Chapter 20 - Shone Complex

Summary

Shone’s complex is a group of left-sided, typically obstructive, lesions of the heart, also referred to as Shone’s syndrome, disorder, or anomaly. Shone’s complex was first described in 1963 as four left-sided heart lesions consisting of a supravalvular ring of the left atrium, a “parachute” mitral valve, muscular or membranous subaortic stenosis, and a coarctation of the aorta. These anomalies cause a progressive limitation of inflow into the left ventricle and outflow obstruction from the left ventricle and aorta. There is also an association with a smaller left ventricular size and decreased left ventricular function. Lesions may progressively worsen over time, causing significant heart failure symptoms, pulmonary hypertension, and arrhythmias. Surgical intervention may range from a single intervention such as aortic arch repair to a combination of multiple procedures such as repair of aortic coarctation along with mitral valve repair and aortic valvotomy. This chapter details the perioperative management of a child with multilevel left-sided obstructive lesions presenting for noncardiac surgery.