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A Contemporary Comparison of the Effect of Shunt Type in Hypoplastic Left Heart Syndrome on the Hemodynamics and Outcome at Fontan Completion
Jean A. Ballweg1, Troy E. Dominguez1, Chitra Ravishankar1, Peter J. Gruber1, Gil Wernovsky1, J. W. Gaynor1, Susan C. Nicolson1, Thomas L. Spray1, Sarah Tabbutt2; 1Children's Hospital of Philadelphia, Philadelphia, PA; 2University of California San Francisco, San Francisco, CA
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Objective: We previously reported no difference in morbidity or mortality in infants undergoing stage 1 and stage 2 reconstruction with either a modified BT shunt (mBTS) or a right ventricular to pulmonary artery conduit (RV-PA). We now compare the hemodynamics and peri-operative course at the time of the Fontan completion and report longer-term survival.
Methods: We retrospectively reviewed the echocardiograms, catheterizations and hospital records of all patients who previously underwent stage 1 reconstruction (S1R) between January 2002 and May 2005 and subsequent surgical procedures, as well as cross-sectional analysis of hospital survivors.
Results: 176 pts with HLHS and variants underwent initial S1R with either mBTS (n = 114) or RV-PA conduit (n = 62). The median duration of follow-up was 53 months (range 1 - 76). By Kaplan-Meier analysis, shunt type did not influence survival or freedom from transplant at 5 years (RV-PA 61%, 95% CL: 47-72% vs. mBTS 70%, 95% CL: 60-77%, p = 0.55). Nintey three pts underwent Fontan (62 mBTS and 31 RV-PA) with 98% (91/93) early survival. Pre-Fontan there was a trend towards higher pulmonary artery pressure (13 ± 8 mmHg vs. 11 ± 3 mmHg, p = 0.05) and common atrial pressure (8 ± 2 mmHg vs. 7 ± 2 mmHg, p = 0.06) in pts with RV-PA conduits. By echo evaluation, there was a trend towards more qualitative moderate to severe ventricular dysfunction (RV-PA 31% (11/35) vs. mBTS 17% (11/65), p= 0.08) and moderate to severe atrioventricular valve regurgitation (RV-PA 38% (13/34) vs. mBTS 17% (11/65), p=0.07) in the RV-PA group. Use of diuretic therapy, ACE inhibition, reflux medications and tube feedings were no different between groups. There was a trend towards increased digoxin use in the RV-PA group (RV-PA 71% (25/35) vs. 65% mBTS (45/69), p = 0.06). Overall 5 pts underwent heart transplantation (RV-PA 4 vs. mBTS 1, p= 0.1) prior to Fontan. There was no difference in age or weight at Fontan, bypass time, ICU or hospital length of stay, post-operative pleural effusions or need for reoperation between groups.
Conclusion: Interim analyses continue to suggest that there is no advantage of one shunt type over another. Longer term follow-up of a randomized patient population remains of utmost importance.
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