Shone's Syndrome Treatment- Cardiology

Shone’s syndrome treatment is used to correct restricted blood flow through the left chambers of the heart.

Shone’s syndrome (Shone's complex or Shone's anomaly) is a rare disorder of the heart. It is present at birth. As the child grows, they may have symptoms like fatigue, coughing at night, and wheezing.

A child with Shone’s syndrome has problems with blood flow from the left upper chamber (L. atrium) to the left lower chamber (L. ventricle), and from the left ventricle to the aorta. The aorta is the largest blood vessel originates begins at the top of the left ventricle.

Complications include elevated blood pressure, abnormal heartbeats, and heart failure. The treatment includes surgery to remove the lesions that cause blockages in the blood flow.

Diagnosis of Shone’s Syndrome

Usually, the diagnosis begins with the assessment of clinical symptoms. Doctors diagnose most cases within the first few weeks of life.   

During a physical examination, the doctor may notice crackling sounds made by the lungs, a rapid heart rate, and abnormal heart sounds.

Imaging tests are necessary for confirming the condition. Thus, your doctor may order the following tests.

• Echocardiography. This test uses sound waves to create moving images of the heart. Your doctor can detect abnormal functions of the heart and its valves using echocardiography.
• MRI. MRI uses a powerful magnet and radio waves to create detailed images of the heart. It allows the doctor to see if the blood flow through blood vessels is normal.
• Cardiac catheterization. During this procedure, the doctor inserts a thin tube (catheter) into a large blood vessel. The tube is slowly moved through the blood vessel to reach the heart.

Moreover, the doctor may diagnose the condition before a child is born. They call it a prenatal diagnosis. The prenatal diagnosis includes echocardiography and ultrasound examination of the fetus.

Based on the findings of these tests, the parents may decide to proceed with the pregnancy.

Shone’s Syndrome Treatment: Things to Know

Surgery is the ultimate treatment for Shone’s syndrome. A patient may need more than one surgery depending on the severity of the lesions.

Surgeries might involve repair of the valve between the left atrium and left ventricle, removal of the lesions, and valve replacement.

Sources:

• https://www.ncbi.nlm.nih.gov/pubmed/18621780
• https://www.sciencedirect.com/science/article/pii/S1110578X18300087
• https://obgyn.onlinelibrary.wiley.com/doi/pdf/10.1002/uog.1753

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