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
Mitral Stenosis, Aortic Stenosis, Coarctation of the
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.
Sekhar KC, Mastan S, Farooqi A, et al: Anaesthetic management of a case of Shone's
syndrome. Ind J Anaesth 48:212, 2004.
Shone JD, Sellers RD, Anderson RC et al: The developmental complex of
“parachute mitral valve,” supravalvular ring of left atrium, subaortic
stenosis and coarctation of aorta. Am J Cardiol