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Early Results of the 1.5 Ventricle Repair for Ebstein’s Anomaly and the Failing Right Ventricle
L. G. Quinonez, J. A. Dearani, F. J. Puga, D. J. Driscoll, H. M. Connolly, G. K. Danielson. Mayo Clinic, Rochester, MN,
BACKGROUND: Patients with Ebstein’s anomaly and a poor right ventricle (RV) pose a surgical challenge and may require transplantation. Our institutional experience with bidirectional cavopulmonary shunts (BDCPS) in this patient population was reviewed. Published experience is limited.
METHODS: Between 1972 and December 2005, 623 patients with Ebstein’s anomaly had surgery at our institution; 11 of these patients had each a BDCPS and a poor RV. All the procedures were performed after July 1999 at a median patient age of 8.3 years [17 months-57 years]. By echocardiography, all the patients had severe Ebstein’s anomaly, severely dilated right-sided chambers, and RV dysfunction; the mean left ventricular ejection fraction (LVEF) was 49% [range 35-72%].
RESULTS: Procedures included BDCPS (11), bioprosthetic tricuspid valve replacement (10), RV resection (2), atrial septal defect closure (1), delayed chest closure (4), and intra-aortic balloon pump (IABP) placement (2). A BDCPS was planned preoperatively in 7 patients; intraoperative indications for the construction of a BDCPS included an inability to wean off cardiopulmonary bypass (2) and hemodynamic instability after chest closure (2). One patient died postoperatively of fungal sepsis; 9 patients were alive at a median of 7 months [1-78 months]. The patient with a preoperative LVEF of 35% had an LVEF of 55% at dismissal.
CONCLUSIONS: The 1.5 ventricle repair can be applied to patients with advanced Ebstein’s anomaly and a poor RV. We believe the BDCPS should be considered in selected patients as an alternative to transplantation or as a salvage procedure for hemodynamic instability after standard repair.
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