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140 Evolving Technical Approach and Results in Hypoplastic Left Heart Syndrome with Intact or Highly Restrictive Atrial Septum
  1. Phuoc Duong,
  2. Angela McBrien,
  3. David Crossland,
  4. John O’Sullivan,
  5. Asif Hasan,
  6. Massimo Griselli
  1. Department and Congenital Cardiology and Surgery, Freeman Hospital, Newcastle Upon Tyne

Abstract

Background We describe our current unit approach and report interstage results in the HLHS with Intact atrial septum (IAS) or highly restrictive interatrial communication (HRIC).

Method A retrospective review of the institutional HLHS programme (2005–2014). Advances in fetal diagnosis lead to delivery planning in hybrid theatre: median sternotomy and interventional defect creation/enlargement.

Results 9 neonates (4 IAS and 5 HRIC) and 5 required immediate intervention postnatally. Delayed/insufficient septostomy in 3 HRIC resulted in death before stage I Norwood even with ECMO support in 2/3. Of the other 6, 2 had trans-atrial stent placement and 1 trans-atrial balloon septostomy (3 had surgical septectomy). 5/6 underwent concomitant bilateral pulmonary artery banding. All the 6 patients reached the Norwood procedure after 27 ± 21 days and 50% required ECMO postoperatively. Trans-atrial stenting resulted in less pre Norwood morbidities. There was no stent-related complications. There was no hospital mortality after Norwood and current inter-stage survival is 100%: 5 patients underwent successfully second-stage palliation, 1 of them had the Fontan completion and subsequently transplanted.

Conclusion Together with advanced fetal diagnosis, effective left atrial decompression especially with trans-atrial stunting using hybrid technique and availability of mechanical support can improve the outcome of HLHS/IAS/HRIC with low interstage morbidity and excellent survival.

  • HLHS/IAS
  • trans-atrial stenting
  • ECMO

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