Scimitar Syndrome

Supporting Your Child’s Journey with Scimitar Syndrome

Scimitar syndrome is a rare congenital heart defect. It affects about one to three out of every 100,000 babies born alive. About twice as many girls as boys are diagnosed with the condition. Learn about baby Michael’s journey with Scimitar syndrome.

It occurs when some of the blood vessels from the right lung drain into the right side instead of going to the left side of the heart as they should. The name scimitar comes from the curved appearance of the abnormal vein, which resembles a scimitar, a type of Turkish sword. The condition is also known as congenital venolobar syndrome or hypogenetic lung syndrome.

What Causes Scimitar Syndrome?

The exact cause of Scimitar syndrome is often unknown. It develops when a fetus is growing in the uterus. Most experts believe it’s from an error in the development of the pulmonary veins and lung tissue.

Scimitar syndrome can be hereditary. Certain factors for the mother can also increase a baby's risk of being born with heart defects, including:

• Taking certain medications during pregnancy, including some antibiotics and antidepressants
• Being exposed to environmental factors like smoking or alcohol consumption during pregnancy

Signs and Symptoms

Some children with Scimitar syndrome do not have any symptoms, while others have severe complications. Infants often have more severe symptoms.

A newborn or infant with Scimitar syndrome may have:

• Difficulty breathing while feeding
• Pale or grayish skin
• Rapid breathing
• Recurrent lung infections
• Swelling around the eyes, belly or legs

Diagnosing Scimitar Syndrome

Other respiratory conditions have similar symptoms, so doctors often rely on imaging to look for the characteristic curve on a chest X-ray. In addition to X-ray, doctors also use these technologies to confirm Scimitar syndrome:

Echocardiography

This heart ultrasound can show abnormal vein drainage and any associated cardiac defects. Fetal echocardiography can sometimes diagnose the condition while the baby is still in the uterus.

Angiography

This advanced imaging technique uses contrast dye along with other imaging, such as CT or MRI, to get a detailed view of the lungs, their veins and arteries, and check blood flow.

Cardiac Catheterization

This minimally invasive procedure is not often used to diagnose Scimitar syndrome, but it can provide a more in-depth knowledge of heart and lung function, including internal pressures and can help determine the extent of the condition.

Can Scimitar Syndrome be Cured?

There’s no cure for Scimitar syndrome, but treatments can help children with the condition live a high quality of life.

How Do Doctors Treat Scimitar Syndrome?

Treatment depends on several factors, such as the severity of symptoms and whether complications such as pulmonary hypertension (high blood pressure in the lungs) are present. Usually, a team of pediatric cardiologists, cardiothoracic surgeons and pulmonologists collaborates to create the best treatment plan.

Treatment often includes:

Observation

Monitoring is often sufficient for children without symptoms. This involves regular appointments to check on your child’s development and symptoms. It may also include managing symptoms such as shortness of breath and preventing lung infections through medication and other therapies.

Surgery

Your child’s care team may recommend surgery if their symptoms are severe or interfere with their daily life. The main goal of surgery is to reroute the irregular drainage to the proper place.

Common surgical approaches include:

• Connecting the scimitar vein directly to the left atrium
• Rerouting the abnormally draining vein by creating a patch inside the heart. This is often done where there's already a hole between the heart's upper chambers.
• Closing off abnormal arteries so blood goes to the lungs instead of bypassing them.
• Surgical removal of the underdeveloped lung, if it causes significant problems for the child. This procedure is known as pneumonectomy.