![]() In addition to posterior malalignment of the conal septum relative to the ventricular septum, Jonas has pointed out a prominent muscle bundle on the left ventricular (LV) free wall that can project into the left ventricular outflow tract (LVOT) (muscle of Moulaert), significantly contributing to outflow obstruction ( Fig. The aortic valve is usually bicuspid (80% to 90%) with varying degrees of commissural fusion and annular hypoplasia. The presence of IAA type B with or without DiGeorge phenotype is an indication for genetic screening for the 22q11 deletion in the patient. Reports of IAA type A with DiGeorge syndrome are rare. IAA is usually a rare anomaly, but in DiGeorge syndrome it is a common defect.Ĭonley and associates reported that 36% of their patients with DiGeorge syndrome had type A IAA, and all of their autopsied patients had congenital heart disease. Using fluorescence in situ hybridization (FISH) analysis, Lewin and colleagues demonstrated that 50% to 80% of patients with IAA type B have 22q11.2 chromosomal deletions. The association of DiGeorge syndrome with type B IAA suggests that both are part of a causally heterogeneous developmental field defect. Unlike those with coarctation, the predominant extracardiac abnormality in these patients is DiGeorge syndrome. Many patients with IAA have extracardiac anomalies. IA, Innominate artery LC, left carotid artery LPA, left pulmonary artery LS, left subclavian artery MPA, main pulmonary artery PDA, posterior descending artery RPA, right pulmonary artery. Type A is interrupted distal to the left subclavian artery, type B between the left subclavian and left carotid arteries, and type C proximal to the left carotid artery. This has been observed in patients with single-ventricle anatomy.Ĭeloria and Patton classification of interrupted aortic arch. In nearly all patients the aortic arch sidedness is left, but rarely the IAA can be right sided with the descending aorta in the right hemithorax. In rare cases, when IAA is not associated with VSD, aortopulmonary window should be suspected. ![]() The VSD is typically conoventricular in origin and is associated with posterior malalignment of the conal septum and varying degrees of left ventricular outflow tract obstruction (LVOTO) at the subvalvar and/or valvar level. Also seen are aortic valve anomalies, truncus arteriosus, and double-outlet right ventricle (RV). All patients have a patent ductus arteriosus, and most have a ventricular septal defect (VSD). Type B IAA is the most common (78%), followed by type A (20%) and type C (2%) ( Fig. Celoria and Patton described the currently used anatomic definitions, in which type A is interrupted distal to the left subclavian artery (LSCA) type B is interrupted between the left subclavian and left carotid arteries in type B1 the origin of the right subclavian artery (RSCA) is on the descending thoracic aorta and type C is interrupted proximal to the left carotid artery. The incidence of IAA is 1% of all congenital heart defects. Interrupted aortic arch (IAA) is a rare but highly lethal form of congenital heart disease, carrying a mortality rate higher than 90% in the neonatal period if not treated.
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