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Re-coarctation Following Stage 1 Reconstruction Does Not Adversely Affect Survival or Outcome at Fontan Completion
Jean A Ballweg1, Troy E Dominguez1, Chitra Ravishankar1, Peter J Gruber1, Gil Wernovsky1, Jonathan J Rome1, Matthew J Gillespie1, J William Gaynor1, Susan C Nicolson1, Thomas L Spray1, Sarah Tabbutt2
1Children's Hospital of Philadelphia, Philadelphia, PA;2University of California San Francisco, San Francisco, CA
BACKGROUND:
To determine the effect of re-coarctation following stage 1 reconstruction (S1R) for hypoplastic left heart syndrome (HLHS) and variants on survival, suitability for Fontan and morbidity at Fontan.
METHODS:
Retrospective review of echocardiograms, catheterizations and hospital records of patients who underwent S1R from January 2002 to May 2005 and cross-sectional analysis of hospital survivors. Kaplan Meier curves were derived for all patients who were alive > 30 days after S1R (n = 146).
RESULTS:
176 patients underwent S1R. 43 patients (23%) developed re-coarctation and underwent balloon angioplasty (BA) (n = 43) and/or surgical intervention (n = 4). Median time to intervention was 123 (1 - 316) days. 7/43 (16%) patients underwent >1 BA. 39/43 (91%) patients underwent intervention prior to second stage reconstruction (S2R) and 4 patients underwent BA between S2R and Fontan. Freedom from death or transplant was no different between those patients who developed re-coarctation requiring intervention compared to those without re-coarctation (p = 0.24). 100 patients have undergone Fontan. Need for reintervention did not decrease suitability for Fontan completion. Echocardiographic data was available for 98 patients prior to Fontan. There was no difference in prevalence of qualitative moderate or severe ventricular systolic function between those with re-coarctation and those without (2/33 (6%) vs. 4/65 (6%), p = 0.86) Qualitatively significant atrioventricular valve regurgitation was more common in patients with re-coarctation than in those without (28/33 (85%) vs. 40/65 (62%), p = 0.02). Median filling pressures at pre-Fontan catheterization were significantly higher in patients who had re-coarctation 8 (5-12) mmHg vs. those without 6 (3-15) mmHg, p = 0.035. Overall mortality following Fontan was 2% and was not different between groups. Length of stay at time of the Fontan was not different between those patients with re-coarctation 8.5 (2-73) days vs. those without 8 (3-37) days, p = 0.86.
CONCLUSIONS:
Re-coarctation following S1R is common. Although patients who underwent reintervention had higher filling pressures and a higher likelihood of significant atrioventricular valve regurgitation, this did not affect their suitability for the Fontan procedure, mortality or hospital length of stay. Continued follow up is necessary to determine the impact of re-coarctation on longer term mortality, morbidity and quality of life. 
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