A 5-year-old girl is found on routine examination to have a pulmonic flow murmur, fixed splitting of P2 and a right ventricular lift. The following is/are true:

b. Radiology report of “scimitar syndrome” findings on the chest film would indicate need for an arteriogram

The findings suggest an atrial septal defect (ASD) that can be confirmed by 2D echocardiography eliminating the need for cardiac catheterization. The ostium secondum type defect is most commonly found, but it is the sinus venosus type that is associated with anomalous pulmonary venous drainage. In the scimitar syndrome, the anomalous pulmonary vein can be seen on a chest radiograph and, since these are associated with a hypoplastic lung that is supplied by an anomalous systemic artery from the aorta, an arteriogram is appropriate. An ASD with a significant left-to-right shunt as demonstrated by a Qp/Qs ratio in excess of 1.5 should be repaired. When the pulmonary vascular resistance is elevated above 10–12 Woods units/m2 the patient is not a candidate for repair due to fixed pulmonary hypertension.