Shone's & CHD

Owen's Diagnosis - Shone's Complex

Shone’s Complex (also known as Shone’s Syndrome, Disorder, or Anomaly) is a rare congenital heart disease that restricts blood flow both in and out of the heart’s left ventricle. The classic Shone's consists of four defects, which are depicted in the illustration below. These are (1) parachute mitral valve, (2) supravalvar mitral membrane, (3) subaortic stenosis, and (4) coarctation of the aorta. But there are also incomplete forms of the disease in which only two or three of these defects are present, and cases in which other congenital heart defects also occur (e.g. patent ductus arteriosus, interrupted aortic arch, bicuspid aortic valve, atrial septal defect, ventricular septal defect).

Shone's Complex information found at Cove Point Foundation

Congenital Heart Defect

A congenital heart defect (CHD) is a heart problem that’s present at birth. It’s caused by improper development of the heart during fetal development. Congenital heart defects are the most common birth defects.

• 1 percent of babies are born with a CHD.

• Nine out of every 1,000 babies born in the US are born with a congenital heart abnormality.

• One third of these babies require intervention to prevent death in the first year of life.

• 85 to 90 percent of CHDs have no known causes.

• 5 to 6 percent are related to chromosome abnormality.

• 3 to 5 percent are related to single gene defects.

• 2 percent are related to environmental factors.

• Approximately 1.3 million people living in the US today were born with a congenital heart defect, and at least half of these individuals are under age 25.

• Although some babies will be diagnosed during gestation or at birth, sometimes the diagnosis is not made until days, weeks, months, or even years after. In some cases, CHD is not detected until adolescence or adulthood

CHD information can be found at Boston Children's Hospital