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Chapter 20 - Shone Complex

from Section 3 - Left-Sided Obstructive Lesions

Published online by Cambridge University Press:  09 September 2021

Laura K. Berenstain
Affiliation:
Cincinnati Children's Hospital Medical Center
James P. Spaeth
Affiliation:
Cincinnati Children's Hospital Medical Center
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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.

Type
Chapter
Information
Congenital Cardiac Anesthesia
A Case-based Approach
, pp. 129 - 136
Publisher: Cambridge University Press
Print publication year: 2021

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References

References

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Suggested Reading

Atkinson, T. M., Giraud, G. D., Togioka, B. M., et al. Cardiovascular and ventilatory consequences of laparoscopic surgery. Circulation 2017; 135: 700–10.CrossRefGoogle ScholarPubMed
Friesen, R. H. Anesthetic drugs in congenital heart disease. Semin Cardiothorac Vasc Anesth 2014; 18: 363–70.CrossRefGoogle ScholarPubMed
Schimke, A., Majithia, A., Baumgartner, R., et al. Intervention and management of congenital left heart obstructive lesions. Curr Treat Options Cardiovasc Med 2013; 15: 632–45.Google Scholar
Shone, J. D., Sellers, R. D., Anderson, R. C., et al. The developmental complex of “parachute mitral valve,” supravalvular ring of left atrium, subaortic stenosis, and coarctation of aorta. Am J Cardiol 1963; 11: 714–25.CrossRefGoogle ScholarPubMed
Spaeth, J. P. and Loepke, A. W. Anesthesia for left-sided obstructive lesions. In Andropoulos, D. B., Stayer, S., Mossad, E. B., et al., eds. Anesthesia for Congenital Heart Disease, 3rd ed. Hoboken, NJ: John Wiley & Sons, 2015; 497515.CrossRefGoogle Scholar

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