Impact of prenatal diagnosis on survival and early neurologic morbidity inneonates with the hypoplastic left heart syndrome

Background. Prenatal echocardiography can identify the fetus that has compl ex congenital heart disease and may improve early management and surgical o utcome. Prenatal diagnosis may be particularly beneficial to patients who h ave hypoplastic left heart syndrome (HLHS) and who are at risk for hypoxic- ischemic insult at presentation. Objectives. We sought to determine whether prenatal diagnosis reduces neuro logic morbidity and operative mortality in patients who undergo palliative surgery for the HLHS. Methods. Data from all patients who had HLHS, except for those with lethal genetic anomalies, and who were admitted to our institution between July 19 92 and September 1997 were analyzed to assess the impact of prenatal diagno sis on preoperative management, neurologic morbidity, and surgical mortalit y. The primary outcome measures were hospital mortality and the incidence o f adverse neurologic events (seizure or coma). Results. There were 216 patients who had HLHS and were referred for surgica l palliation, 79 (36.6%) of whom had been diagnosed prenatally. All patient s who had been diagnosed prenatally were delivered in an advanced nursery a nd were started on prostaglandin E-1 on the first day of life. Patients who se HLHS was diagnosed postnatally were begun on prostaglandin E1 later in l ife (median = day 2 [range = 1- 28 days]). There were 4 preoperative deaths and 53 operative or postoperative deaths. Overall hospital mortality was 2 6.4% and did not differ between patients whose HLHS had been diagnosed pren atally and those whose HLHS had been diagnosed postnatally. With the use of multivariable analysis, prenatal diagnosis was associated with fewer adver se perioperative neurologic events in the patients whose HLHS had been diag nosed prenatally than in those whose HLHS had been diagnosed postnatally (o dds ratio = 0.46). Conclusions. These data suggest that prenatal diagnosis has a favorable imp act on treatment of patients who have HLHS and are undergoing staged pallia tion and reduces early neurologic morbidity. Prenatal diagnosis was not ass ociated with reduced hospital mortality. It is possible that prenatal diagn osis may improve long-term neurologic outcome.