OUTCOME AFTER PRENATAL-DIAGNOSIS OF THE HYPOPLASTIC LEFT-HEART SYNDROME

Objective-To derive figures in the current surgical era for counsellin g in early pregnancy after the diagnosis of fetal hypoplastic left hea rt syndrome. Setting-A tertiary referral centre for paediatric cardiol ogy and cardiac surgery. Design-A retrospective study of the outcome i n all cases of hypoplastic left heart syndrome presenting in fetal lif e between mid-1993 and the end of 1996. Patients-The diagnosis was mad e in 30 fetuses. In four of 12 identified before 24 weeks' gestation t he mothers chose to terminate the pregnancy. There was an intention to treat in 24 of the remaining fetuses. Main outcome measure-Survival t o six months of postnatal life. Results-Of the 24 infants, five were n ot offered Norwood stage 1 because of trisomy 18 (n = 2), unfavourable cardiac anatomy (n = 2), or neurological impairment (n = 1). One furt her infant did not survive to cardiac surgery after gastrointestinal s urgery. Of the remaining 18 patients, eight had features that were con sidered to increase the risk of surgical repair. Of the 18 patients wh o underwent Norwood stage 1, there were nine survivors. There was a su rvival rate of 70% in infants undergoing surgery with no complicating features, a 50% survival of the all surgical candidates, and 37.5% sur vival from an intention to treat position. Conclusions-At the initial diagnosis of fetal hypoplastic left heart syndrome, the overall surviv al appears to be less than 40%. Evaluation must include detailed extra cardiac and intracardiac assessment to predict the risks of surgical t reatment. Prenatal counselling can be modified as pregnancy advances, depending on the detection or exclusion of complicating factors.