Deletion 22q11 in patients with interrupted aortic arch. Marino B, Digilio MC, Persiani M, Di Donato R, Toscano A, Giannotti A, Dallapiccola B. Frequency of 22q11 deletions in patients with conotruncal defects. Goldmuntz E, Clark BJ, Mitchell LE, Jawad AF, Cuneo BF, Reed L, McDonald-McGinn D, Chien P, Feuer J, Zackai EH, Emanuel BS, Driscoll DA. Incidence and significance of 22q11.2 hemizygosity in patients with interrupted aortic arch. Rauch A, Hofbeck M, Leipold G, Klinge J, Trautmann U, Kirsch M, Singer H, Pfeiffer RA. A genetic etiology for interruption of the aortic arch type B. Lewin MB, Lindsay EA, Jurecic V, Goytia V, Towbin JA, Baldini A. Cardiovascular anomalies in DiGeorge syndrome and importance of neural crest as a possible pathogenetic factor. Association of interrupted aortic arch with malformations producing reduced blood flow to the fourth aortic arches. Hemodynamic considerations in the development of narrowing of the aorta. Interruption of the aortic arch and coarctation of the aorta: pathogenetic relations. The ductus arterious and associated cardiac anomalies in interruption of the aortic arch. Oppenheimer-Dekker A, Gittenberger-de Groot AC, Roozendaal H. Solitary interruption of the arch of the aorta. Risk factors associated with mortality and interventions in 472 neonates with interrupted aortic arch: a Congenital Heart Surgeons Society study. McCrindle BW, Tchervenkov CI, Konstantinov IE, Williams WG, Neirotti RA, Jacobs ML, Blackstone EH. Congenital Heart Surgery Nomenclature and Database Project: patent ductus arteriosus, coarctation of the aorta, interrupted aortic arch. Anatomic features affect mortality and initial LVOT procedures, whereas characteristics of the arch repair affect arch reintervention.īacker CL, Mavroudis C. Recurrence of arch obstruction has been reported from 10 to 30 %, while recurrence of LVOTO has averaged 20 %. Current early mortality for repair of IAA with VSD ranges from 4 to 10 %. A severe LVOTO may require more aggressive procedures, e.g. Aortic arch reconstruction is accomplished by end-to-side anastomosis with the ascending aorta, more or less associated with patch augmentation of the anastomosis. Staged repair was popular in the past, but primary repair through a median sternotomy (using either deep hypothermic circulatory arrest or moderately hypothermic antegrade selective cerebral perfusion) is currently the preferred approach. DiGeorge syndrome is another frequently associated feature with important clinical implications. Careful preoperative assessment, and particularly the identification of a severe left ventricular outflow tract obstruction (LVOTO, <4 mm), is crucial for a successful outcome. The critical condition of these neonates dictates early referral and surgical treatment. Only type B is, but not exclusively, a true cono-truncal anomaly. Three types of IAA – A, B and C – are classicaly recognized. Interrupted aortic arch (IAA), usually associated with a ventricular septal defect (VSD), is a rapidly fatal congenital heart anomaly with ductus-dependent systemic circulation.

Interrupted aortic arch survival rate