Very rare disease characterized by severe and often lethal 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. Incomplete Shone complex defined as combination of at least one left ventricular inflow and one left ventricular outflow obstruction.
Mitral Stenosis, Aortic Stenosis, Coarctation of the Aorta.
Occurs in 0.6% of children 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. Associated with substantial morbidity related to arrhythmia, hear failure, and interventions. Survival in children undergoing surgery is better than predicted.
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.
et al: Shone complex: An under-recognized congenital heart disease with substantial morbidity in adulthood. Can J Cardiol
MS, De la Uz
et al: Late outcomes in children with Shone’s complex: A single-centre, 20-year experience. Cardiol Young
et al: Anaesthetic management of a case of Shone’s syndrome. Ind J Anaesth 48:212, 2004.
et al: The developmental complex of “parachute mitral valve,” supravalvular ring of left atrium, subaortic stenosis and coarctation of aorta. Am J Cardiol