Scimitar Syndrome is a rare congenital heart defect characterized by Partial (PAPVR) or Total (TAPVR) Anomalous Pulmonary Venous Return. The syndrome gets its name from the curved shape formed by the pulmonary vein. On a chest radiograph, the vein is said to look like a scimitar, a curved sword originating in the Middle East.
The abnormal pulmonary vein may be connected to the inferior vena cava or directly to the right atrium, sending already oxygen-rich blood into the chamber that sends blood to the lungs. Because this is an inefficient way for the heart to work, the heart can become enlarged and weakened. Untreated, this CHD can lead to dyspnea (breathlessness), atrial arrhythmias, pulmonary hypertension, and heart failure.
Other components of scimitar syndrome include dextrocardia and hypoplasia. Dextrocardia, the severe rotation of the heart onto the right side of the chest or the mirror-image presentation of the heart, requires no surgical intervention. Hypoplasia, however, can be associated with recurrent bouts of pneumonia, and in some cases, pneumonectomy or removal of the hypoplastic lung is necessary.
For some patients, conservative treatment is an option. But if more than half of the pulmonary veins empty into the right atrium, it may be necessary to reroute blood flow into the left atrium. This is achieved by surgically constructing a “baffle” or tunnel out of the patient’s own pericardium, creating a pathway through the right chamber and into the left.
A different approach may be warranted for patients with a large systemic collateral artery. Coil occlusion, the placement of a coil to block blood flow, can improve symptoms of heart failure in infants with scimitar syndrome and can be a good surgical correction for this condition.
On a personal note, Scimitar Syndrome is the heart condition that my own child has battled. See these related articles on this website: Dr. Edward Bove, Nathan’s Prayer, About, Partial Anomalous Pulmonary Venous Return, A Memory of Scimitar Syndrome, Three Year Anniversary.
Also see my article in Parents magazine: “Nathan’s Wonder Slide”
Does your child have Scimitar Syndrome? Is he/she facing surgery? Consider adding your child to our prayer list.
Tags: baffle, CHD, coil occlusion, Congenital Heart Defects, dextrocardia, heart, hypoplasia, Nathan, Nathan's Prayer, Nathan's Wonder Slide, PAPVR, Parents Magazine, Partial Anomalous Pulmonary Venous Return, Scimitar Syndrome, TAPVR