What is aortic arch disruption? Aortic arch disruption is a disruption in the anatomy and luminal continuity of the aortic arch and is a rare congenital heart condition that accounts for approximately 1% of congenital heart disease in infants and children. In a few cases, there may be fibrous strips connecting the proximal aortic arch to the distal descending aorta. interrupted aortic arch was first described by Steidele in 1778. Aortic arch disruption rarely occurs alone and is almost always combined with other cardiovascular malformations, most commonly ventricular septal defect and patent ductus arteriosus. What are the manifestations of aortic arch disruption? The pathophysiological changes of this cardiac malformation are very complex and severe, so the clinical manifestations occur early and develop rapidly. When aortic arch disruption is combined with ductus arteriosus, the proximal part of the disruption, i.e. the upstream, is normally supplied by the ascending aorta, and the distal descending aorta is supplied by the pulmonary artery via the ductus arteriosus, resulting in non-oxygenated blood with low oxygen content in the lower half of the body. Common clinical manifestations include: 1. In those with ventricular septal defect, the ventricular level is left-to-right shunt, pulmonary blood flow is severely increased, congestive heart failure and severe pulmonary hypertension can occur early after birth, and the condition can deteriorate rapidly. Parents may feel that the child has feeding difficulties, shortness of breath, excessive sweating, and slow weight gain. The child also has differential cyanosis (different color and blood oxygenation in the upper and lower extremities) and unequal blood pressure in the extremities. 2.Differential cyanosis can be reduced as pulmonary hypertension develops and causes intracardiac bidirectional shunts. 3.As the arterial catheter constricts or closes, the child soon exhibits severe acidosis as well as liver and kidney failure due to inadequate perfusion of the lower body, manifested by anuria, elevated glutamate transaminase, and necrotizing small bowel inflammation. 4. As the disease continues to progress, the acidosis further worsens, eventually leading to damage to all organs, including the brain and heart itself. The child presents with convulsions and diminished response. The diagnosis of aortic arch disruption relies on echocardiography, cardiovascular CT or angiography. 1, Echocardiography is an important routine diagnostic tool for aortic arch disruption and has the advantage of identifying the aortic arch disruption malformation along with the diagnosis of intracardiac malformations. 2, CT and MRI are of confirmatory significance for the diagnosis of aortic arch disruption. Contrast-enhanced magnetic resonance angiography sequences are the most reliable for diagnosis. Because CT and MRI are non-invasive and do not require femoral artery cannulation, they can basically replace cardiovascular angiography in children and infants. 3. Cardiovascular angiography has been the gold standard for the diagnosis of aortic arch disruption for many years. Left ventriculography or ascending aortography should be performed whenever possible during the examination. In the presence of a ventricular septal defect, the contrast agent of left ventriculography can enter the descending aorta through the defect, the right ventricle, the pulmonary artery and the arterial conduit, which can simultaneously show both ends of the interrupted aortic arch. The natural prognosis of aortic arch disruption is poor, with 75% of deaths occurring in the neonatal period (28 days). Therefore, children with aortic arch disruption should be treated surgically as soon as possible after birth, regardless of age. There are many surgical treatment options, which should be selected based on the patient’s specific situation and the experience of the operator. In the early years, palliative surgery, mainly pulmonary artery circumflexion, was often used for newborns and infants, followed by radical surgery at the age of 3-5 years. Currently, the pediatric center of Fulbright Hospital uses a radical treatment strategy of one-stage surgery for children with combined intracardiac malformations. Surgical treatment is contraindicated in older patients with irreversible pulmonary vasculopathy. The surgery consists of two main components: aortic arch reconstruction and correction of intracardiac malformations. Aortic arch reconstruction can be performed by direct anastomosis of the aortic arch. The surgery is performed under intravenous compound general anesthesia, lower body stop circulation and selective cerebral perfusion, and the descending aorta is incised and anastomosed to the aortic arch. For the correction of combined intracardiac malformations, the basic principle is that biventricular repair should be performed in the neonatal period if two ventricles are present. Combined ventricular septal defect, permanent arterial trunk, and main pulmonary artery septal defect are usually treated with conventional surgical approaches. If combined with transposition of the great arteries, ventricular septal defect, and interrupted aortic arch one should perform aortic transposition, ventricular defect repair, and direct aortic arch anastomosis. What are the treatment results This disease is difficult to treat and requires a high level of surgeons, extracorporeal circulation and anesthesiologists, and is carried out in only a few cardiac centers in China. The pediatric center of Fu Wai Hospital, the largest cardiac center in China, completed 30 cases in 2014, the largest group of clinical reports in China, with a 100% success rate of surgery and no death. However, the biggest problem affecting the long-term outcome of surgical treatment is the presence of a certain incidence of restenosis, and these patients may require reoperation. The main factors contributing to this are related to surgical technique and the use of artificial materials that are unlikely to grow with age. Thanks to improved surgical techniques, most patients can be treated with a direct aortic arch anastomosis technique, avoiding the use of artificial materials, which can reduce the incidence of anastomotic restenosis.