Scimitar syndrome
Encyclopedia
Scimitar syndrome, or pulmonary venolobar syndrome, is a rare congenital heart defect
characterized by anomalous venous return from the right lung. This anomalous pulmonary venous return can be either partial (PAPVR) or total (TAPVR). The syndrome associated with PAPVR is more commonly known as Scimitar syndrome after the curvilinear pattern created on a chest radiograph by the pulmonary veins that drain to the inferior vena cava. This radiographic density often has the shape of a scimitar
, a type of curved sword.
. This is called the Scimitar Sign. Associated abnormalities include right lung hypoplasia with associated dextroposition of the heart, pulmonary artery hypoplasia and pulmonary sequestration.
Congenital heart defect
A congenital heart defect is a defect in the structure of the heart and great vessels which is present at birth. Many types of heart defects exist, most of which either obstruct blood flow in the heart or vessels near it, or cause blood to flow through the heart in an abnormal pattern. Other...
characterized by anomalous venous return from the right lung. This anomalous pulmonary venous return can be either partial (PAPVR) or total (TAPVR). The syndrome associated with PAPVR is more commonly known as Scimitar syndrome after the curvilinear pattern created on a chest radiograph by the pulmonary veins that drain to the inferior vena cava. This radiographic density often has the shape of a scimitar
Scimitar
A scimitar is a backsword or sabre with a curved blade, originating in Southwest Asia .The Arabic term saif translates to "sword" in general, but is normally taken to refer to the scimitar type of curved backsword in particular.The curved sword or "scimitar" was widespread throughout the Muslim...
, a type of curved sword.
Presentation
The anomalous venous return forms a curved shadow on chest x-ray such that it resembles a scimitarScimitar
A scimitar is a backsword or sabre with a curved blade, originating in Southwest Asia .The Arabic term saif translates to "sword" in general, but is normally taken to refer to the scimitar type of curved backsword in particular.The curved sword or "scimitar" was widespread throughout the Muslim...
. This is called the Scimitar Sign. Associated abnormalities include right lung hypoplasia with associated dextroposition of the heart, pulmonary artery hypoplasia and pulmonary sequestration.