Hypoplastic left heart syndrome: a novel surgical strategy for small-volume centres?

Eur J Cardiothorac Surg. 2017 May 1;51(5):1003-1008. doi: 10.1093/ejcts/ezx021.

Abstract

Objectives: We describe in a prospective study, a novel surgical technique for the management of hypoplastic left heart syndrome inspired by the hybrid Norwood approach.

Methods: This new neonatal palliation comprises replacement of the patent ductus arteriosus (PDA) and aortic arch plasty with a pulmonary homograft associated with the banding of both pulmonary arteries and atrial septectomy, under cardiopulmonary bypass without aortic clamping and cardioplegia. Initial results led to tightening of the pulmonary artery band from 3.5 mm to 2.5 mm.

Results: From July 2014 to May 2016, 15 patients were initially palliated (13 patients with aortic atresia/mitral atresia, 1 with double inlet right ventricle, 1 with tricuspid atresia) at a median age of 5 days (1-8 days) and a median weight of 3.0 kg (2.3-3.9 kg). All but 2 patients were discharged from the hospital: There was 1 early in-hospital death and 1 patient required hospitalization in the intensive care unit until stage 2. There were also 4 interstage deaths. Nine patients underwent Stage 2 and 1 patient is still waiting. Compared to the classic Norwood procedure, early mortality decreased significantly (43.3-6.7%; P = 0.0074) and overall mortality before Stage 2 was halved (61.6-33.3%). Postoperative morbidity was also reduced.

Conclusions: This new surgical palliative approach to hypoplastic left heart syndrome, particularly useable technically, seems to combine the advantages of the hybrid procedure by avoiding cardiac ischaemia and ventriculotomy without the complications of PDA stenting and restrictive atrial septectomy. Although the mortality rate decreased significantly, it remains substantial in small-volume centres, especially in the interstage period.

Keywords: Hybrid Norwood procedure; Hypoplastic left heart syndrome; Norwood procedure; Pulmonary artery banding; Pulmonary homograft.

MeSH terms

  • Allografts / surgery
  • Allografts / transplantation
  • Aorta, Thoracic / surgery
  • Female
  • Humans
  • Hypoplastic Left Heart Syndrome / mortality
  • Hypoplastic Left Heart Syndrome / surgery*
  • Infant, Newborn
  • Male
  • Norwood Procedures* / adverse effects
  • Norwood Procedures* / methods
  • Norwood Procedures* / mortality
  • Norwood Procedures* / statistics & numerical data
  • Postoperative Complications
  • Prospective Studies
  • Pulmonary Artery / surgery
  • Pulmonary Artery / transplantation