Outcome after prenatal diagnosis of hypoplastic left-heart syndrome: a case series

Background Traditionally, after prenatal diagnosis of hypoplastic left-hear t syndrome (HLHS) couples have been offered termination of pregnancy or com fort care. Success of postnatal surgical options such as the Norwood proced ure have been associated with survival of up to 60%. Whether survival is af fected 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 199 4 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 o verall 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 pare ntal choice and they died after compassionate care. Of the 36 babies who ha d 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 ov erall survival rate for fetuses with HLHS is 25% (if terminated pregnancies are excluded). Interpretation Fetal echocardiography allows early diagnosis of HLHS and gi ves clinicians the opportunity to triage this group dependent on prenatal f indings, including karyotyping and the exclusion of other structural anomal ies. These prospective data provide up-to-date information on the basis of which parents can make decisions.