Diagnósticos
Shone syndrome may be diagnosed during pregnancy or after birth. Other times, Shone syndrome may not be recognized until adulthood.
Diagnosis can be challenging because one area of narrowing may be more obvious than the others. For example, a baby may first get care for a narrow area in the aorta. Later tests may show that a heart valve or another part of the left side of the heart is narrow too.
Tests
Your healthcare professional may do tests to find Shone syndrome and learn more about the heart. Tests can help find which parts of the heart are affected. The tests needed depend on age, symptoms and past heart treatments. Tests may include:
- Echocardiogram. This is the main test used to diagnose Shone syndrome. It uses sound waves to create pictures of the beating heart. The test shows the structure of the heart chambers and valves. It also shows how well blood moves through the heart and heart valves. An echocardiogram can show blockages on the left side of the heart.
- Fetal echocardiogram. This type of echocardiogram is done during pregnancy. It may help find congenital heart defects before birth. However, not every part of Shone syndrome may be seen before birth.
- Cardiac magnetic resonance imaging (MRI). This test uses magnetic fields and radio waves to create detailed images of the heart and blood vessels. It can give more information about the heart's structure, pumping chambers and blood flow.
- Computerized tomography scan (CT scan) of the heart. This test uses several X-rays to make detailed pictures of the heart. It may be used to get more information or to guide treatment.
- Cardiac catheterization. A thin tube called a catheter is guided through a blood vessel to the heart. This can measure pressures inside the heart and lungs. It may be especially important when the care team is concerned about pulmonary hypertension.
In adults, finding one congenital heart defect on the left side of the heart can be a reason to look carefully for others.
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Tratamientos
Treatment for Shone syndrome depends on the heart changes and how well blood moves through the heart. Treatment may include medicines and surgery. Sometimes a heart transplant is needed. But if the heart is working as it should and symptoms are mild, treatment may not be needed right away. Your care team can talk with you about next steps.
Medicines
Medicines cannot fix the heart structure changes in Shone syndrome. But they may help manage symptoms or complications.
For example, medicines may be used to:
The medicines needed depend on specific heart changes in Shone syndrome. Talk with your care team about the benefits and possible side effects of any medicine prescribed.
Surgery or other procedures
If narrowing keeps the heart from working well, surgery or a heart procedure usually is done right away. There is not one surgery that fixes all of Shone syndrome. Treatment typically focuses on one heart change at a time. Often, the most serious blockage is treated first. Other structure changes may be treated during the same surgery or at another time.
Surgery or procedures to treat Shone syndrome may include:
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Balloon angioplasty. This treatment uses a thin tube called a catheter and a tiny balloon to help widen a narrowed area of the aorta. This helps improve blood flow. A metal mesh tube called a stent may be placed in the area to keep the aorta open.
Sometimes surgery is needed instead to fix the narrowed area of the aorta.
- Surgery to remove extra heart tissue. A surgeon may remove extra tissue above the mitral valve or below the aortic valve that is blocking blood flow.
- Mitral valve repair or replacement. When possible, surgeons generally try to fix the person's own mitral valve rather than replace it.
- Other procedures. Many other procedures may help improve blood flow through the left side of the heart. The type of procedure or surgery depends on the structure changes that need to be fixed.
Some children with Shone syndrome need more surgery or procedures over time. This can happen as a child grows. It also can happen if a narrowed area comes back or gets worse.
Heart transplant
Heart transplant may be considered when Shone syndrome causes serious heart trouble that continues despite other treatments. Complications such as pulmonary hypertension and the effects of previous heart surgeries can make transplant decisions more complex. Your care team carefully considers when to recommend a heart transplant.
Prognosis
Prognosis is an estimate of what may happen over time and how well you may do after treatment. There is no single life expectancy that applies to everyone with Shone syndrome.
Many people with Shone syndrome do well with treatment and regular heart care.
People with Shone syndrome need heart care for life, even when they feel well. Areas of the heart repaired during childhood may narrow again or change over time. Other heart changes that need treatment may be found later in life. Adults with Shone syndrome can have heart valve issues, repeated narrowing, irregular heartbeats, pulmonary hypertension or heart failure years after childhood surgery. Regular care from a team that specializes in adult congenital heart disease can help find these complications early.
Estrategias de afrontamiento y apoyo
Living with Shone syndrome can mean years of follow-up, testing and sometimes repeated procedures. The care team helps you plan each step. This includes the move from pediatric heart care to adult congenital heart disease care when the time is right.
You can take an active part in your care or your child's care. It may help to keep a record of:
- The exact heart conditions that have been diagnosed.
- Previous heart procedures and surgeries.
- Current medicines. Include their dosages.
- Recent imaging and test results.
- Contact information for your congenital heart disease care team.
You may find that talking with other people who have been through the same situation brings you comfort and encouragement. Ask your healthcare team if there are any support groups in your area.
Pregnancy
It may be possible to have a successful pregnancy with mild congenital heart disease. But pregnancy causes the heart to pump more blood, which may put extra strain on a heart affected by Shone syndrome. A healthcare professional may advise you not to get pregnant if you have complex congenital heart disease, such as Shone syndrome.
The risk depends on your specific heart conditions, previous repairs, heart function and complications such as pulmonary hypertension. Before becoming pregnant, talk with your healthcare team about the possible risks and complications. Together you can discuss and plan for any special care needed during pregnancy.
During pregnancy, your care team also may include a maternal-fetal medicine specialist and other healthcare professionals experienced in heart disease during pregnancy.
Preparación para la consulta
If you or your child has Shone syndrome, appointments often focus on whether any area of narrowing has changed and how well the heart is working.
Make an appointment for a health checkup with a doctor trained in treating congenital heart disease, even if there are no health complications. It's important for people with congenital heart disease to have regular checkups.
What you can do
Whether the appointment is for you or your child, gather information that can help the care team understand any changes in health.
Make a list of:
- Changes in symptoms or new symptoms and when they started.
- Changes in feeding or eating, growth, activity or ability to exercise.
- Episodes of a fast or irregular heartbeat, dizziness, or fainting.
- Previous heart operations and catheter procedures.
- Medicines, vitamins and supplements being taken.
- Questions you want to ask the care team.
Preparing a list of questions can help you and your healthcare professional make the most of your time together. You might want to ask questions such as:
- Which parts of the heart are affected?
- How severe is each area of narrowing?
- Which heart condition needs the closest follow-up?
- Does treatment need to happen now?
- Could another procedure or surgery be needed later?
- How often are follow-up tests needed?
- What physical activities are safe?
- Which symptoms mean I should call the care team?
- What are the risks of becoming pregnant?
- Would genetic counseling be useful for my family?
What to expect from your doctor
The healthcare team may ask questions to understand symptoms and how the heart condition is affecting daily life.
Your healthcare team may ask:
- When were the heart conditions first diagnosed?
- What procedures or surgeries have been done?
- Have symptoms changed recently?
- Is feeding or eating a concern?
- Has exercise or activity become more difficult?
- Have there been palpitations, dizziness or fainting?
- Are there symptoms while resting or sleeping?
- What medicines are being taken?
- When was the last echocardiogram, MRI or CT scan?
Oct. 01, 2026