Shone's complex

Question 1

What is Shone’s complex?

Answer 1

Sone’s complex is a rare congenital heart disease with:
(CAM)

-coarctation of aorta

• valvular and subvalvular aortic stenosis
• mitral stenosis (parachute deformity of he mitral vave)

-small left ventricle due to to inlet and outlet obstruction

Question 2

Shone’s complex can be associated with:

Answer 2

• PDA
• Interrupted aortic arch
• Bicuspid aortic valve
• Atrial septal defect
• Ventricular septal defect

Question 3

How is Shone’s complex treated?

Answer 3

Coarctation of aorta - prostaglandin is used for severe coarctation of the neonate. Surgical tx is preferred however.

Subaortic stenosis - septal myetomy - excise excess tissue below the aortic valve

mitral stenosis - repaired with mechanical valve (St. Jude mechanical valve for example)

Question 4

How does Shone differ from hypoplastic left heart?

Answer 4

Sone’s complex is not associated with hypoplasia of the left heart and aorta which is a fundamental feature of hypoplastic left heart complex.