Quality of outcome for patients with double inlet left ventricle and similar morphologies – impact of coexisting aortic arch obstruction

  • Dr Michael Seitz, The University of Sydney, Australia
  • Dr Benedict Raj, The Children's Hospital at Westmead, Sydney., Australia
  • Mr Andrew Cole, The Children's Hospital at Westmead, Sydney., Australia
  • Dr Stephen Cooper, The Children's Hospital at Westmead, Sydney., Australia
  • A/Prof Gary Sholler, The Children's Hospital at Westmead, Sydney., Australia
  • Dr Ian Nicholson, The Children's Hospital at Westmead, Sydney., Australia
  • A/Prof Richard Chard, The Children's Hospital at Westmead, Sydney., Australia
  • A/Prof David Winlaw, The Children's Hospital at Westmead, Sydney., Australia

    • Double-inlet-left-ventricle (DILV) and similar pathologies are characterised by potential for systemic outflow tract obstruction at the bulboventricular foramen. This is best managed pre-emptively by performing a Damus-Kaye-Stansel (DKS) as part of a Norwood or when performing the superior cavopulmonary connection. We have shown that the latter strategy does not produce inferior mid term results. We evaluated late outcomes using this approach with a focus on the impact of aortic arch obstruction (AAO).
    Methods
    60 consecutive patients with DILV, l-TGA and similar were identified, 1990-2007. Quality indicators noted included number of procedures, adverse events, time to Fontan completion and late ventricular function.
    Results
    19 had AAO. 12(63%) achieved Fontan, 1 is waiting and 2(11%) are not suitable for completion, 4(21%) died. 1 patient required a Norwood and has achieved completion.
    41 had no AAO. 30(73%) achieved Fontan, 8(20%) are waiting and 1 is not suitable, 1(2%) is lost, 1 died(2%).
    70% achieved Fontan within 5 years in both groups. The AAO group required significantly more operations, were more likely to be on long-term diuretics and only 50% were in NYHA class I vs 77% in the remainder.
    Conclusions
    Overall mortality for the group was acceptable, 5/60(8%), however the patients with AAO have a more protracted course with worse long-term outcome. This subset may be better served by a Norwood procedure to minimise ventricular dysfunction resulting from ‘minor’ residual arch gradients and PA banding whilst those without AAO can be well managed by a DKS as an infant rather than neonate.