Diagnosis and prognosis of fetuses with double outlet right ventricle.

OBJECTIVES: To examine the echocardiographic features, associated anomalies, and outcomes of fetuses diagnosed with double outlet right ventricle (DORV).

METHODS: Fetuses with DORV were identified from 987 fetal echocardiograms performed from August 1, 2002 to August 1, 2004. The medical records of 19 fetuses with DORV were reviewed.

RESULTS: Nineteen fetuses were diagnosed with DORV. The pregnancy was terminated in 15.8% (3/19) of cases. Postnatal assessment was not obtained in 26.3% (5/19) cases. Accurate prenatal diagnosis of the relationship of the ventricular septal defect (VSD) with the great arteries was achieved in 14 of 14 cases (100%). Chromosomal abnormalities (21.4%), abnormalities of body situs (35.7%), and other extracardiac abnormalities were diagnosed in association. Only 4 of 13 (30.8%) infants with confirmed DORV survived beyond 28 days.

CONCLUSIONS: DORV is found in fetuses with a large spectrum of associated cardiac and extracardiac lesions. The overall prognosis for fetuses with DORV is poor throughout the spectrum of these lesions, not only related to the primary lesion but also depending on the associated intra- and extracardiac abnormalities. While the complexity of the heart disease in fetuses with DORV makes detailed fetal diagnosis challenging, it can be accomplished with a high degree of accuracy.