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PART II: - HEREDITARY AND GENETIC CONDITIONS AND MALFORMATIONS

Published online by Cambridge University Press:  06 January 2010

Louis R. Caplan
Affiliation:
Beth Israel Deaconess Medical Center, Boston
Julien Bogousslavsky
Affiliation:
Valmont Clinique, Glion, Switzerland
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Summary

Pulmonary arteriovenous malformations (PAVMs) are rare occurrences among the population. These malformations can be acquired in various clinical settings or be congenital. Single PAVMs <2 cm in diameter may be asymptomatic. The severity of symptoms is believed to be proportionally related to the size (diameter) of the PAVMs. The specific anatomy of the vascular malformation must then be analyzed either with helical computed tomography (CT) or magnetic resonance angiography (MRA). Digital subtraction angiography is eventually performed. The aims of treatment are to prevent neurological complications and pulmonary hemorrhage and to improve hypoxemia. Lobectomy or pneumonectomy is performed only in rare situations such as in patients in whom the feeding artery is so short that there is a risk of coil migration. Embolization therapy is applied for PAVMs, and is carried out by transcatheter embolotherapy, which consists in obliterating the feeding artery.
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Publisher: Cambridge University Press
Print publication year: 2008

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