Partial anomalous left pulmonary artery along with aortic coarctation in an infant with Kabuki syndrome.
We report an antenatally diagnosed fetal coarctation delivered prematurely and confirmed to have coarctation as well as additional nonobstructing anomalous left pulmonary artery branch (aLPA) from right pulmonary artery (RPA) and significant dysmorphic features. The baby underwent an uncomplicated arch repair, but had numerous multisystem and growth related issues prompting a diagnosis of Kabuki syndrome (KS) at 1 year of age. While coarctation and hypoplastic left heart syndrome are observed in this syndrome, this is the second reported case of aLPA in KS and the first with the forme fruste of left-sided obstruction as well as aLPA in this group of patients.
Division of Pediatric Cardiology, Seattle Children's Hospital, University of Washington, Seattle, Washington 98105, USA. Aarti.bhat@seattlechildrens.org
Aarti H
AH
Bhat
Jennifer
J
Davenport
Mark
M
Cocalis
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging* / Q000601:surgery*; D001017:Aortic Coarctation / Q000000981:diagnostic imaging* / Q000601:surgery*; D005145:Face / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000601:surgery; D006402:Hematologic Diseases / Q000000981:diagnostic imaging* / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging* / Q000601:surgery; D016896:Treatment Outcome; D016216:Ultrasonography, Prenatal; D015837:Vestibular Diseases / Q000000981:diagnostic imaging* / Q000601:surgery*