OBJECTIVE: To examine the association between single umbilical artery (SUA) and cardiac defects and to determine whether patients with SUA require specialist fetal echocardiography.METHODS: Incidence and type of cardiac defects were determined in fetuses with SUA detected at routine second-trimester ultrasound examination.RESULTS: A routine second-trimester scan was performed in 46 272 singleton pregnancies at a median gestation of 22 (range, 18-25) weeks and an SUA was diagnosed in 246 (0.5%). Cardiac defects were diagnosed in 16 (6.5%) of these cases, including 10 (4.3%) in a subgroup of 233 with no other defects and in six (46.2%) of the 13 with multiple defects. In 11 (68.8%) of the 16 cases with cardiac defects the condition was readily diagnosable by evaluating the standard four-chamber view and the views of the great arteries. In the remaining cases there was left persistent superior vena cava or small ventricular septal defect, where prenatal diagnosis may not be important because they are not associated with adverse outcome.CONCLUSION: Although SUA is associated with an increased incidence of cardiac defects it may not be necessary to refer such patients for specialist fetal echocardiography because the defects are detectable by evaluating standard cardiac views that should be part of the routine second-trimester scan.