049ONE AND A HALF VENTRICLE REPAIR IN ASSOCIATION WITH TRICUSPID VALVE REPAIR FOR EBSTEIN'S ANOMALY AND FAILING RIGHT VENTRICLE

• Published in: Interactive cardiovascular and thoracic surgery 2013-10, Vol.17 (suppl_2), p.S80-S81
• Main Authors: Prifti, E., Baboci, A., Fagu, A., Bonacchi, M., Giunti, G., Dado, E., Vanini, V., Kajo, E., Veshti, A.
• Format: Article
• Language: English
• ISSN: 1569-9293; 1569-9285
• DOI: 10.1093/icvts/ivt372.49

Objectives: Bidirectional cavopulmonary shunt (BDG) may improve early outcomes in patients with Ebstein's anomaly and impaired right ventricular function. We reviewed our experience with the one and a half ventricle repair in this patient population. Methods: Between 2000 and 2013, 10 patients underwent one and a half ventricle repair for Ebstein's anomaly. All of them had a BDG constructed. The median age at operation was 15 years (55 months-27.8 years). All patients had severe Ebstein's anomaly with dilated right-sided chambers and impaired right ventricular function. The mean left ventricular ejection fraction was 55%. The associated malformation was ASD in five patients. Results: Procedures included BDG (10), tricuspid valve replacement (1), tricuspid valve repair (9), and right ventricular plication according to Danielson (4) or Carpentier (6), and ASD closure (5). BDG was planned preoperatively in eight patients and was performed in two other patients for haemodynamic instability. The azygos vein was left open in all patients undergoing one and a half ventricle repair to prevent severe postoperative central venous hypertension. One patient underwent a Fontan operation 24 hours after the first procedure. This patient died during the second procedure due to low cardiac output. At follow-up, tricuspid incompetence of eight surviving patients with bidirectional cavopulmonary shunt undergoing tricuspid repair was mild in six and moderate in two. One of the patients undergoing repair at 19 years old, became a mother three years later, with an excellent pregnancy period. Conclusions: BDG may be considered as a planned procedure in patients with Ebstein's anomaly and impaired right ventricular function or as an intraoperative salvage manoeuvre.