Familial Scimitar Syndrome
Three-Dimensional Visualization of Anomalous Pulmonary Vein in Young Sisters
Scimitar syndrome is a rare congenital disorder characterized by an anomalous connection of the pulmonary vein with the inferior vena cava. The anomalous vein appears as a “scimitar”-like shadow on a chest x-ray. We recently encountered Scimitar syndrome in 2 sisters and demonstrated the 3D structure of the anomalous vein by computed tomography (CT).
A 27-year-old woman was referred to our hospital for further evaluation of an abnormal shadow in the right lower lung field on chest x-ray (Figure 1⇓). She was asymptomatic. The 3D CT clearly demonstrated an abnormal pulmonary vein that drained into the suprahepatic inferior vena cava (Figure 2⇓). Cardiac catheterization showed a mean pulmonary artery pressure of 15 mm Hg, a pulmonary/systemic flow ratio of 1.86, and a left-to-right shunt ratio of 46%. Corrective surgery was not performed because of her lack of symptoms and relatively low shunt ratio.
This patient was the 31-year-old sister of the patient described in case 1. She also had an abnormal shadow in the right lower lung field on chest x-ray (Figure 3⇓). She had mild dyspnea on exertion, which began at ≈20 years of age. The 3D CT demonstrated an abnormal pulmonary vein, which drained into the suprahepatic inferior vena cava (Figure 4⇓). On cardiac catheterization, the mean pulmonary artery pressure was 16 mm Hg, the pulmonary/systemic flow ratio was 2.05, and the left-to-right shunt ratio was 53%. The patient subsequently underwent surgery to switch the anomalous pulmonary vein to the left atrium.
- Copyright © 2001 by American Heart Association