Author + information
- Shilpa Shah,
- Steven Schwartz,
- Andrew Goodwin,
- Osami Honjo,
- Glen Van Arsdell,
- Mike Seed,
- Jennifer Russell and
- Alejandro Floh
Background: In efforts to improve survival following the stage 1 Norwood operation, our institution adopted a comprehensive treatment approach that included standardization of surgery, perioperative management, and a dedicated interstage home surveillance program. We hypothesized this would improve one-year survival with preserved ventricular function (VF) and atrio-ventricular valve function (AVVF) in patients undergoing the Norwood operation.
Methods: This retrospective cohort study compared patients who underwent stage 1 palliation October 2012-January 2015 (Group 1) with historical (Group 2) controls. Patients’ clinical status was extracted from the medical record. VF and AVVF were determined from echocardiograms closest to one year of age. VF was preserved if normal or mildly reduced and AVVF if mildly regurgitant or better. The primary outcome was transplant-free survival with preserved ventricular function (VF) and AVV function at 1 year.
Results: 69 patients (27 Group 1, 42 Group 2) were included. More group 1 patients had diagnosed extracardiac syndromes (5% vs 22%, p=0.05). At stage 1, group 1 had longer circulatory arrest times (26 vs 19 min p=0.02), decreased 14-day freedom from open chest (6.3 vs 8.6 days, p=0.01), and a decrease in 100 hospital-free days (40 vs 55 days p=0.01). Group 1 had a trend toward higher transplant-free survival at stage 2 (85% vs 73%, p=0.25). At 1 year follow-up (median 14 months, IQR 11-18), there was no significant difference in transplant-free survival (70.4% vs 64%, p=0.6) or transplant-free survival with preserved VF and AVVF (55% vs 52%; group 1 vs 2; p=0.627). Of survivors without preserved VF/AVVF, 83% had moderate or severe AVVR and 16% had moderate ventricular dysfunction. Regression analysis demonstrated that shorter duration of open chest (p=0.01) and inclusion in group 1 (p=0.04) were predictors of good VF/AVVF at 1 year.
Conclusions: Implementation of a structured comprehensive management strategy was not associated with improved transplant-free survival at one year, with or without preserved VF and AVVF. A paradigm shift in treatment strategy is required to improve long term survival in this vulnerable population.
Poster Hall, Hall C
Saturday, March 18, 2017, 9:45 a.m.-10:30 a.m.
Session Title: Congenital Heart Disease: Innovations in Single Ventricle Heart Disease
Abstract Category: 10. Congenital Heart Disease: Pediatric
Presentation Number: 1182-017
- 2017 American College of Cardiology Foundation