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| Current Surgical Therapy for Ebstein's Anomaly in Neonates |
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Back to 86th Annual Meeting Back to Program Outline Objective: Newborns presenting in profound heart failure due to Ebstein's anomaly (E.A.) have historically had poor outcomes. We report our institutional experience with the surgical management of E.A. in severely symptomatic neonates. Methods: A retrospective review of all patients (n=16) undergoing neonatal intervention for E.A. between '92 and '05 has been carried out. The indication for operation was overt heart failure associated with tricuspid regurgitation, depressed RV function and severe cardiomegaly. The magnitude of cardiac enlargement was assessed by CT ratio and GOS ratio (area of RA + atrialized RV/ area functional RV + LA+LV). The operative strategy was to first assess for the possibility of TV repair with or without RVOT reconstruction. If this was not feasible then RV exclusion was performed by over sewing the TV with a pericardial patch. A reduction atrioplasty was done and depending on the extent of the atrialized portion of the RV plication was performed. A modified BT shunt provided pulmonary blood flow. This univentricular approach (Starnes procedure) evolved to include a fenestration in the TV patch to allow for RV decompression. Analysis included overall and group specific survival as well as the testing of perioperative clinical, morphologic, and surgical variables for correlation with mortality and morbidity. Results: : Mean age and weight at operation was 8+10 days and 3.1+ 0.4 kg. Tricuspid valve repair was undertaken in 3 patients with one requiring conversion to RV exclusion three months after the initial operation. In those with RV exclusion the TV patch was fenestrated in 10 and non-fenestrated in 3. One patient had heart transplant as the initial procedure. There were 5 hospital deaths (31%) and no late mortality among the survivors. Survival in the cohort with a fenestrated TV patch was 80% (8/10) vs. 33% (1/3) for the non-fenestrated group. This difference did not reach statistical significance although the trend seems clinically important. There was no difference in the CT ratio (0.82 fen vs 0.84 non-fen: p=0.802) or the GOS ratio (1.2 fen vs. 1.02 non-fen: p= 0.477) between the two groups. Among the 10 survivors of RV exclusion, 2 have had completion of their Fontan, 7 have had BDG, and one is awaiting BDG Conclusions: Right ventricular exclusion with a fenestrated TV patch combined with RA and RV plasty and a BT shunt (Starnes procedure) has provided effective palliation for neonates presenting with critical Ebstein's Anomaly and a TV that cannot be repaired. Back to 86th Annual Meeting Back to Program Outline |
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