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Chapter 43 - Pulmonary Hypertension and Moyamoya Disease

from Section 7 - Miscellaneous Lesions and Syndromes

Published online by Cambridge University Press:  09 September 2021

By
Timothy D. Switzer  and
Neil M. Goldenberg
Edited by
Laura K. Berenstain  and
James P. Spaeth
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

Moyamoya disease is a cerebrovasculopathy of unknown etiology characterized by chronic progressive stenosis of the arteries of the circle of Willis. An extensive collateralized circulation forms, giving rise to the smoky appearance seen on cerebral angiography (moyamoya is Japanese for “puff of smoke”). Primary clinical features of moyamoya disease, especially in children, are related to cerebral ischemia and include transient ischemic attacks and stroke. The goals of treatment are primarily to restore blood flow to the affected areas, either by direct or indirect bypass, as there is no curative treatment for moyamoya disease. This chapter details the perioperative management of a patient with coexisting morbidities of moyamoya disease, coarctation of the aorta, and pulmonary hypertension, discussing the concerns related to each and their considerations in developing an anesthetic management plan.

Keywords

coarctation of the aortamoyamoya diseaseneuroimagingpulmonary hypertensionpulmonary vasodilators
Type
Chapter
Information
Congenital Cardiac Anesthesia
A Case-based Approach
 , pp. 331 - 337
Publisher: Cambridge University Press
Print publication year: 2021

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References

Suggested Reading

Abman, S. H., Hansmann, G., Archer, S. L., et al. Pediatric pulmonary hypertension. Circulation 2015; 132: 2037–99.Google Scholar
Appireddy, R., Ranjan, M., Durafourt, B. A., et al. Surgery for moyamoya disease in children. J Child Neurol 2019; 088307381984485.Google Scholar
Arlachov, Y. and Ganatra, R. H. Sedation/anaesthesia in paediatric radiology. Br J Radiol 2012; 85: e1018–31.CrossRefGoogle ScholarPubMed
Del Cerro, M. J., Abman, S., Diaz, G., et al. A consensus approach to the classification of pediatric pulmonary hypertensive vascular disease: Report from the PVRI Pediatric Taskforce, Panama 2011. Pulm Circ 2011; 1: 286–98.Google Scholar
Lammers, A. E., Adatia, I., Del Cerro, M. J., et al. Functional classification of pulmonary hypertension in children: Report from the PVRI Pediatric Taskforce, Panama 2011. Pulm Circ 2011; 1: 280–5.Google Scholar
Pritts, C. D. and Pearl, R. G. Anesthesia for patients with pulmonary hypertension. Curr Opin Anaesthesiol 2010; 23: 411–16.Google Scholar

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