Shone's Syndrome

Also known as: Shone syndrome, Shone’s complex, Shone's anomaly.

What is Shone's syndrome?

Shone’s syndrome, is a rare form of congenital heart disease where there is a combination of four left-sided heart defects (obstructions), which include:
  1. Aortic coarctation (narrowing of the aorta)
  2. Obstruction below the aortic valve (subaortic obstruction- blockage below the valve)
  3. Mitral valve leaflets which are thickened and stuck together giving the valve a “parachute” shape
  4. Abnormalities of the mitral valve with stenosis (narrowing) and leaking (mitral regurgitation). These get worse over time.
 

What causes Shone’s syndrome? 

Shone’s syndrome develops very early on in the fetus and occurs in both sexes, and all races, or ethnic groups. The cause is unknown.
 

What are the symptoms of Shone's syndrome? 

Symptoms of congestive heart failure (which can occur in the first week of life) include fatigue, rapid breathing and wheezing, faster than normal heart rate, poor oral intake, poor weight gain, fluid retention (edema) in the legs, pallor (anemia), and frequent pneumonias.
 

What are Shone’s syndrome care options?

Depending on when the diagnosis is made, (sometimes the diagnosis is made when the baby is still in the mother's uterus), early delivery, medications and a number of surgeries or catheter-based techniques are required to treat the individual problems related to Shone’s syndrome. Several procedures are needed to fix all the problems.

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: 3/23/2018 2:06:28 PM

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Redmond Burke, MD is the Director of Cardiovascular Surgery at Nicklaus Children's Hospital.


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