Sekarski, Chassot Pg, von Segesser Lk, Antonio F. Corno, P. Tozzi |
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Rok vydání: | 2002 |
Předmět: |
Adult
Male medicine.medical_specialty Time Factors Adolescent Child Cyanosis Ebstein Anomaly Echocardiography Doppler Color Female Heart Atria Heart Septum Humans Treatment Outcome Tricuspid Valve Insufficiency Ventricular Dysfunction Left Ventricular Dysfunction Right Internal medicine Ebstein's anomaly medicine cardiovascular diseases Tricuspid valve Ejection fraction business.industry General Medicine medicine.disease Right pulmonary artery Surgery medicine.anatomical_structure Ventricle Cardiology cardiovascular system Tricuspid Valve Regurgitation business Venous return curve |
Zdroj: | Swiss Medical Weekly, vol. 132, no. 33-34, pp. 485-488 Scopus-Elsevier |
ISSN: | 1424-7860 |
Popis: | 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. |
Databáze: | OpenAIRE |
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