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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. [ABSTRACT FROM AUTHOR]

The article presents an abstract of the study "Outcome of prenatally diagnosed hypoplastic left heart syndrome between 1994 and 1999." The study uses data of all cases of prenatally diagnosed hypoplastic left heart syndrome referred to Fetal Medicine Unit at Birmingham Women's Hospital between 1994 and 1999. The study finds that 36 out of 87 couples opted for abortion after diagnosis of hypoplastic left heart syndrome using fetal echocardiography.

SummaryBackground: Traditionally, after prenatal diagnosis of hypoplastic left-heart syndrome (HLHS) couples have been offered termination of pregnancy or comfort care. Success of postnatal surgical options such as the Norwood procedure have been associated with survival of up to 60%. Whether survival is affected by the congenital anomaly being identified prenatally or postnatally remains uncertain.Methods: We reviewed all cases of prenatally diagnosed HLHS referred to the Fetal Medicine Unit at Birmingham Women's Hospital over 6 years between 1994 and 1999.Findings: 87 cases of HLHS were referred at a median gestational age (95% CI) of 23 (19-37) weeks. Of these, 53 (61%) chose prenatal karyotyping. The overall frequency of abnormal karyotype was found in seven of 59 cases (12%) and associated structural anomalies in 18 of 87 (21%). After counselling, 38 of 87 couples (44%) chose termination of pregnancy. Of the remaining 49 fetuses, 11 (23%) were not considered for postnatal surgery because of parental choice and they died after compassionate care. Of the 36 babies who had surgery postnatally, 12 survived (33%). We recorded a survival rate of 38% for the stage-1 Norwood procedure in the prenatally diagnosed HLHS in our centre. These data suggest that at the point of prenatal detection, the overall survival rate for fetuses with HLHS is 25% (if terminated pregnancies are excluded).Interpretation: Fetal echocardiography allows early diagnosis of HLHS and gives clinicians the opportunity to triage this group dependent on prenatal findings, including karyotyping and the exclusion of other structural anomalies. These prospective data provide up-to-date information on the basis of which parents can make decisions. [ABSTRACT FROM AUTHOR]