The Single Ventricle Heart in the Fetus: Accuracy of Prenatal Diagnosis and Outcome

OBJECTIVES: The purpose of this study was to determine the diagnostic accuracy of fetal echocardiography in evaluating anatomic details of the single ventricle heart and the outcome of fetuses diagnosed with this anomaly. STUDY DESIGN: This is a retrospective study of 57 fetuses in which the results of fetal echocardiography were compared with the diagnoses at postnatal echocardiography, and postnatal surgical outcome was reviewed. RESULTS: Diagnostic accuracy was present in predicting morphology of the predominant ventricle, visceral situs, presence of pulmonary or aortic outflow tract obstruction, and presence of obstructed pulmonary venous outflow (sensitivity 100%). However, the ability to predict for a ductal dependent pulmonary circulation was poor (sensitivity 63%). Errors were made in the fetal assessment of ventricular size and viability such that in three cases, postnatal plans were altered toward a two-ventricular intervention. Of the 57 fetuses, intervention was elected in 37 (75%). Termination or nonintervention was elected in 14, and and 6 died before intervention. Of those operated on, 71% are presently alive after various stages of intervention. CONCLUSIONS: Accurate diagnosis of the fetal single ventricle heart is possible, and outcome is improving. Caution must be used in judging ventricular size and in predicting ductal dependent pulmonary circulation.