Figure 1. Artist's rendition of the anomalous right pulmonary vein entering the IVC below the diaphragm. Reprinted with permission from the Society of Thoracic Surgeons.^[11]

Figure 2. Case 1 (A.B.). The cardiac silhouette is shifted to the right with opacification of the right hemithorax and left upper lobe atelectasis. Note left stomach bubble.

Figure 3. Case 2 (C.D.). Note right lung opacification, cardiac dextroposition, increased left pulmonary vascular markings, hemivertebrae (arrowhead), and rib anomalies (arrows).

Figure 4. Case 3 (E.F.) with complete right lung opacification and cardiac dextroposition.

Figure 5. Case 4 (G.H.). Note mediastinal shift with trachea and endotracheal tube deviated to the right and rib anomalies (arrows).

Figure 6. Case 5 (I.J.) at 27 months of age. Note right mediastinal shift and the anomalous right pulmonary vein, the so-called scimitar sign (arrows), visible through the cardiac silhouette.

Figure 7. Two-dimensional echocardiogram showing the anomalous right pulmonary vein (scimitar vein) entering the RA near the RA-IVC junction. See the corresponding video clip for the real-time echocardiogram at www.advancesinneonatalcare.org.

Figure 8. Pulmonary venous angiogram with the catheter in the RPA showing the anomalous scimitar vein draining from the right lung into the IVC and filling the RA.

Figure 9. (A) Angiograms with the catheter in the descending aorta showing the anomalous systemic arterial supply from the descending aorta to the right lower lobe. (B) Same patient after coil (arrows) embolization of the anomalous systemic artery collateral.

Figure 10. A technique used to repair scimitar syndrome by diverting the anomalous pulmonary venous drainage from the IVC into the LA. The anomalous pulmonary venous drainage is directed from its orifice in the IVC to the LA through the ASD using a long pericardial patch. Reprinted with permission from the Society of Thoracic Surgeons.^[11]

Figure 11. Another technique used to repair scimitar syndrome by diverting the anomalous pulmonary venous drainage from the IVC into the LA. The anomalous pulmonary vein has been divided off the IVC and reimplanted into the posterior wall of the RA. A short pericardial baffle is then used to divert flow to the LA through an ASD. Reprinted with permission from the Society of Thoracic Surgeons.^[11]