Pathologic aneurysmal dilation of the ascending aorta and dilation of the main pulmonary artery in patients with Kabuki syndrome: valve-sparing aortic root replacement.

TitlePathologic aneurysmal dilation of the ascending aorta and dilation of the main pulmonary artery in patients with Kabuki syndrome: valve-sparing aortic root replacement.
Publication TypeJournal Article
Year of Publication2007
AuthorsDyamenahalli U, Abraham B, Fontenot E, Prasad V, Imamura M
JournalCongenital heart disease
Volume2
Issue6
Pagination424-8
Date Published2007 Nov
Abstract

We report the aneurysmal dilation of the ascending aortai and the main pulmonaryi artery in 2 children with Kabuki syndrome. In 1 patient, there was progressive aneurysmal dilation of the ascending aorta necessitating aortoplasty. Histologic examination of the resected aorta revealed disrupted and fragmented elastic fibers in the medial layer, along with mucinous degeneration of the aortic wall. This is the first recognition and report of these findings as part of the Kabuki syndrome.

Citation Key18377436