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A patient with Kabuki syndrome and a parachute mitral valve was referred for mitral stenosis. Echocardiography indicated right ventricular dilatation and a suspected vertical ascending vein. Cardiac CT confirmed dual drainage of the left upper pulmonary vein and hemiazygos vein continuity. Despite echocardiographic limitations, surgery was performed. This rare case is reported due to its unique anatomical variation.

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.

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A rare case of parachute mitral valve and dual drainage of the left upper pulmonary vein in a patient with Kabuki syndrome

Chapter 20 - Shone Complex

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A rare case of parachute mitral valve and dual drainage of the left upper pulmonary vein in a patient with Kabuki syndrome

Chapter 20 - Shone Complex

Summary