![]() ![]() ![]() The procedure yielded minimal intraoperative blood loss and enabled complete postoperative recovery without any sequelae. We repaired our patient's IAA in a single stage with a 16-mm Dacron graft. Ventral aortic repair through a midline approach-the preferred technique for repairing IAA in adult patients-avoids damaging the extensive network of collateral vessels and in all likelihood reduces the risk of morbidity and death. Follow-up examinations have revealed good graft patency. The first report of a trisomy of distal chromosome 5q associated with interrupted aortic arch interruption is reported, and it is concluded that gene dosage. 8–16 In no case have neurologic, renal, or gastrointestinal complications, re-interventions, or in-hospital or late deaths been noted. In adults, IAA has typically been repaired in a single stage by means of an extra-anatomic approach. This can provide essential information regarding your individual case. Should I have more tests done Your caregiver may refer you for genetic counseling and genetic testing. We sought to analyze factors associated with these different surgical approaches. We hypothesize that these adult patients actually had a severe form of aortic coarctation that transformed into an interruption-which would mean that the adult form of aortic interruption is altogether different from the neonatal form. In many cases, however, no genetic cause is found. In patients with interrupted aortic arch and ventricular septal defect (VSD) with a small left ventricular outflow tract (LVOT), either aortopulmonary amalgamation or a Ross-Konno type procedure can be performed to create stable systemic outflow. Infants with IAA typically have an associated VSD, 6 whereas adults do not and type B interruption is prevalent in infants, 6 whereas type A interruption has been reported most often in adults. These patients can later present with features of systemic hypertension and have what is called the adult form of IAA.ĭifferences exist between the neonatal and adult forms of IAA. 6,7 It is not known what causes these collateral vessels to form so early in life. In neonatal IAA, substantial collateral circulation must be present to enable survival to adulthood, 1 and some neonates with IAA develop collateral vessels that maintain blood flow to the distal aorta. 5 In these instances, the presentation ranged from no symptoms to swollen limbs with higher blood pressures in the upper limbs than in the lower limbs. Early diagnosis and intervention for IAA is critical for the baby’s survival.As of 2010, investigators had identified only 30 cases of isolated IAA in adults in the world medical literature. Mutation in the NKX2-5 gene (600584) has been found in a patient with interrupted aortic arch and in a patient with PTA. In babies with IAA, when this vessel closes, the blood flow to the body is stopped. Within the first three days after delivery, the ductus arteriosis-a blood vessel that allows for circulation during fetal development-will typically close. IAA is a relatively rare defect-only 1% of all congenital heart defects are IAA. However, i t is almost always associated with other cardiac defects, such as ventricular septal defect, patent ductus arteriosus, bicuspid aortic valve, subaortic stenosis, aortopulmonary window, truncus arteriosus, d-transposition of the great arteries, double outlet right ventricle, atrioventricular septal defect, or single ventricle.īabies with IAA have poor circulation and weak or absent pulse on the lower half of their body and in some cases, the left arm. Doctors do not know exactly what causes this defect, but it is usually associated with other heart conditions as well as some genetic disorders such as DiGeorge syndrome (22q11.2 deletion syndrome). IAA happens very early in a baby’s development, usually between the 5 th and 7 th week of fetal development. See illustrations that show an example of a heart with IAA Only about 4% of babies with IAA have Type C. Type C: This is a complete interruption in the aorta between the innominate artery and the left common carotid artery. Interrupted Aortic Arch Pregnancy Issues Pregnancy may be undergone without unusual risk in the absence of residual cardiac defects or stenosis of the aorta, either at the site of the aortic arch repair or in the left ventricular outflow tract. Type B can be related to possible genetic conditions. Type B: This is a complete interruption in the aorta that occurs between the left common carotid artery and the left subclavian artery branch. Type A: This is a complete interruption in the aorta that occurs after the left subclavian artery branch. An interrupted aortic arch (IAA) is a complete interruption, or gap, in the aorta that prevents normal blood flow through the heart. There are three types of IAA, based on where the gap in the aorta occurs. ![]()
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