Perinatal Outcomes in Hypoplastic Left Heart Syndrome

Abstract

Aim: To review our experience in fetuses with prenatally diagnosed hypoplastic left heart syndrome (HLHS). Materials and Method: Retrospective study of fetuses prenatally diagnosed with HLHS between 2020 and 2022 in a tertiary referral center. Results: 29 fetuses with HLHS were identified. 13 of all 29 cases (44.8%) had associated cardiac abnormalities and 8 cases (27.5%) had coexisting extracardiac abnormalities. Only a small minority of cases underwent invasive diagnostic testing (20%), and all of which had a normal karyotype. Most cases were delivered at term (58.6%), median gestational week at delivery was 37 (range, 34-39), and the mean standard deviation (SD) birthweight was 3099±455 grams. In addition, HLHS was more common in male than in female fetuses (69% vs 31%). Termination of pregnancy (TOP) was selected in 4 (13.7%) of the diagnosed 29 cases and performed before at ≤ 26 weeks of pregnancy. There were 3 intrauterine fetal demise (IUFD) at 23,29, and 34 weeks of gestation. Among the 29 cases of HLHS, 22 (75.8%) were live born. 3 of 22 live born infants were managed by medical procedures as they were not appropriate surgical candidates and later died at 22nd, 23rd and 25th of postnatal day. Surgery was performed in 15 cases and only one of them is are still alive. Mortality rate in the first three months of neonatal period was 96.5%. Conclusion: Fetal echocardiography allows an accurate diagnosis of HLHS, which is made even in the late first trimester. Despite early prenatal detection and postnatal surgical interventions, the results from our center in last two years reflect poor outcomes for HLHS.