Trisomy 21 and functional single ventricle: Feasibility study for patients undergoing completion Fontan

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

    • OBJECTIVE: There is scarcity of data regarding management of children with trisomy 21 syndrome (T21S) and a functional single ventricle (FSV). A review of patients with T21S with FSV was conducted to identify factors that may contribute to improved outcome.

    METHODS: From September 1998 to August 2008, we identified 9 (4 males and 5 females) FSV patients with T21S among 146 who had undergone Bidirectional Glenn (BDG) and 138 Completion Fontan (CF). Mean age at BDG was 33.3+/-14.6 weeks and at CF 28.5+/-12.6 months. All patients had unbalanced complete atrioventricular canal. One had subaortic stenosis.

    RESULTS: With no hospital deaths and one mortality prior to CF no CF were taken down. Pulmonary artery banding (PAB) was the initial procedure in 8 patients and aortopulmonary shunt in 1. PAB was adjusted for PA pressure 20% of systemic. All patients progressed to pulsatile BDG and one had Damus-Kay-Stansel with BDG. Median Follow-up was 53 months (18to93). Six of 8 survivors after BDG underwent a successful CF and 2 awaiting CF. Two CF were fenestrated. Aspirin and warfarin was used postoperatively. No arrhythmias, AV node block or ventricular dysfunction were noted.

    CONCLUSION: Trisomy 21 syndrome has been reported as a risk factor in patients with FSV, mainly, due to persistent high PAP. This study shows that single ventricle pathway repair in patients with T21S can produce encouraging results. Early tight PAB and continued pulmonary vascular maturation due to pulsatile BDG may contribute to this outcome. Careful patient selection for CF is warranted.