|How Is It Treated?
The treatment of Shone’s Complex consists of addressing each of the constituent defects.
1. Coarctation of the aorta – Initially a prostaglandin infusion is used for severe coarctation in the neonate. Surgical treatment is usually preferred. Two forms of coarctation surgery are Excision with End-to-End Anastomosis and Subclavian Flap Angioplasty. Alternatively, in older children, especially with recoarctation after surgical repair, may be treated with a transcatheter balloon angioplasty. A catheter (hollow tube) is inserted into the obstructed area of the aorta and a balloon (with or without a stent) is expanded to open up the vessel.
2. Subaortic Stenosis – Obstruction of the outflow tract from the left ventricle into the aorta may be treated surgically by excising the excess tissue below the aortic valve (septal myectomy). If other forms of aortic stenosis are present, surgical repair may involve the replacement of the aortic valve.
3. Mitral Stenosis (caused by “Parachute” mitral valve and by Supravalvar mitral membrane) – are usually repaired by surgery. The mitral stenosis may be corrected by valve repair (e.g. “Sliding Plasty” Technique) or valve replacement (e.g. with a St. Jude mechanical valve).
Other procedures may be necessary to repair other defects, such as patent ductus arteriosus or atrial or ventricular septal defects. Mitral valve repair is usually performed early to avoid the onset of pulmonary hypertension.