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:
- Aortic coarctation (narrowing of the aorta)
- Obstruction below the aortic valve (subaortic obstruction- blockage below the valve)
- Mitral valve leaflets which are thickened and stuck together giving the valve a “parachute” shape
- 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: 6/12/2018 2:01:00 PM
From the Newsdesk
Naialee Perez had just given birth to her first child, a baby boy named Liam, when a category five hurricane was making its way towards her hometown in the island of Puerto Rico. Liam was on a ventilator and undergoing treatment for a congenital heart defect in Hospital del Niño in San Juan while those on the island prepared for what would become one of the most catastrophic hurricanes in its history.
While he was still inside his mother’s womb, Luife was diagnosed with transposition of the great arteries, a congenital heart defect. Shortly after birth, Luife was taken by ambulance to the cardiac team at Nicklaus Children’s. The pediatric cardiology team took Luife’s heart apart, piece by delicate piece, and successfully, put it back together. Today, Luife is a healthy, active and outgoing 8-year-old boy who wears his “Scar of Honor” with pride.