Scimitar syndrome presenting in infancy

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Scimitar syndrome presenting in infancy Charles B Huddleston, MD, Vernat Exil, MD, Charles E Canter, MD, Eric N Mendeloff, MD The Annals of Thoracic Surgery Volume 67, Issue 1, Pages 154-159 (January 1999) DOI: 10.1016/S0003-4975(98)01227-2

Fig 1 Frame taken from the cardiac catheterization of one of the patients. The catheter travels up the inferior vena cava into the anomalous pulmonary vein. The dye demonstrates the vein entering the inferior vena cava at a level below the diaphragm. Although it is difficult to determine, the vein overlies the right heart border so that it could not be seen distinct from the cardiac silhouette. There was no “scimitar sign” on the plain chest x-ray. The Annals of Thoracic Surgery 1999 67, 154-159DOI: (10.1016/S0003-4975(98)01227-2)

Fig 2 (A) An artist’s rendition of the anomalous pulmonary venous drainage of the right lung entering the inferior vena cava below the diaphragm, just above the hepatic veins. (B) The pulmonary vein has been divided off the inferior vena cava taking a bit of the wall of the inferior vena cava with it. It is brought up through the diaphragm and opened longitudinally to enlarge the orifice. It is then sewn to an opening in the mid-portion of the posterolateral wall of the right atrium. The defect in the inferior vena cava is patched with either pericardium or synthetic material. Looking into the right atrium through an atriotomy in the anterior wall, the newly created orifice of the anomalous pulmonary vein is baffled to the left atrium through an atrial septal defect using pericardium or synthetic material. The Annals of Thoracic Surgery 1999 67, 154-159DOI: (10.1016/S0003-4975(98)01227-2)

Fig 3 The anomalous pulmonary venous drainage is directed from its orifice in the inferior vena cava to the left atrium through the atrial septal defect. A very large patch of pericardium or synthetic material is necessary for this to reach the atrial septal defect. The right atriotomy nearly always needs to extend down into the inferior vena cava and this usually needs to be patched to enlarge it to allow for the space taken up by the baffle inside the inferior vena cava. The Annals of Thoracic Surgery 1999 67, 154-159DOI: (10.1016/S0003-4975(98)01227-2)

Fig 4 This is a typical chest roentgenogram on one of the infants in our series. Note how the cardiac silhouette is displaced to the right. This occurs to such a degree that it is unusual to see a “scimitar sign” in these young patients. The Annals of Thoracic Surgery 1999 67, 154-159DOI: (10.1016/S0003-4975(98)01227-2)

Fig 5 A straight lateral view of an angiogram of the anomalous right pulmonary vein. The vein in this patient ran a course through the lung that was posterior to the hilum and was not amenable to reimplantation higher into the right atrium. One can note that the vein lies even posterior to the esophagus, which is represented by the radiopaque line on the nasogastric tube marked by the arrow. The Annals of Thoracic Surgery 1999 67, 154-159DOI: (10.1016/S0003-4975(98)01227-2)