Accuracy of the fetal echocardiogram in double-outlet right ventricle

Double-outlet right ventricle (DORV) is a complex congenital heart defect with heterogeneous anatomy. Patients require a variety of surgical interventions, and the long-term prognosis is variable. Therefore, accurate prenatal diagnosis is important in providing parents with appropriate counseling.

Medical records were reviewed in patients with a diagnosis of DORV who had fetal echocardiography at our institution from 1998 to 2004. Pre- and postnatal diagnoses were compared, anticipated surgical procedure was compared with surgery performed, and neonatal outcome was assessed.

The study group consisted of 49 fetal patients with 6 in utero deaths (including 4 terminations), 2 patients lost to follow-up, and 41 live births. Postnatal echocardiograms or autopsy results were available on 42 patients. The overall accuracy of fetal echocardiography in making a correct diagnosis of DORV was 76%. Accurate prenatal prediction of the type of cardiac surgery that would be performed was made in 91% of patients who had surgery. Among live births, survival to hospital discharge was 76%. Survival was 50% in patients with extracardiac or chromosomal anomalies, compared with 92% in infants without additional anomalies.

In most cases, careful evaluation by fetal echocardiography can determine essential anatomic details in fetuses with DORV with enough accuracy to allow for accurate counseling regarding the type of surgery needed. Survival is better than previously reported but is poor in patients with extracardiac or chromosomal abnormalities.