Related Experiment Video
Updated: Sep 28, 2026
Echocardiographic Evaluation of Atrial Communications before Transcatheter Closure
Published on: February 8, 2022
Ebstein's anomaly: one and a half ventricular repair
Antonio F Corno1, Pierre-Guy Chassot, Maurice Payot
1Cardiovascular Surgery, Centre Hospitalier Universitaire Vandois, Lausanne. antonio.corno@chuv.hospvd.ch
Insights
One and a half ventricular repair offers a promising surgical option for late-presenting Ebstein's anomaly patients with compromised ventricular function. This innovative approach significantly improves cyanosis, heart failure symptoms, and overall cardiac function.
Area of Science:
- Cardiology
- Congenital Heart Disease Surgery
Background:
- Ebstein's anomaly presenting late often involves severe right ventricular dysfunction, atrial enlargement, and tricuspid regurgitation.
- Conventional repair in such cases is associated with high mortality, morbidity, and suboptimal functional outcomes.
Observation:
- Three patients (8, 16, 35 years) with advanced Ebstein's anomaly underwent one and a half ventricular repair.
- The procedure included atrial septal defect closure, tricuspid valve repair, and superior vena cava to right pulmonary artery anastomosis.
Findings:
- All patients survived the surgery with a mean follow-up of 33 months.
- Complete resolution of cyanosis and heart failure symptoms was observed.
- Postoperative echocardiography revealed reduced tricuspid regurgitation and improved left ventricular function.
Implications:
- One and a half ventricular repair is a viable strategy for selected Ebstein's anomaly patients with compromised biventricular function.
- This technique can lead to significant functional recovery and improved quality of life.
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.
