Antenatal perspective of hypoplastic left heart syndrome: 5 years on

Shree Vishna Rasiah ¹, Andrew K Ewer ^1*, Paul Miller ², John G Wright ², David J Barron ², William J Brawn ² and Mark D Kilby ¹

¹ Birmingham Women's Hospital NHS Trust, United Kingdom
² Birmingham Children's Hospital NHS Trust, United Kingdom

^* To whom correspondence should be addressed. E-mail: a.k.ewer{at}bham.ac.uk.

Accepted 19 June 2007

Abstract

Introduction:Palliative staged reconstructive surgery has radically altered the outcome of babies with hypoplastic left heart syndrome (HLHS). We had previously reviewed the outcome of babies diagnosed antenatally with classical HLHS. We undertook this review 5 years later, when paediatric cardiothoracic and postnatal paediatric intensive care techniques have been further refined.

Aim:To compare the current outcome of antenatally diagnosed HLHS with our previous series.

Method:We reviewed all cases of antenatally diagnosed HLHS at Birmingham Women’s Hospital between 01/01/2000 and 31/12/2004 and compared these to our previous series.

Results:79 fetuses were identified with HLHS. The median gestational age at diagnosis was 22 weeks. Following counselling, 25.3% of couples chose to terminate the pregnancy which was significantly lower then the previous cohort 43.7% (p=0.01). Of the 59 couples who continued with the pregnancy there were 4 stillbirths and 2 cases lost to follow-up. Subsequently there were 53 live births of whom 6 babies had an alternative major congenital heart disease diagnosed postnatally. 10 babies were not considered for surgery because of parental wishes and died after compassionate care. Finally, 31 babies underwent surgery. The early (30 days) surgical mortality following Stage 1 Norwood procedure was 19.4% and 20 patients are still alive to date. In the cohort of intention-to-treat cases, the overall survival was 46.9% (23/49).

Conclusion:Five years on, the number of parents choosing termination after an antenatal diagnosis of HLHS has almost halved. 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 when compared with those identified postnatally.

Keywords: antenatal diagnosis, hypoplastic left heart syndrome, outcome

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