Shone’s complex is a heart condition that is present at birth. Babies with the condition have at least three defects that affect blood flow in the left side of their heart. Usually, treatment includes surgery soon after birth. Adults with Shone’s complex need to visit a cardiologist (heart doctor) regularly.
Shone’s complex is a congenital (present at birth) heart disease. It affects how blood flows both into and out of the left side of the heart. The multiple sites of blockage in the blood flow through the left side of the heart differentiates Shone’s syndrome from other isolated heart defects. Shone’s syndrome is another name for Shone’s complex.
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Shone’s complex is named for the person who discovered it. Originally, Shone’s was associated with four types of heart problems. Now, experts have identified up to eight components of congenital heart defects related to Shone’s syndrome.
Babies born with Shone’s complex have at least three of these heart conditions:
Shone’s complex is very rare. It accounts for less than 1% of all congenital heart diseases.
Shone’s complex is variable. Some patients are diagnosed before birth, while others are diagnosed as a result of heart murmurs or other findings including:
Babies born with Shone’s complex may also have symptoms of congestive heart failure, such as:
Healthcare providers don’t always know exactly what causes Shone’s complex. Some factors can increase the chances that a baby will be born with a congenital heart defect. Some of these risks include genetic and environmental factors.
Healthcare providers may use a range of tests to diagnose Shone’s complex. These tests tell providers about the heart’s blood flow and size:
Most babies who have Shone’s complex require surgery soon after birth. The surgery type and technique depend on which of the eight possible defects the baby has.
The surgery type also depends on how severe the baby’s symptoms are. Often, babies need multiple surgeries to treat all the heart defects of Shone’s complex.
Adults who have Shone’s complex need to visit a cardiologist (heart doctor) at least once a year. During this visit, your cardiologist uses a few tests to check your heart’s function:
There is no guaranteed way to prevent having a child with Shone’s complex. You can increase the chances that your baby will be healthy by:
Babies who receive treatment for Shone’s complex often grow into adults who experience a high quality of life. In general, babies who have surgery tend to have good outcomes. A baby’s chances of a positive outlook also depend on how complex or severe the congenital heart defects are.
You may also want to ask your healthcare provider:
A note from Cleveland Clinic
Shone’s complex is a congenital heart disease. It involves multiple heart defects that affect blood flow. Babies born with the condition may show signs of congestive heart failure. Most babies need surgery shortly after birth to treat Shone’s complex. Babies who have surgery usually grow up to live healthy and full lives. Adults who were born with Shone’s complex need regular follow-up with a cardiologist for the rest of their life.
Last reviewed by a Cleveland Clinic medical professional on 08/04/2021.
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