(733) Perinatal outcomes in pregnancies complicated by a prenatal diagnosis of critical congenital heart disease (CCHD)

Prenatal diagnosis of CCHD allows for optimal perinatal decision-making. We sought to examine care pathways and perinatal outcomes on prenatally and postnatally diagnosed cases over a four-year period.

Study Design:

This retrospective cohort study sought to determine the perinatal outcome of pregnancies complicated by CCHD detected prenatally and postnatally from 01/2019 to 12/2022. Patients attending a tertiary perinatology center in Ireland and its affiliated secondary centers underwent mid-trimester fetal cardiac ultrasound examination. All pregnancies diagnosed with CCHD were referred prenatally to the Fetal-medicine/cardiology service, offered genetic testing and counselling. Data sources included retrospective electronic chart and ultrasound report review and neonatology/pediatric records. All cases were treated in a single national pediatric cardiac center. CCHD was defined as duct-dependency or structural abnormality requiring urgent intervention in the 1st month of life.

Results:

Among 49,950 screened pregnancies, the incidence of CCHD was 0.21% (104/49,950) with a prenatal detection rate of 91% (95/104). In the prenatally diagnosed group, 41% of cases were complicated by extracardiac structural abnormalities, a genetic diagnosis or both. 10% chose pregnancy termination, 29% opted for perinatal palliation and 7% died in utero. Among 67 cases evaluated for neonatal cardiac intervention, 17 (25%) were deemed not surgical candidates.  All remaining neonates 52/67 (78%) had surgery or catheterization or both in the first month of life. Survival to Day 30 of life was 88% (46/52) and 85% (44/52) at 1 year in the prenatally diagnosed cohort. All 9 cases of CCHD first diagnosed in the postnatal period (all of whom had aortic coarctation or critical pulmonary stenosis) were alive at 1 year.

Conclusion:

40% of all pregnancies complicated by CCHD will not undergo neonatal cardiac intervention, by virtue of parental choice determined either prenatally or after birth. Survival at one year for infants deemed suitable for CCHD surgery is in excess of 85%.