What is Shone's syndrome?

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

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.

  

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Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: 1/29/2019 3:21:13 PM