Hypoplastic Left Heart Syndrome: Feasibility study for patients undergoing completion Fontan at or prior to 2 years of age

  • Anastasios Polimenakos, Heart Institute for Children at Advocate Hope Children's Hospital / Rush University Medical Center, United States
  • Shyam Sathanandam, Heart Institute for Children at Advocate Hope Children's Hospital, United States
  • Chawki el Zein, Heart Institute for Children at Advocate Hope Children's Hospital / Rush University, United States
  • Bettina Cuneo, Heart Institute for Children at Advocate Hope Children's Hospital, United States
  • Mary Barth, Heart Institute for Children at Advocate Hope Children's Hospital, United States
  • Robert Higgins, Rush University Medical Center, United States
  • Michel Ilbawi, Heart Institute for Children at Advocate Hope Children's Hospital, United States

    • OBJECTIVE:There are limited data regarding management of children with Hypoplastic left heart syndrome(HLHS) and completion Fontan(CF) at or prior to 2 years of age. A study was undertaken to investigate intermediate outcomes.

    METHODS:From August 1999 to December 2008, 52 HLHS-survivors underwent extracardiac-conduit CF [29 prior to (group A) vs 23 after 25 months of age(groupB)]. Mean weight and median follow-up was 9.9+/-1.3kg and 79 months(2 to 112) for groupA vs 12.1+/-2.2kg and 87 months(1 to 97) for groupB, respectively. Polytetrafluoroethylene conduits were used. Perioperative outcome variables were studied.

    RESULTS:Overall mortality was 1.9% with 1 hospital death(groupB) and no late mortality. In groupA, 16 CF had 20mm conduit(vs 18mm in 13). Thirteen groupB patients received 18mm conduit(vs 20mm in 10). No CF was taken down. Cardiopulmonary bypass was 78+/-37.3min in groupA vs 77+/-33.9min in groupB(p ns). Sixteen had fenestrated CF (8 each group, p<0.05). Mean pulmonary artery size, McGoon ratio, transpulmonary gradient in groups A vs B were 7.94+/-0.59mm vs 7.87+/-0.58(p ns), 1.79+/-0.2 vs 1.77+/-0.19(p ns), 4.1+/-1.1mmHg vs 3.5+/-0.9(p ns). Aspirin and warfarin were used postoperatively. Mean hospital length of stay(LOS), ICU-LOS, pleural drainage duration between groupA and B were 10.9+/-5.8days vs 12.7+/-6.1(p ns), 5.6+/-2.6days vs 6.7+/-2.9(p ns), 7.6+/-3.8days vs 8.7+/-4.1(p ns), respectively. Between groups no difference in ventilatoty support time, arrhythmias, sinus/AV node dysfunction, protein-losing-enteropathy and thromboembolic events was noted.

    CONCLUSION:In HLHS patients CF can be performed with good intermediate results at or prior to 2 years of age. Age at CF had no effect in conduit size selection.