Double Aortic Arch: An Unreported Anomaly with Kabuki Syndrome

• DOI: 10.1007/s00246-008-9286-0
• Publication Date: 2008-08-06T14:58:43Z
• Authors: Sergio Moral; Flavio Zuccarino; Pablo Loma-Osorio
• Keywords: Kabuki; Kabuki syndrome; Dwarfism; Great vessels

Kabuki syndrome is a rare congenital disorder characterized by mental retardation, postnatal dwarfism, visceral anomalies, and peculiar facies (resembling the makeup of actors in Kabuki, traditional Japanese theater) initially described in Japan. Congenital heart and great vessel defects are the most frequent visceral anomalies found in children with Kabuki syndrome. Although many cardiovascular defects have been described, a double aortic arch has never been reported with Kabuki syndrome.