Appropriate treatment for scimitar syndrome, a rare congenital heart defect, depends on the specifics of a patient’s case, but may involve surgery. Patients with mild cases may not need specific treatment and in fact sometimes receive a late diagnosis, while severe cases may be in obvious need of surgical management shortly after birth. In this condition, some of the freshly oxygenated blood from the lungs fails to reach the rest of the body because of a vascular abnormality. A cardiologist can evaluate the patient to determine how to proceed with treatment.
Severe cases often manifest symptoms shortly after birth, or early in childhood. Children may cry, develop fatigue easily, and struggle for breath. They can also experience bluing in the extremities, indicating that a circulatory problem is occurring and their bodies are not getting enough oxygen. A medical imaging study of the chest can show the distinctive radiographic signs associated with this condition; scimitar syndrome is named for the crescent shape created by the abnormal venous return in the chest, which looks somewhat like a scimitar.
If a patient is in distress, or the amount of blood reaching the left side of the heart is not sufficient to meet long-term needs, surgery may be necessary. In surgery, the doctor can create a connection between the right and left sides of the heart to allow the oxygenated blood to drain properly. Another option is to reposition the abnormal blood vessel. The best approach for scimitar syndrome can depend on the surgeon’s experience, the patient’s anatomy, and the specifics of the case, and may involve some discussion with a care team before developing a plan.
One potential issue with treatment for scimitar syndrome is that if the case is severe, the patient may have other abnormalities. These could create complications in surgery or might necessitate a wait on surgery for the heart problem. A careful evaluation is necessary to check for any other issues that might need to be considered when providing treatment to the patient, and sometimes genetic testing is recommended if these traits could have a familial connection.
Prospects for a patient with scimitar syndrome can be highly variable. The surgeon should be able to provide more information about the potential outcomes and what kind of recovery can be expected. Some patients lead highly active lives after the surgery, and may not experience future complications. Others may need to return for additional surgeries and followups because of complications related to other conditions or the original scimitar defect.