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Antenatal perspective of hypoplastic left heart syndrome: 5 years on
  1. S V Rasiah1,2,3,4,
  2. A K Ewer1,2,3,4,
  3. P Miller1,2,3,4,
  4. J G Wright1,2,3,4,
  5. D J Barron1,2,3,4,
  6. W J Brawn1,2,3,4,
  7. M D Kilby1,2,3,4
  1. 1
    Department of Neonatology, Birmingham Women’s Hospital NHS Trust, Birmingham, UK
  2. 2
    Department of Paediatric Cardiology, Birmingham Children’s Hospital NHS Trust, Diana, Princess of Wales Children’s Hospital, Birmingham, UK
  3. 3
    Department of Paediatric Cardiac Surgery, Birmingham Children’s Hospital NHS Trust, Diana, Princess of Wales Children’s Hospital, Birmingham, UK
  4. 4
    Department of Fetal Medicine, Birmingham Women’s Hospital NHS Trust, Birmingham, UK
  1. Dr A K Ewer, Department of Neonatology, Birmingham Women’s Hospital NHS Trust, Edgbaston, Birmingham B15 2TG, UK; a.k.ewer{at}bham.ac.uk

Abstract

Background: Palliative staged reconstructive surgery has radically altered the outcome of babies with hypoplastic left heart syndrome (HLHS).

Aim: To compare the current outcome of antenatally diagnosed HLHS with a series 5 years previously now that paediatric cardiothoracic and postnatal paediatric intensive care techniques have been further refined.

Method: Comparison of all cases of HLHS diagnosed antenatally at Birmingham Women’s Hospital between 1 January 2000 and 31 December 2004 with results of the previous series.

Results: 79 fetuses were identified with HLHS. The median gestational age at diagnosis was 22 weeks. After counselling, 20 (25.3%) couples terminated the pregnancy compared with 43.7% in the previous cohort (p = 0.01). Of the 59 couples who continued with the pregnancy, four had stillbirths and two were lost to follow-up. Subsequently, there were 53 live births, of which six babies had an alternative major congenital heart disease diagnosed postnatally; 10 babies were not considered for surgery (parents’ wishes) and died after compassionate care; 31 babies underwent surgery. The early (30 days) surgical mortality after stage 1 Norwood procedure was 19.4% and 20 patients are still alive. In the cohort of intention-to-treat cases, the overall survival was 46.9% (23/49).

Conclusion: The number of parents choosing termination after an antenatal diagnosis of HLHS has almost halved since 5 years ago. Despite the significant increase in surgical survival following stage 1 Norwood in this period, in the intention-to-treat cohort the survival was 46.9%. These data again highlight the poorer outcome for babies with congenital malformations diagnosed in utero in comparison with those identified postnatally.

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Footnotes

  • Competing interests: None.

  • Ethical approval: Ethical approval was obtained from the South Birmingham Local Research Ethics Committee and the Birmingham Women’s Hospital, Research and Development Committee.

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