Abstract 18124: Delayed Staged Palliation Does Not Affect Clinical Outcomes for Children With Hypoplastic Left Heart Syndrome and Variants
Introduction: Impact of delayed stage II and/or Fontan palliation on survival and Fontan physiology is not well understood.
Hypothesis: Delayed staged palliation results in lower survival and worse Fontan outcomes.
Methods: All 232 patients with HLHS undergoing stage I palliation from 2000-2011 were reviewed (Figure). The clinical course for patients who had delayed stage II (>7 months) or Fontan (>3.5 years) was documented. Patients were classified as having either minor or significant reasons for delayed palliation. Rates of on-time and overall Fontan completion, survival, transplant, and post-Fontan failure were compared between patients who did not meet (G1; n=28) and met (G2; n=125) conventional stage II palliation timelines. Rates of post-Fontan failure were also compared for patients who did not meet (G3; n=44) and met (G4; n=74) conventional Fontan completion timelines.
Results: Delayed stage II palliation resulted in equivalent rates of on-time (G1: 15 [53.6%] vs G2: 59 [47.2%]; p=0.54) and delayed (G1: 10 [35.7%] vs G2: 34 [27.2%]; p=0.37) Fontan completion, with no difference noted between rates of delay because of minor or significant reasons (p<0.10). There was a trend toward greater overall Fontan completion (G1: 25 [89.3%] vs G2: 93 [74.4%]; p=0.09) in the delayed stage II group. The rates of interstage death (G1: 2 [7.1%] vs G2: 17 [13.6%]; p=0.53) and transplant (G1: 1 [3.6%] vs G2: 6 [4.8%]; p=1.00) were similar between groups. The rates of Fontan failure were comparable (G1: 1 [3.6%] vs G2: 3 [2.4%]; p=0.56). For patients having delayed Fontan, the rate of Fontan failure was comparable (G3: 1 (2.3%) vs G4: 3 (4.1%); p=1.00).
Conclusions: Delayed timing of stage II and Fontan palliation did not impact survival or Fontan failure. While close followup and sensible rehabilitation strategy are likely contributors to equivalent outcomes, this result may partly be due to patients who never reached subsequent palliation because of inter-stage death or transplant.
Author Disclosures: D. Chetan: None. C. Haller: None. S.M. Schwartz: None. C.A. Caldarone: None. G.S. Van Arsdell: None. O. Honjo: None.
- © 2016 by American Heart Association, Inc.