Shone Complex or Syndrome

Very rare disease characterized by severe and often lethal triple cardiac malformation. It is a series of four obstructive or potentially obstructive left-sided cardiac lesions consisting of supravalvular mitral ring, parachute deformity of the mitral valve, subaortic stenosis, and coarctation of the aorta.

Mitral Stenosis, Aortic Stenosis, Coarctation of the Aorta.

In the United States, it has been suggested that congenital mitral valve stenosis occurs in 0-5% of patients with congenital heart disease. There is no racial or sex predilection.

This syndrome is present at birth. It is an association of multiple levels of left ventricular inflow and outflow obstruction (subvalvar and valvar left ventricular outflow tract obstruction, coarctation of the aorta, and mitral stenosis). Death can occur in first weeks of life.

Document the level of obstruction (heart catheterization with or without provocative testing). Quantify the severity and anatomy of the obstruction (clinical examination, ECG, chest radiographs, echo-Doppler, transesophageal echocardiography to define precisely the anatomy, MRI to assess associated lesions such as pulmonary artery stenosis or coarctation). Prophylactic antibiotics should be used as indicated. Consider invasive intraoperative blood pressure measurement. Perioperative cardiac monitoring is mandatory.